Criteria for the Classification and Diagnosis of the Rheumatic Diseases

Transcription

1 APPENDIX I Criteria for the Classification and Diagnosis of the Rheumatic Diseases The criteria presented in the following section have been developed with several different purposes in mind. For a given disorder, one may have criteria for (1) classification of groups of patients (e.g., for population surveys, selection of patients for therapeutic trials, or analysis of results on interinstitutional patient comparisons); (2) diagnosis of individual patients; and (3) estimations of disease frequency, severity, and outcome. The original intention was to propose criteria as guidelines for classification of disease syndromes for the purpose of assuring correctness of diagnosis in patients taking part in clinical investigation rather than for individual patient diagnosis. However, the proposed criteria have in fact been used as guidelines for patient diagnosis as well as for research classification. One must be cautious in such application because the various criteria are derived from the use of analytic techniques that allow the minimum number of variables to achieve the best group discrimination, rather than to attempt to arrive at a diagnosis in an individual patient. The proposed criteria are empiric and not intended to include or exclude a particular diagnosis in any individual patient. They are valuable in offering a standard to permit comparison of groups of patients from different centers that take part in various clinical investigations, including therapeutic trials. The ideal criterion is absolutely sensitive (i.e., all patients with the disorder show this physical finding or the positive laboratory test) and absolutely specific (i.e., the positive finding or test is never present in any other disease). Unfortunately, few such criteria or sets of criteria exist. Usually, the greater the sensitivity of a finding, the lower its specificity, and vice versa. When criteria are established attempts are made to select reasonable combinations of sensitivity and specificity. An updated listing of additional criteria sets for rheumatic and musculoskeletal disorders is available on the American College of Rheumatology website ( index.asp?aud=mem). CRITERIA FOR THE CLASSIFICATION Of FIBROMYALGIA a 1. History of widespread pain Definition. Pain is considered widespread when all of the following are present: pain in the left side of the body, pain in the right side of the body, pain above the waist, and pain below the waist. In addition, axial skeletal pain (cervical spine or anterior chest or thoracic spine or low back) must be present. In this definition, shoulder and buttock pain is considered as pain for each involved side. Low back pain is considered lower segment pain. 2. Pain in 11 of 18 tender point sites on digital palpation. b Definition. Pain, on digital palpation, must be present in at least 11 of the following 18 tender point sites: Occiput: bilateral, at the suboccipital muscle insertions. Low cervical: bilateral, at the anterior aspects of the intertransverse spaces at C5 C7. Trapezius: bilateral, at the midpoint of the upper border. Supraspinatus: bilateral, at origins, above the scapula spine near the medial border. Second rib: bilateral, at the second costochondral junctions, just lateral to the junctions on upper surfaces. Lateral epicondyle: bilateral, 2 cm distal to the epicondyles. Gluteal: bilateral, in upper outer quadrants of buttocks in anterior fold of muscle. Greater trochanter: bilateral, posterior to the trochanteric prominence. Knee: bilateral, at the medial fat pad proximal to the joint line. SOURCE: Adapted from Wolfe F, Smythe HA, Yunus MS, et al. The American College of Rheumatology 1990 criteria for the classification of fibromyalgia. Report of the multicenter criteria committee. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For classification purposes, patients will be said to have fibromyalgia if both criteria are satisfied. Widespread pain must have been present at least 3 months. The presence of a second clinical disorder does not exclude the diagnosis of fibromyalgia. b Digital palpation should be performed with an approximate force of 4 kg. For a tender point to be considered positive the subject must state that the palpation was painful. Tender is not to be considered painful. 669

2 670 APPENDIX I CRITERIA FOR THE CLASSIFICATION OF RHEUMATOID ARTHRITIS a CRITERION DEFINITION 1. Morning stiffness Morning stiffness in and around the joints, lasting at least 1 hour before maximal improvement 2. Arthritis of three or more joint areas At least three joint areas simultaneously have had soft tissue swelling or fluid (not bony overgrowth alone) observed by a physician. The 14 possible areas are right or left PIP, MCP, wrist, elbow, knee, ankle, and MTP joints 3. Arthritis of hand joints At least one area swollen (as defined above) in a wrist, MCP, or PIP joint 4. Symmetric arthritis Simultaneous involvement of the same joint areas (as defined in 2) on both sides of the body (bilateral involvement of PIPs, MCPs, or MTPs is acceptable without absolute symmetry) 5. Rheumatoid nodules Subcutaneous nodules, over bony prominences, or extensor surfaces, or in juxta-articular regions, observed by a physician 6. Serum rheumatoid factor Demonstration of abnormal amounts of serum rheumatoid factor by any method for which the result has been positive in 5% of normal control subjects 7. Radiographic changes Radiographic changes typical of rheumatoid arthritis on posteroanterior hand and wrist radiographs, which must include erosions or unequivocal bony decalcification localized in or most marked adjacent to the involved joints (osteoarthritis changes alone do not qualify) SOURCE: Reprinted from Arnett FC, Edworthy SM, Bloch DA. et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988;31: , with permission of the American College of Rheumatology. ABBREVIATIONS: MCP, metacarpophalangeal; MTP, metatarsophalangeal; PIP, proxomal interphalangeal. a For classification purposes, a patient shall be said to have rheumatoid arthritis if he/she has satisfied at least four of these seven criteria. Criteria 1 through 4 must have been present for at least 6 weeks. Patients with two clinical diagnoses are not excluded. Designation as classic, definite, or probable rheumatoid arthritis is not to be made. CLASSIFICATION OF PROGRESSION OF RHEUMATOID ARTHRITIS Stage I, Early *1. No destructive changes on roentgenographic examination 2. Radiographic evidence of osteoporosis may be present Stage II, Moderate *1. Radiographic evidence of osteoporosis, with or without slight subchondral bone destruction; slight cartilage destruction may be present *2. No joint deformities, although limitation of joint mobility may be present 3. Adjacent muscle atrophy 4. Extra-articular soft tissue lesions, such as nodules and tenosynovitis may be present Stage III, Severe *1. Radiographic evidence of cartilage and bone destruction, in addition to osteoporosis *2. Joint deformity, such as subluxation, ulnar deviation, or hyperextension, without fibrous or bony ankylosis 3. Extensive muscle atrophy 4. Extra-articular soft tissue lesions, such as nodules and tenosynovitis may be present Stage IV, Terminal *1. Fibrous or bony ankylosis 2. Criteria of stage III SOURCE: Reprinted from Steinbrocker O, Traeger CH. Batterman RC. Therapeutic criteria in rheumatoid arthritis. JAMA 1949;140: , with permission. * The criteria prefaced by an asterisk are those that must be present to permit classification of a patient in any particular stage or grade.

3 CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 671 CRITERIA FOR CLINICAL REMISSION IN RHEUMATOID ARTHRITIS a Five or more of the following requirements must be fulfilled for at least 2 consecutive months: 1. Duration of morning stiffness not exceeding 15 minutes 2. No fatigue 3. No joint pain (by history) 4. No joint tenderness or pain on motion 5. No soft tissue swelling in joints or tendon sheaths 6. Erythrocyte sedimentation rate (Westergren method) less than 30 mm/hour for a female or 20 mm/hour for a male SOURCE: Reprinted from Pinals RS, Masi AT, Larsen RA. et al. Preliminary criteria for clinical remission in rheumatoid arthritis. Arthritis Rheum 1981;24: , with permission of the American College of Rheumatology. a These criteria are intended to describe either spontaneous remission or a state of drug-induced disease suppression, which simulates spontaneous remission. No alternative explanation may be invoked to account for the failure to meet a particular requirement. For instance, in the presence of knee pain, which might be related to degenerative arthritis, a point for no joint pain may not be awarded. Exclusions: Clinical manifestations of active vasculitis, pericarditis, pleuritis or myositis, and unexplained recent weight loss or fever attributable to rheumatoid arthritis will prohibit a designation of complete clinical remission. CRITERIA FOR CLASSIFICATION OF FUNCTIONAL STATUS IN RHEUMATOID ARTHRITIS a Class I Class II Class III Class IV Completely able to perform usual activities of daily living (self-care, vocational, and avocational) Able to perform usual self-care and vocational activities, but limited in avocational activities Able to perform usual self-care activities, but limited in vocational and avocational activities Limited in ability to perform usual self-care, vocational, and avocational activities SOURCE: Reprinted from Hochberg MC, Chang RW, Dwosh I. et al. The American College of Rheumatology 1991 revised criteria for the classification of global functional status in rheumatoid arthritis. Arthritis Rheum 1992;35; , with permission of the American College of Rheumatology. a Usual self-care activities include dressing, feeding, bathing, grooming, and toileting. Avocational (recreational and/or leisure) and vocational (work, school, homemaking) activities are patient-desired and age- and sex-specific. AMERICAN COLLEGE OF RHEUMATOLOGY PRELIMINARY DEFINITION OF IMPROVEMENT IN RHEUMATOID ARTHRITIS (ACR20) Required { 20% improvement in tender joint count 20% improvement in swollen joint count + 20% improvement in three of the following five: Patient pain assessment Patient global assessment Physician global assessment Patient self-assessed disability Acute-phase reactant (ESR or CRP) DISEASE ACTIVITY MEASURE METHOD OF ASSESSMENT APPENDIX I 1. Tender joint count ACR tender joint count, an assessment of 28 or more joints. The joint count should be done by scoring several different aspects of tenderness, as assessed by pressure and joint manipulation on physical examination. The information on various types of tenderness should then be collapsed into a single tender-versus-nontender dichotomy. 2. Swollen joint count ACR swollen joint count, an assessment of 28 or more joints. Joints are classified as either swollen or not swollen. 3. Patient s assessment of pain A horizontal visual analog scale (usually 10 cm) or Likert scale assessment of the patient s current level of pain. 4. Patient s global assessment of disease activity The patient s overall assessment of how the arthritis is doing. One acceptable method for determining this is the question from the AIMS instrument: Considering all the ways your arthritis affects you, mark X on the scale for how well you are doing. An anchored, horizontal, visual analog scale (usually 10 cm) should be provided. A Likert scale response is also acceptable. (continued)

4 672 APPENDIX I AMERICAN COLLEGE OF RHEUMATOLOGY PRELIMINARY DEFINITION OF IMPROVEMENT IN RHEUMATOID ARTHRITIS (ACR20) (continued) DISEASE ACTIVITY MEASURE 5. Physician s global assessment of disease activity 6. Patient s assessment of physical function METHOD OF ASSESSMENT A horizontal visual analog scale (usually 10 cm) or Likert scale measure of the physician s assessment of the patient s current disease activity. Any patient self-assessment instrument which has been validated, has reliability, has been proven in RA trials to be sensitive to change, and which measures physical function in RA patients is acceptable. Instruments which have been demonstrated to be sensitive in RA trials include the AIMS, the HAQ, the Quality (or Index) of Well Being, the MHIQ, and the MACTAR. 7. Acute-phase reactant value A Westergren ESR or a CRP level. SOURCE: Reprinted from Felson DT, Anderson JJ, Boers M, et al. American College of Rheumatology preliminary definition of improvement in rheumatoid arthritis. Arthritis Rheum 1995;38: , with permission of the American College of Rheumatology. ABBREVIATIONS: ACR, American College of Rheumatology; AIMS, Arthritis Impact Measurement Scales; CRP, C-reactive protein; ESR, erythrocyte sedimentation rate; HAQ, Health Assessment Questionnaire; MACTAR, McMaster Toronto Arthritis Patient Preference Disability Questionnaire; MHIQ, McMaster Health Index Questionnaire; RA, rheumatoid arthritis. CRITERIA FOR THE CLASSIFICATION OF SPONDYLOARTHROPATHY a Inflammatory spinal pain or Synovitis Asymmetric or Predominantly in the lower limbs and one or more of the following Positive family history Psoriasis Inflammatory bowel disease Urethritis, cervicitis, or acute diarrhea within 1 month before arthritis Buttock pain alternating between right and left gluteal areas Enthesopathy Sacroiliitis VARIABLE Inflammatory spinal pain Synovitis Family history Psoriasis Inflammatory bowel disease Alternating buttock pain Enthesopathy Acute diarrhea DEFINITION History or present symptoms of spinal pain in back, dorsal, or cervical region, with at least four of the following: (a) onset before age 45, (b) insidious onset, (c) improved by exercise, (d) associated with morning stiffness, (e) at least 3 months duration Past or present asymmetric arthritis or arthritis predominantly in the lower limbs Presence in first-degree or second-degree relatives of any of the following: (a) ankylosing spondylitis, (b) psoriasis, (c) acute uveitis, (d) reactive arthritis, (e) inflammatory bowel disease Past or present psoriasis diagnosed by a physician Past or present Crohn s disease or ulcerative colitis diagnosed by a physician and confirmed by radiographic examination or endoscopy Past or present pain alternating between the right and left gluteal regions Past or present spontaneous pain or tenderness at examination of the site of the insertion of the Achilles tendon or plantar fascia Episode of diarrhea occurring within 1 month before arthritis

5 CRITERIA FOR THE CLASSIFICATION OF SPONDYLOARTHROPATHY a (continued) VARIABLE Urethritis Sacroiliitis DEFINITION Nongonococcal urethritis or cervicitis occurring within 1 month before arthritis Bilateral grade 2 4 or unilateral grade 3 4, according to the following radiographic grading system: 0 = normal, 1 = possible, 2 = minimal, 3 = moderate, and 4 = ankylosis SOURCE: Reprinted from Dougados M, Van Der linden S, Juhlin R, et al. The European Spondylarthropathy Study Group preliminary criteria for the classification of spondylarthropathy. Arthritis Rheum 1991;34: , with permission of the American College of Rheumatology. a This classification method yields a sensitivity of 78.4% and a specificity of 89.6%. When radiographic evidence of sacroiliitis was included, the sensitivity improved to 87.0% with a minor decrease in specificity to 86.7%. Definition of the variables used in classification criteria follow. CRITERIA FOR THE DIAGNOSIS OF RHEUMATIC FEVER a MAJOR MANIFESTATIONS MINOR MANIFESTATIONS SUPPORTING EVIDENCE OF PRECEDING STREPTOCOCCAL INFECTION Carditis Clinical findings Positive throat culture or rapid streptococcal antigen test Polyarthritis Arthralgia Elevated or rising streptococcal antibody titer Chorea Erythema marginatum Subcutaneous nodules Fever Laboratory findings Elevated acute phase reactants Erythrocyte sedimentation rate C-reactive protein Prolonged PR interval SOURCE: Reprinted from Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young, American Heart Association: guidelines for the diagnosis of rheumatic fever: Jones criteria, updated JAMA 1992;268: , with permission. a If supported by evidence of preceding group A streptococcal infection, the presence of two major manifestations, or of one major and two minor manifestations indicates a high probability of acute rheumatic fever. CRITERIA FOR THE CLASSIFICATION AND REPORTING OF OSTEOARTHRITIS OF THE HAND, HIP, AND KNEE CLASSIFICATION CRITERIA FOR OSTEOARTHRITIS OF THE HAND, TRADITIONAL FORMAT a Hand pain, aching, or stiffness and Three or four of the following features: Hard tissue enlargement of 2 or more of 10 selected joints Hard tissue enlargement of 2 or more DIP joints Fewer than three swollen MCP joints Deformity of at least 1 of 10 selected joints SOURCE: Reprinted from Altman R, Alarcon G, Appelrouth D, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hand. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. ABBREVIATION: MCP, metacarpophalangeal. a The 10 selected joints are the second and third distal interphalangeal (DIP), the second and third proximal interphalangeal (PIP), and the first carpometacarpal (CMC) joints of both hands. This classification method yields a sensitivity of 94% and a specificity of 87%. APPENDIX I CLASSIFICATION CRITERIA FOR OSTEOARTHRITIS OF THE HIP, TRADITIONAL FORMAT a Hip pain and At least two of the following three features: ESR 20 mm/hour Radiographic femoral or acetabular osteophytes Radiographic joint space narrowing (superior, axial, and/or medial) SOURCE: Reprinted from Altman R, Alarcon G, Appelrouth D, et al. The American College of Rheumatology criteria for the classification and reporting of osteoarthritis of the hip. Arthritis Rheum 1991;34: , with permission of the American College of Rheumatology. ABBREVIATION: ESR, erythrocyte sedimentation rate (Westergren). a This classification method yields a sensitivity of 89% and a specificity of 91%. (continued)

6 674 APPENDIX I CRITERIA FOR THE CLASSIFICATION AND REPORTING OF OSTEOARTHRITIS OF THE HAND, HIP, AND KNEE (continued) CRITERIA FOR CLASSIFICATION OF OSTEOARTHRITIS (OA) OF THE KNEE Clinical and laboratory Knee pain plus at least five of nine: Age >50 years Stiffness <30 minutes Crepitus Bony tenderness Bony enlargement No palpable warmth ESR <40 mm/hour RF <1 : 40 SF OA 92% sensitive 75% specific Clinical and radiographic Knee pain plus at least one of three: Age >50 years Stiffness <30 minutes Crepitus + Osteophytes 91% sensitive 86% specific Clinical a Knee pain plus at least three of six: Age >50 years Stiffness <30 minutes Crepitus Bony tenderness Bony enlargement No palpable warmth 95% sensitive 69% specific SOURCE: Reprinted from Altman R, Asch E, Bloch G, et al. Development of criteria for the classification and reporting of osteoarthritis: classification of osteoarthritis of the knee. Arthritis Rheum 1986;29: , with permission of the American College of Rheumatology. ABBREVIATIONS: ESR, erythrocyte sedimentation rate (Westergren); RF, rheumatoid factor; SF OA, synovial fluid signs of OA (clear, viscous, or white blood cell count <2000/mm 3 ). a Alternative for the clinical category would be four of six, which is 84% sensitive and 89% specific. CRITERIA FOR THE CLASSIFICATION OF ACUTE GOUTY ARTHRITIS A. The presence of characteristic urate crystals in the joint fluid, or B. A tophus proved to contain urate crystals by chemical means or polarized light microscopy or the presence of 6 of the following 12 clinical, laboratory, and x-ray phenomena listed below: 1. More than one attack of acute arthritis 2. Maximal inflammation developed within 1 day 3. Attack of monarticular arthritis 4. Joint redness observed 5. First metatarsophalangeal joint painful or swollen 6. Unilateral attack involving first metatarsophalangeal joint 7. Unilateral attack involving tarsal joint 8. Suspected tophus 9. Hyperuricemia 10. Asymmetric swelling within a joint (radiograph) 11. Subcortical cysts without erosions (radiograph) 12. Negative culture of joint fluid for microorganisms during attack of joint inflammation SOURCE: Adapted from Wallace SL, Robinson H, Masi AT, et al. Preliminary criteria for the classification of the acute arthritis of primary gout. Arthritis Rheum 1977;20: , with permission of the American College of Rheumatology.

7 CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 675 CRITERIA FOR THE CLASSIFICATION OF SYSTEMIC LUPUS ERYTHEMATOSUS a CRITERION DEFINITION 1. Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds 2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions 3. Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation 4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by a physician 5. Arthritis Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion 6. Serositis (a) Pleuritis-convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion OR (b) Pericarditis-documented by ECG or rub or evidence of pericardial effusion 7. Renal disorder (a) Persistent proteinuria greater than 0.5 g per day or greater than 3+ if quantitation not performed OR (b) Cellular casts may be red cell, hemoglobin, granular, tubular, or mixed 8. Neurologic disorder (a) Seizures in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance OR (b) Psychosis in the absence of offending drugs or known metabolic derangements; e.g., uremia, ketoacidosis, or electrolyte imbalance 9. Hematologic disorder (a) Hemolytic anemia with reticulocytosis OR (b) Leukopenia, less than 4000/mm 3 total on two or more occasions OR (c) Lymphopenia, less than 1500/mm 3 on two or more occasions OR (d) Thrombocytopenia, less than l00,000/mm 3 in the absence of offending drugs 10. Immunologic disorder b (a) Anti-DNA: antibody to native DNA in abnormal titer OR (b) Anti-SM: presence of antibody to SM nuclear antigen OR (c) Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test 11. Antinuclear antibody An abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome SOURCE: Adapted from Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus (SLE). Arthritis Rheum 1982;25: , with permission of the American College of Rheumatology. SOURCE: Adapted from Hochberg ME. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus [letter]. Arthritis Rheum 1997;40:1725, with permission of the American College of Rheumatology. a This classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person must have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation. b The modifications to criterion number 10 were made in CRITERIA FOR THE CLASSIFICATION OF SYSTEMIC SCLEROSIS (SCLERODERMA) a A. Major criterion Proximal scleroderma: Symmetric thickening, tightening, and induration of the skin of the fingers and the skin proximal to the metacarpophalangeal or metatarsophalangeal joints. The changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen). APPENDIX I B. Minor criteria 1. Sclerodactyly: Above-indicated skin changes limited to the fingers 2. Digital pitting scars or loss of substance from the finger pad: Depressed areas at tips of fingers or loss of digital pad tissue as a result of ischemia 3. Bibasilar pulmonary fibrosis: Bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenogram; may assume appearance of diffuse mottling or honeycomb lung. These changes should not be attributable to primary lung disease. SOURCE: Adapted from Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 1980;23: , with permission of the American College of Rheumatology. a For the purposes of classifying patients in clinical trials, population surveys, and other studies, a person shall be said to have systemic sclerosis (scleroderma) if the one major or two or more minor criteria are present. Localized forms of scleroderma, eosinophilic fasciitis, and the various forms of pseudoscleroderma are excluded from these criteria.

8 676 APPENDIX I CRITERIA FOR THE DIAGNOSIS OF POLYMYOSITIS AND DERMATOMYOSITIS a CRITERION DEFINITION 1. Symmetrical weakness Weakness of limb-girdle muscles and anterior neck flexors, progressing over weeks to months, with or without dysphagia or respiratory muscle involvement 2. Muscle biopsy evidence Evidence of necrosis of type I and n fibers, phagocytosis, regeneration with basophilia, large vesicular sarcolemmal nuclei and prominent nucleoli, atrophy in a perifascicular distribution, variation in fiber size, and an inflammatory exudate, often perivascular 3. Elevation of muscle enzymes Elevation in serum of skeletal muscle enzymes, particularly creatine phosphokinase and often aldolase, serum glutamate oxaloacetate, and pyruvate transaminases, and lactate dehydrogenase 4. Electromyographic evidence Electromyographic triad of short, small, polyphasic motor units, fibrillations, positive sharp waves, and insertional irritability, and bizarre, high-frequency repetitive discharges 5. Dermatologic features A lilac discoloration of the eyelids (heliotrope) with periorbital edema, a scaly, erythematous dermatitis over the dorsum of the hands (especially the metacarpophalangeal and proximal interphalangeal joints, Gottron s sign), and involvement of the knees, elbows, and medial malleoli, as well as the face, neck, and upper torso Data from Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975;292: , with permission. a Confidence limits can be defined as follows: For a definite diagnosis of dermatomyositis, three of four criteria plus the rash must be present. For a definite diagnosis of polymyositis, four criteria must be present without the rash. For a probable diagnosis of dermatomyositis, two criteria plus the rash must be present. For a probable diagnosis of polymyositis, three criteria must be present without the rash. For a possible diagnosis of dermatomyositis, one criterion plus the rash must be present. For a possible diagnosis of polymyositis, two criteria must be present without the rash. The following findings exclude a diagnosis of dermatomyositis or polymyositis. Evidence of central or peripheral neurologic disease, including motor-neuron disorders with fasciculations or long-tract signs, sensory changes, decreased nerve conduction times, and fiber-type atrophy and grouping on muscle biopsy. Muscle weakness with a slowly progressive, unremitting course and a positive family history or calf enlargement to suggest a muscular dystrophy. Biopsy evidence of granulomatous myositis such as with sarcoidosis. Infections, including trichinosis, schistosomiasis, trypanosomiasis, staphylococcosis, and toxoplasmosis. Recent use of various drugs and toxins, such as clofibrate and alcohol. Rhabdomyolysis as manifested by gross myoglobinuria related to strenuous exercise, infections, crush injuries, occlusions of major limb arteries, prolonged coma or convulsions, high-voltage accidents, heat stroke, the malignant-hyperpyrexia syndrome, and envenomation by certain sea snakes. Metabolic disorders such as McArdle s syndrome. Endocrinopathies such as thyrotoxicosis, myxedema, hyperparathyroidism, hypoparathyroidism, diabetes mellitus, or Cushing s syndrome. Myasthenia gravis with response to cholinergics, sensitivity to d-tubocurarine, and decremental response to repetitive nerve stimulation. CRITERIA FOR THE CLASSIFICATION OF SJÖGREN S SYNDROME a 1. Ocular symptoms Definition. A positive response to at least one of the following three questions: (a) Have you had daily, persistent, troublesome dry eyes for more than 3 months? (b) Do you have a recurrent sensation of sand or gravel in the eyes? (c) Do you use tear substitutes more than three times a day? 2. Oral symptoms Definition. A positive response to at least one of the following three questions: (a) Have you had a daily feeling of dry mouth for more than 3 months? (b) Have you had recurrent or persistently swollen salivary glands as an adult? (c) Do you frequently drink liquids to aid in swallowing dry foods? 3. Ocular signs Definition. Objective evidence of ocular involvement, determined on the basis of a positive result on at least one of the following two tests: (a) Schirmer-I test ( 5 mm in 5 minutes) (b) Rose bengal score ( 4, according to the van Bijsterveld scoring system) 4. Histopathologic features Definition. Focus score 1 on minor salivary gland biopsy (focus defined as an agglomeration of at least 50 mononuclear cells; focus score defined as the number of foci per 4 mm 2 of glandular tissue)

9 CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 677 CRITERIA FOR THE CLASSIFICATION OF SJÖGREN S SYNDROME a (continued) 5. Salivary gland involvement Definition. Objective evidence of salivary gland involvement, determined on the basis of a positive result on at least one of the following three tests: (a) Salivary scintigraphy (b) Parotid sialography (c) Unstimulated salivary flow ( 1.5 ml in 15 minutes) 6. Autoantibodies Definition. Presence of at least one of the following serum autoantibodies: (a) Antibodies to Ro/SS-A or La/SS-B antigens (b) Antinuclear antibodies (c) Rheumatoid factor Exclusion criteria: preexisting lymphoma, acquired immunodeficiency syndrome, sarcoidosis, or graft-versus-host disease SOURCE: Reprinted from Vitali C, Bombardieri S, Moutsopoulos HM, et al. Preliminary criteria for the classification of Sjögren s syndrome. Arthritis Rheum 1993;36: , with permission of the American College of Rheumatology. a For primary Sjögren s syndrome, the presence of three of six items showed a very high sensitivity (99.1%), but insufficient specificity (57.8%). Thus, this combination could be accepted as the basis for a diagnosis of probable primary Sjögren s syndrome. However, the presence of four of six items (accepting as serologic parameters only positive anti Ro/SS-A and anti La/SS-B antibodies) had a good sensitivity (93.5%) and specificity (94.0%), and therefore may be used to establish a definitive diagnosis of primary Sjögren s syndrome. CRITERIA FOR THE CLASSIFICATION OF POLYARTERITIS NODOSA a CRITERION DEFINITION 1. Weight loss 4 kg Loss of 4 kg or more of body weight since illness began, not due to dieting or other factors 2. Livedo reticularis Mottled reticular pattern over the skin of portions of the extremities or torso 3. Testicular pain or tenderness Pain or tenderness of the testicles, not due to infection, trauma, or other causes 4. Myalgias, weakness, or leg Diffuse myalgias (excluding shoulder and hip girdle) or weakness of muscles or tenderness of leg tenderness muscles 5. Mononeuropathy or Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy polyneuropathy 6. Diastolic BP >90 mm Hg Development of hypertension with the diastolic BP higher than 90 mm Hg 7. Elevated BUN or creatinine Elevation of BUN >40 mg/dl or creatinine >1.5 mg/dl, not due to dehydration or obstruction 8. Hepatitis B virus Presence of hepatitis B surface antigen or antibody in serum 9. Arteriographic abnormality Arteriogram showing aneurysms or occlusions of the visceral arteries, not due to arteriosclerosis, fibromuscular dysplasia, or other non-inflammatory causes APPENDIX I 10. Biopsy of small or Histologic changes showing the presence of granulocytes or artery containing medium-sized polymorphonuclear leukocytes and mononuclear leukocytes in the artery wall SOURCE: Reprinted from Lightfoot RW Jr, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. ABBREVIATIONS: BP, blood pressure; BUN, blood urea nitrogen. a For classification purposes, a patient shall be said to have polyarteritis nodosa if at least 3 of these 10 criteria are present. The presence of any three or more criteria yields a sensitivity of 82.2% and a specificity of 86.6%.

10 678 APPENDIX I CRITERIA FOR THE CLASSIFICATION OF HENOCH SCHONLEIN PURPURA a CRITERION DEFINITION 1. Palpable purpura Slightly raised palpable hemorrhagic skin lesions, not related to thrombocytopenia 2. Age at disease onset Patient 20 years or younger at onset of first symptoms 3. Bowel angina Diffuse abdominal pain, worse after meals, or the diagnosis of bowel ischemia, usually including bloody diarrhea 4. Wall granulocytes on biopsy Histologic changes showing granulocytes in the walls of arterioles or venules SOURCE: Reprinted from Mills JA, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Henoch Schonlein purpura. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For purposes of classification, a patient shall be said to have Henoch Schonlein purpura if at least two of these four criteria are present. The presence of any two or more criteria yields a sensitivity of 87.1% and a specificity of 87.7%. CRITERIA FOR THE CLASSIFICATION OF CHURG STRAUSS SYNDROME a CRITERION DEFINITION 1. Asthma History of wheezing or diffuse high-pitched rales on expiration 2. Eosinophilia Eosinophilia >10% on white blood cell differential count 3. Mononeuropathy or polyneuropathy Development of mononeuropathy, multiple mononeuropathies, or polyneuropathy (i.e., glove/stocking distribution) attributable to a systemic vasculitis 4. Pulmonary infiltrates, nonfixed Migratory or transitory pulmonary infiltrates on radiographs (not including fixed infiltrates), attributable to a systemic vasculitis 5. Paranasal sinus abnormality History of acute or chronic paranasal sinus pain or tenderness or radiographic opacification of the paranasal sinuses 6. Extravascular eosinophils Biopsy including artery, arteriole, or venule, showing accumulations of eosinophils in extravascular areas SOURCE: Adapted from Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For classification purposes, a patient shall be said to have Churg Strauss syndrome if at least four of these six criteria are positive. The presence of any four or more of the six criteria yields a sensitivity of 85% and a specificity of 99.7%. CRITERIA FOR THE CLASSIFICATION OF WEGENER S GRANULOMATOSIS a CRITERION DEFINITION 1. Nasal or oral inflammation Development of painful or painless oral ulcers or purulent or bloody nasal discharge 2. Abnormal chest radiograph Chest radiograph showing the presence of nodules, fixed infiltrates, or cavities 3. Urinary sediment Microhematuria (> 5 red blood cells per high power field) or red cell casts in urine sediment 4. Granulomatous inflammation Histologic changes showing granulomatous inflammation within the wall of an artery or in on biopsy the perivascular or extravascular area (artery or arteriole) SOURCE: Reprinted from Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener s granulomatosis. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For purposes of classification, a patient shall be said to have Wegener s granulomatosis if at least two of these four criteria are present. The presence of any two or more criteria yields a sensitivity of 88.2% and a specificity of 92.0%.

11 CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 679 CRITERIA FOR THE CLASSIFICATION OF GIANT CELL ARTERITIS a CRITERION DEFINITION 1. Age at disease onset 50 years Development of symptoms or findings beginning at age 50 or older 2. New headache New onset of or new type of localized pain in the head 3. Temporal artery abnormality Temporal artery tenderness to palpation or decreased pulsation, unrelated to arteriosclerosis of cervical arteries 4. Elevated erythrocyte sedimentation rate Erythrocyte sedimentation rate 50 mm/hour by the Westergren method 5. Abnormal artery biopsy Biopsy specimen with artery showing vasculitis characterized by a predominance of mononuclear cell infiltration or granulomatous inflammation, usually with multinucleated giant cells SOURCE: Reprinted from Hunder GG, Bloch DA, Michel BA, et al. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For purposes of classification, a patient shall be said to have giant cell (temporal) arteritis if at least three of these five criteria are present. The presence of any three or more criteria yields a sensitivity of 93.5% and a specificity of 91.2%. CRITERIA FOR THE CLASSIFICATION OF TAKAYASU ARTERITIS a CRITERION DEFINITION 1. Age at disease onset 40 years Development of symptoms or findings related to Takayasu arteritis at age 40 years 2. Claudication of extremities Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities 3. Decreased brachial artery pulse Decreased pulsation of one or both brachial arteries 4. BP difference >10 mm Hg Difference of >10 mm Hg in systolic blood pressure between arms 5. Bruit over subclavian arteries or aorta Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta 6. Arteriogram abnormality Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or similar causes; changes usually focal or segmental SOURCE: Reprinted from Arend WP, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. ABBREVIATIONS: BP, blood pressure (systolic; difference between arms). a For purposes of classification, a patient shall be said to have Takayasu arteritis if at least three of these six criteria are present. The presence of any three or more criteria yields a sensitivity of 90.5% and a specificity of 97.8%. APPENDIX I CRITERIA FOR THE CLASSIFICATION OF HYPERSENSITIVITY VASCULITIS. a CRITERION DEFINITION 1. Age at disease onset >16 years Development of symptoms after age Medication at disease onset Medication was taken at the onset of symptoms that may have been a precipitating factor 3. Palpable purpura Slightly elevated purpuric rash over one or more areas of the skin; does not blanch with pressure and is not related to thrombocytopenia 4. Maculopapular rash Flat and raised lesions of various sizes over one or more areas of the skin 5. Biopsy including arteriole and venule Histologic changes showing granulocytes in a perivascular or extravascular location SOURCE: Reprinted from Calabrese LH, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of hypersensitivity vasculitis. Arthritis Rheum 1990;33: , with permission of the American College of Rheumatology. a For purposes of classification, a patient shall be said to have hypersensitivity vasculitis if at least three of these five criteria are present. The presence of any three or more criteria yields a sensitivity of 71.0% and a specificity of 83.9%.

12 DIAGNOSTIC GUIDELINES FOR KAWASAKI SYNDROME. a 1. Fever lasting >5 days: Plus four of the following criteria: 2. Polymorphous rash 3. Bilateral conjunctival injection 4. One or more of the following mucous membrane changes: Diffuse injection of oral and pharyngeal mucosa Erythema or fissuring of the lips Strawberry tongue 5. Acute, nonpurulent cervical lymphadenopathy (one lymph node must be >1.5 cm) 6. One or more of the following extremity changes: Erythema of palms and/or soles Indurative edema of hands and/or feet Membranous desquamation of the fingertips SOURCE: Reprinted from Kawasaki T, Kosaki T, Okawa S, et al. A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan. Pediatrics 1974;54: , with permission. a Other illnesses with similar clinical signs must be excluded. CRITERIA FOR THE DIAGNOSIS OF BEHÇET S DISEASE. a CRITERION DEFINITION 1. Recurrent oral ulceration Minor aphthous, major aphthous, or herpetiform ulceration observed by physician or patient, which recurred at least three times in one 12-month period Plus two of 2. Recurrent genital ulceration Aphthous ulceration or scarring, observed by physician or patient 3. Eye lesions Anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis observed by ophthalmologist 4. Skin lesions Erythema nodosum observed by physician or patient, pseudofolliculitis, or papulopustular lesions; or acneiform nodules observed by physician in postadolescent patients not on corticosteroid treatment 5. Positive pathergy test Read by physician at hours SOURCE: Reprinted from International Study Group for Behçet s Disease. Criteria for diagnosis of Behçet s disease. Lancet 1990;335: , with permission. a Findings applicable only in the absence of other clinical explanations. The presence of recurrent oral ulceration and any two of the remaining criteria yields a sensitivity of 91% and a specificity of 96%. PRELIMINARY CLASSIFICATION CRITERIA FOR ANTIPHOSPHOLIPID SYNDROME. a Vascular thrombosis (a) One or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ and (b) Thrombosis confirmed by imaging or Doppler studies or histopathology, with the exception of superficial venous thrombosis and (c) For histopathologic confirmation, thrombosis present without significant evidence of inflammation in the vessel wall. Pregnancy morbidity (a) One or more unexplained deaths of a morphologically normal fetus at or beyond the 10th week of gestation, with normal fetal morphology documented by ultrasound or by direct examination of the fetus or (b) One or more premature births of a morphologically normal neonate at or before the 34th week of gestation because of severe pre-eclampsia or severe placental insufficiency or (c) Three or more unexplained consecutive spontaneous abortions before the 10th week of gestation, with maternal anatomic or hormonal abnormalities and paternal and maternal chromosomal causes excluded. Laboratory criteria (a) Anticardiolipin antibody of IgG and/or IgM isotype in blood, present in medium or high titer on two or more occasions at least 6 weeks apart, measured by standard enzyme-linked immunosorbent assay for beta 2 glycoprotein l dependent anticardiolipin antibodies or (b) Lupus anticoagulant present in plasma on two or more occasions at least 6 weeks apart, detected according to the guidelines of the International Society on Thrombosis and Hemostasis. SOURCE: Adapted from Wilson WA, Gharavi AE. Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Report of an International Workshop. Arthritis Rheum 1999;42: with permission of the American College of Rheumatology. a Definite APS is considered to be present if at least one of the clinical and one of the laboratory criteria are met.

13 CRITERIA FOR THE CLASSIFICATION AND DIAGNOSIS 681 WORLD HEALTH ORGANIZATION CRITERIA FOR THE DIAGNOSIS OF OSTEOPENIA AND OSTEOPOROSIS. Normal BMC or BMD not more than 1 standard deviation below peak adult bone mass T score > 1 Osteopenia Osteoporosis Severe Osteoporosis BMC or BMD that lies between 1 and 2.5 standard deviations below peak adult bone mass T score between 1 and 2.5 BMC or BMD value more than 2.5 standard deviations below peak adult bone mass T score 2.5 BMC or BMD value more than 2.5 standard deviations below peak adult bone mass and the presence of one or more fragility fractures T score 2.5 plus fragility fracture SOURCE: Adapted from Assessment of fracture risk and its application to screening for postmenopausal osteoporosis. Report of a WHO study group. World Health Organ Techn Rep Ser 1994;843: a World Health Organization criteria for the diagnosis of osteoporosis based on bone mineral content (BMC) or bone mineral density (BMD) measurements. These criteria can be applied to either the central or peripheral skeletal measurement sites. CRITERIA FOR THE DIAGNOSIS OF JUVENILE RHEUMATOID ARTHRITIS (JRA). I. General The JRA Criteria Subcommittee in 1982 reviewed the 1977 Criteria (1) and recommended that juvenile rheumatoid arthritis be the name for the principal form of chronic arthritic disease in children and that this general class should be classified into three onset subtypes: systemic, polyarticular, and pauciarticular. The onset subtypes may be further subclassified into subsets as indicated below. The following classification enumerates the requirements for the diagnosis of JRA and the three clinical onset subtypes and lists subsets of each subtype that may be useful in further classification. II. General criteria for the diagnosis of juvenile rheumatoid arthritis A. Persistent arthritis of at least 6 weeks duration in one or more joints B. Exclusion of other causes of arthritis (see list of exclusions) III. JRA onset subtypes The onset subtype is determined by manifestations during the first 6 months of disease and remains the principal classification, although manifestations more closely resembling another subtype may appear later. A. Systemic onset JRA: This subtype is defined as JRA with persistent intermittent fever (daily intermittent temperatures to 103 F or more) with or without rheumatoid rash or other organ involvement. Typical fever and rash will be considered probable systemic onset JRA if not associated with arthritis. Before a definite diagnosis can be made, arthritis, as defined, must be present. B. Pauciarticular onset JRA: This subtype is defined as JRA with arthritis in four or fewer joints during the first 6 months of disease. Patients with systemic onset JRA are excluded from this onset subtype. C. Polyarticular JRA: This subtype is defined as JRA with arthritis in five or more joints during the first 6 months of disease. Patients with systemic JRA onset are excluded from this subtype. D. The onset subtypes may include the following subsets: 1. Systemic onset a. Polyarthritis b. Oligoarthritis 2. Oligoarthritis (pauciarticular onset) a. Antinuclear antibody (ANA) positive chronic uveitis b. Rheumatoid factor (RF) positive c. Seronegative, B27 positive d. Not otherwise classified 3. Polyarthritis a. RF positivity b. Not otherwise classified APPENDIX I IV. Exclusions A. Other rheumatic diseases 1. Rheumatic fever 2. Systemic lupus erythematosus 3. Ankylosing spondylitis 4. Polymyositis or dermatomyositis 5. Vasculitic syndromes 6. Scleroderma 7. Psoriatic arthritis 8. Reiter s syndrome 9. Sjögren s syndrome 10. Mixed connective tissue disease 11. Behçet s syndrome (continued)

14 682 APPENDIX I CRITERIA FOR THE DIAGNOSIS OF JUVENILE RHEUMATOID ARTHRITIS (JRA). (continued) IV. Exclusions B. Infectious arthritis C. Inflammatory bowel disease D. Neoplastic diseases including leukemia E. Nonrheumatic conditions of bones and joints F. Hematologic diseases G. Psychogenic arthralgia H. Miscellaneous 1. Sarcoidosis 2. Hypertrophic osteoarthropathy 3. Villonodular synovitis 4. Chronic active hepatitis 5. Familial Mediterranean fever V. Other proposed terminology Juvenile chronic arthritis (JCA) and juvenile arthritis (JA) are new diagnostic terms currently in use in some places for the arthritides of childhood. The diagnoses of JCA and JA are not equivalent to each other, nor to the older diagnosis of juvenile rheumatoid arthritis or Still s disease. Hence reports of studies of JCA or JA cannot be directly compared with one another nor to reports of JRA or Still s disease. Juvenile chronic arthritis is described in more detail in a report of the European Conference on the Rheumatic Diseases of Children (2) and juvenile arthritis in the report of the Ross Conference (3). 1. JRA Criteria Subcommittee of the Diagnostic and Therapeutic Criteria Committee of the American Rheumatism Association. Current proposed revisions of the JRA criteria. Arthritis Rheum 1977;20(Suppl): Ansell BW. Chronic arthritis in childhood. Ann Rheum Dis 1978;37: Fink CW. Keynote address: Arthritis in childhood. Report of the 80th Ross Conference in Pediatric Research. Columbus, OH: Ross Laboratories; 1979:1 2.