Rheumatology Objec1ves. Rheumatology Objec1ves By the end of this presenta1on, the a<endee should be able to: 5/5/11
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1 April 29, 2011 Presented by: Elizabeth M. Etherton, MSN, ACNP- BC, CNP Rheumatology Specialty Clinic University of New Mexico Health Science Center Rheumatology Objec1ves By the end of this presenta1on, the a<endee should be able to: Review the most common rheumatologic diagnoses including rheumatoid arthritis, systemic lupus erythematosus, gout and vasculitis along with common signs and symptoms. Differentiate between emergent, urgent and routine problems common among these rheumatologic diagnoses. Review when to treat, how to treat and when to refer to a different level of care. Rheumatology Objec1ves Cont d Discuss appropriate use of NSAIDS, DMARDS and biologic medications in rheumatology patients as well as appropriate monitoring techniques, immunizations, routine labs. Review the latest American College of Rheumatology (ACR) diagnostic criteria and treatment guidelines for fibromyalgia. 1
2 Famous People Who are they and what rheumatologic diseases did they have? Who is this man??????? 2
3 Rheumatoid Arthri1s Definition chronic inflammatory disease that causes pain, stiffness, swelling and limitations in motion and function of multiple joints. Incidence 0.2/1000 in males and 0.4/1000 in females. Age of onset Etiology Exact etiology not understood. RA is an autoimmune condition that develops due to immune system malfunction. Results in inflammation and destruction of articular and extra- articular structures. Rheumatoid Arthri1s Diagnostic Criteria Must include 4 criteria: Morning stiffness > 1 hour Polyarthritis ( 3 or more joints- lasting > 12 weeks) Involvement of hands (MCP and PIP) Symmetry Rheumatoid Factor Anti- citrullinated protein antibodies (anti- ccp) Rheumatoid Nodules Radiographic changes Rheumatoid Arthri1s Subjective joint pain, morning stiffness, swelling, weakness and instability. Objective Joint deformity, effusion, redness, heat and crepitus. Diagnostic Studies Lab + RF, +HLA- DR4, increased sedimentation rate and C Reactive Protein, H/H, thrombocytopenia, elevated liver enzymes, + urine protein Pulmonary impaired PFTs Radiographic soft tissue swelling, peri articular osteoporosis, narrowed or obliterated joint spaces and erosions. 3
4 Rheumatoid Arthri1s Rheumatoid Arthri1s Differential Diagnoses Reactive arthritis SLE Psoriatic arthritis Sarcoidosis Fibromyalgia Sjogren s Syndrome Septic arthritis Spondylarthropathies Osteoarthritis Rheumatoid Arthri1s Management Goal: Early initiation of treatment to reduce or prevent progression and damage. Pain control. Improved function and quality of life. Diagnostic tests CRP, ESR, CBC with diff, UA, LFTs, Chem 7, RF, Hepatitis Panel, anti- CCP Other tests to rule in or out other dz ANA, anti- Smith, anti- RNP, SSA, SSB, C3 and C4, APA, Uric acid, ANCA, CK, Lyme, HLA- B27 4
5 Rheumatoid Arthri1s Radiology bilateral hands, feet, chest x- ray TB skin test History and Exam Onset, progression, mitigating factors Review of systems Past medical, surgical, family and social history. History of blood transfusions, medication use as well as illicit drug use. Physical Exam GALS + HEENT + cardiopulmonary G gait A arms L legs S spine Assess for joint swelling, redness, heat, deformity, ROM, pain on active and passive movement. Rheumatoid Arthri1s Plan Initiate therapy with DMARD at the appropriate lowest dose. Methotrexate with folic acid Hydroxychloroquine - antimalarial Leflunomide inhibits pyrimidine synthesis Biologics TNF inhibitor may need to fail 2 DMARDS before insurance will cover May initiate prednisone for ACUTE relief of symptoms until DMARD takes effect in 8 or more weeks. Avoid narcotic use. Tylenol or short- term NSAID use is OK. Rheumatoid Arthri1s Order appropriate labs/diagnostics for monitoring of therapy. Bone density DEXA initiate bisphosphonate if appropriate CBC, diff, AST, ALT, CRP, ESR with Methotrexate or leflunomide, biologics Eye exam with CBC, diff every 6 months on Hydroxychloroquine PPD every year for MTX, TNF inhibitors RTC every 2-4 months depending on symptom control. 5
6 Rheumatoid Arthri1s When to initiate MD consult or referral: Significantly abnormal lab work Red, hot or very swollen joints Non- healing sores or wounds Acute SOB, chest pain or fever Significant medication side effects Patient planning surgery Treatment failure Art and Science Which famous painter had a severe case of RA beginning in 1890? Systemic Lupus Erythematosus Definition inflammatory autoimmune disease affecting multiple organs. Incidence 1.6 to 7.6/100,000. Female/male 12:1 in childbearing age range and 2:1 in the overall affected population Etiology Genetic predisposition mediated by hormonal factors, autoimmune defects and environmental factors such as viruses, allergens, silica dust, cigarette smoking and UV light. Diagnostic Criteria ACR Criteria update of 1982 revised criteria Subjective Fatigue, malaise, subjective fever, joint pain, headache, complaints of lesions, ulcerations or rashes, esp. photosensitive Objective Synovitis, lymphadenopathy, myositis, ocular inflammation, cutaneous lesions, rashes, cognitive impairment, weight loss, anemia, proteinuria 6
7 SLE Causes? Systemic Lupus Erythematosus Diagnostic Studies Labs - + ANA is hallmark. Anti- ds DNA, Anti- RNP, Anti- Smith can also be positive. C3 and C4 may be low. May have + antiphospholipid antibodies Radiological x- rays may show soft tissue swelling, but typically do not show erosive type of arthritis renal ultrasound assess kidney size or obstruction if nephritis suspected Echocardiography if suspect pericardial involvement MRI if suspicious of other organ involvement or neurologic symptoms Differential Diagnoses - multiple SLE The Great Imitator 7
8 Systemic Lupus Erythematosus Management goals- Early initiation of treatment to reduce or prevent progression and damage Pain control Improved function and quality of life Diagnostics Labs same as for RA Radiology x- rays affected joints and chest for interstitial disease CT/MRI as appropriate Renal biopsy for diagnosis of nephritis Systemic Lupus Erythematosus History and Exam same as with RA Plan: Initiate therapy appropriate for diagnosis and condition. Usually a DMARD, hydroxychloroquine at appropriate dose. May initiate prednisone for acute relief of symptoms until DMARD takes effect. May also use MTX, azathioprine, mycophenolate mofetil, cyclophosphamide. These patients will be followed by the MD. TNF inhibitors are contraindicated. Systemic Lupus Erythematosus Plan cont d Narcotics should be avoided. Tylenol or short term NSAIDs as appropriate. Order appropriate lbs/diagnostics for monitoring of therapy and disease activity. Return visits in 2 to 6 months depending on stability. Initiate MD consult or referral for Pregnancy Significantly abnormal labs esp. proteinuria Significant medication side effects Same as for RA 8
9 Lupus Celebrity Gout Definition disease by which tissue deposition of monosodium urate crystals occurs as a result of hyperuricemia supersaturation of extracellular fluid. Incidence increases with age. Rare in men under 30 and premenopausal women. Prevalence is 5-28/1000 in men and 1-6/1000 in women. Male to female ratio is 2-7:1 Etiology Develops as overproduction of urate or under excretion of urate. May be a combination of both. Diagnostic Criteria should be based on analysis of synovial fluid indicating crystals. Subjective ABRUPT onset of painful, red, swollen and warm joint. Objective Erythema, effusion and heat of affected joint. May have evidence of desquamation post swelling. Gout Diagnostic Studies Lab Synovial fluid needle shaped intra or extracellular crystals, WBCs 20,000 to 100,000 with predominance of neutrophils immunoflourescent crystals Uric Acid 6-7 Differential Diagnoses Radiographic soft tissue swelling; bony erosions (punched out); tophi. Osteopenia is absent. Joint space is preserved until late in disease. 9
10 Gout Gout Management Goals Symptoms relief Restore function Prevent joint damage Testing Synovial fluid biopsy Uric acid level Autoimmune panel to rule out other conditions Gout H&P same with evidence of effusion perform joint aspiration. Plan Medications Acute attack NSAIDS indomethacin drug of choice 50mg qid x hours. Taper as attack subsides. Colchicine 0.6mg q hour until symptoms ease. (max 6 mg/ 24 hrs) Prednisone 30-50mg taper over 7-10 days Local steroid joint injections. 10
11 Gout Prevention urate lowering agents Allopurinol- 100mg daily increasing to 300 to 600mg daily Febuxostat start with 40mg daily may increase to 80mg DO NOT stop during a flare May flare upon initiation of treatment Famous Gout Sufferers Vasculi1des Definition - presences of leukocytes in the vessel wall with reactive damage to mural structures. Classifications Vasculitic syndromes are categorized by the predominant sizes of blood vessels and types of vessels most commonly affected among patient with the disorder. 11
12 Vasculi1des Large vessel vasculitis Takayasu arteritis Giant cell arteritis Medium sized vessel vasculitis Polyarteritis nodosa Kawasaki disease Isolated central nervous system vasculitis Small vessel vasculitis Churg- Strauss arteritis Wegener s granulomatosis Vasculi1des Small vessel vasculitis Microscopic polyarteritis Henoch- Schönlein purpura Essential cryoglobulinemic vasculitis Hypersensitivity vasculitis Vasculitis secondary to connective tissue disorders Vasculitis secondary to viral infections Vasculi1des Etiology Exact mechanisms underlying these disorders are unclear. Diagnostic criteria vary according to age, presentation, s/s, labs, biopsy results. Clinical features suggestive of vasculitis: Mononeuritis multiplex asymmetric polyneuropathy Palpable purpura Pulmonary- renal involvement 12
13 Vasculi1des Labs ANA Complement Cryoglobulinemia ANCA Biopsy Imaging Arteriograms Vasculi1des Plan Management by MD Therapy depends upon nature and severity of the vasculitis Initiate appropriate medications Initial daily cyclophosphamide and corticosteroids Maintenance Methotrexate, Azathioprine Alternative therapy Mycophenolate Mofetil, Bactrim, Cyclosporine, Etanercept Resistant and relapsing disease Mycophenolate Mofetil, anti- TNF alpha therapy, Rituximab, Anti- t cell antibodies, IVIG Vasculi1des Palpable purpura Livedo reticularis 13
14 Other Rheumatologic Diseases Psoriatic arthritis Ankylosing spondylitis Reactive arthritis Systemic scleroderma Inflammatory Bowel disease arthropathy Sjogren s syndrome Reynaud s phenomenon Pa1ent Educa1on Emphasize the importance of physical activity, healthy lifestyle. Encourage adequate calcium and Vitamin D intake. Encourage smoking cessation. With DMARD or TNF inhibitors, review signs of infection. Patient should stop medications in the event of serious infection ie. pneumonia, zoster. DC at least one week before surgery and restart after sutures out, or according to surgeon. Encourage routine flu and pneumonia vaccines. NO live vaccines Zoster/Shingles, Rubella. With NSAIDs, review risk of PUD/GERD. If childbearing age on methotrexate, must use double birth control Category X SEVERELY TERATOGENIC Fibromyalgia Definition: Chronic widespread muscular pain and symptoms such as fatigue, sleep disturbances, stiffness, cognitive and memory problems, and symptoms of depression or anxiety. More localized pain conditions often occur in patients with fibromyalgia migraine or tension headaches, TMJ, IBS, GERD, irritable bladder, pelvic pain syndromes. Symptoms can wax and wane over time. Stress often worsens these symptoms. 14
15 Fibromyalgia Causes? Certain types of genes predispose Some triggering factor spine disorders, arthritis, trauma or other triggering physical stressors Emotional stressors may be trigger Results in changes in the way the body communicates with the spinal cord and brain associated with altered levels of chemicals and proteins in the CNS. Allodynia, hyperanalgesia, sleep disturbances. Fibromyalgia Fast Facts Affects 2-4% of the population, predominantly women Diagnosed based on patient symptoms and physical examination. No lab, rad or other test. May be used to exclude other conditions. Medication is not the only treatment. Patient education, exercise, self- management skills and alternative therapies help treat symptoms. ACR Classifica1on of Fibromyalgia 2010 update to original 199o guidelines Tender point count has been replaced Widespread Pain Index Symptom Severity Other symptoms - fatigue, waking unrefreshed and cognitive symptoms Exclude other disorders causing pain symptoms selbsthilfe- rmk.info/resources/ NewFibroCriteriaSurvey2010.pdf 15
16 FIBRO pneumonic F fatigue, fibrofog I insomnia B blues- anxiety, depression R rigidity - stiffness O OW!!!, work difficulties Fibromyalgia Therapy Lifestyle Changes ADLs, maintenance of household chores Healthy lifestyle Good sleep hygiene Alternative methods yoga, Tai Chi Medications affect seratonin and norepinephrine duloxetine, milnacipran, venlafaxine Amitriptyline, cyclobenzaprine, fluoxetine, paroxetine or setraline. Side effects related to their specific formulation. Fibromyalgia Medications (cont d) Pregabalin and gabapentin work by blocking the over activity of nerve cells involved in pain transmission. Side effects include dizziness, sleepiness, swelling and weight gain. Opioids are not recommended Not because of dependence issues anecdotal evidence suggest may cause greater pain sensitivity or persistence of chronic pain. Useful to treat sleep problems with medications that improve sleep, but can also treat pain. 16
17 Fibromyalgia Other therapies such as yoga, therapeutic massage, acupuncture and other body- based therapies should be recommended as being helpful to relieve muscle spasms and stiffness. Self- management is integral to a meaningful improvement in symptoms and daily function. Case Studies Rheumatoid Arthritis Systemic Lupus Erythematosus Gout Fibromyalgia RA Case Study Anita, aged 62, has a one year history of bilateral hand pain characterized by morning stiffness lasting > 2 hours. She also reports that over the last 3 months, she is having widespread symmetrical joint swelling (hands, wrists, feet, elbows, knees and shoulders) 17
18 RA She has been treating herself with OTC ibuprofen with diminishing relief. She arrives at your primary care office in Littletown, NM requesting pain medication. Anita states she is also feeling run down, with little energy. She also reports swollen glands in her neck and a 10 pound weight loss over the past year. RA Physical exam reveals bilateral hand swelling with synovitis of MCP (metocarpalphalangeal joints) bilaterally. You order labs to include CBC, diff, AST, ALT, Creatinine, ESR, C reactive protein, anti CCP antibodies. To rule out other causes of arthritis, what other blood tests would you order? What x- rays would you order? RA CBC reveals WBC 8.4, Hgb 12.9, plt 449. ESR 24, CRP 2.8, Cr 1.0 RF 22, anti- CCP is positive ANA + - 1:40 dsdna-, antism -, SS- A -, SS- b Radiology of hands and feet show periarticular osteoporosis with juxta- articular erosions 18
19 RA Diagnosis made! Rheumatoid arthritis. Rapidly progressing. Treatment plan? TB skin test Initiate DMARD and prednisone taper Consider methotrexate initially. If pain and swelling continue after 3 months of treatment, may add another DMARD or add an TNF- alpha inhibitor such as adalimumab or etanercept Monitor quarterly CNBC, diff, AST, ALT, Cr, ESR, CRP SLE Amber, a 22 year old college student is being followed by Rheumatology at the University Hospital. You have been her primary care provider ever since she turned 18 and her pediatrician fired her. She presents today with complaints of not feeling well. She c/o s of swollen glands, a low grade fever, and a severe headache unrelieved with acetaminophen. She states that she is having trouble concentrating on her studies. SLE Her current regimen for lupus includes hydroxychloroquine 200mg po bid. She is on very low dose prednisone at 2.5mg daily. She is on low dose BCP. Stat labs reveal: elevated ESR, normochromic normocytic anemia with a WBC of 3.0. Normal WBC for her is What do you think? Remember that lupus is the great imitator. 19
20 SLE Approximately 2/3 s of patients with SLE have neuropsychiatric manifestations. The lupus headache has been operationally defined as severe, disabling, and persistent. It is not responsive to narcotic analgesics. This would be a very appropriate urgent referral to the rheumatologist, as it can progress to seizures or an acute confusional state, in the past simply referred to as delusional. Think about increasing prednisone. Gout Jerry, a 55 year old, auto mechanic, presents in your office as an acute visit. Complaints include a sudden onset of swollen great toe which is causing him excruciating pain. Onset was yesterday, and he has been soaking his foot in Epsom salts. He has never had anything like this before. He states that he has been taking anything he can get his hands on to ease the pain. Past history includes excessive alcohol intake, high cholesterol for which he refuses medication, HTN and morbid obesity. He is borderline diabetic. Gout On exam, you note a swollen, red, warm great toe. You put a needle into the joint and aspirate some fluid and send it for analysis. WBC 15,000, predominantly neutrophils. You send the fluid for culture and sensitivity. Uric acid level is
21 5/5/11 Gout Initial treatment? If he has repeated gout attacks over the next year, what should you consider? Educational issues? ETOH Weight Diet Drugs to avoid. Fibromyalgia Case Study
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