ARTHRITIS RADIOGRAPHIC ABNORMALITIES OF RHEUMATOID ARTHRITIS IN PATIENTS WITH DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSTS

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ARTHRITIS 82 RHEUMATISM OFFICIAL JOURNAL OF THE AMERICAN RHEUMATISM ASSOCiA I ION SECTION OF THE ARTHRITIS FOUNDATION 1 RADIOGRAPHIC ABNORMALITIES OF RHEUMATOID ARTHRITIS IN PATIENTS WITH DIFFUSE

1 ARTHRITIS 82 RHEUMATISM OFFICIAL JOURNAL OF THE AMERICAN RHEUMATISM ASSOCiA I ION SECTION OF THE ARTHRITIS FOUNDATION 1 RADIOGRAPHIC ABNORMALITIES OF RHEUMATOID ARTHRITIS IN PATIENTS WITH DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSTS DONALD RESNICK, JOHN CURD, ROBERT F. SHAPIRO. and KENNETH B. WIESNER Radiographic abnormalities of rheumatoid arthritis (RA) in 8 patients with diffuse idiopathic skeletal hyperostosis (DISH) included atypical features: lack of osteoporosis, bone sclerosis and proliferation about erosions, osteophytosis, and bony ankylosis. Atypical clinical features included a high incidence of flexion contractures of elbows, wrists, ankles, or knees. It is not surprising that bone production occurs about involved articulations in patients with RAoDISH, as the latter disorder is characterized by bony proliferation at sites of ligament and tendon attachment to bone in the axial and extraaxial skeleton, perhaps related to stress. The radiographic abnormalities of bone and soft tissue in rheumatoid arthritis (RA) are well known. Typically, RA manifests itself radiographically as sym From the Departments of Radiology (DR) and Internal Medicine (JC), Veterans Administration Hospital, San Diego. California. and the University Hospital. University of California, San Diego. and Sacramento Rheumatology Consultants (RFS. KBW). Sacramento, Calinornia. Donald Resnick, M.D.: Picker Scholar, Picker Foundation, Chief. Department of Radiology, Veterans Administration Hospital and Associate Professor of Radiology, University of California. San Diego; John Curd, M.D.: Helen Hay Whitney Fellow in the R heumatology Division, University of California, San Diego; Robert F. Shapiro. M.D., Kenneth B. Wiesner, M.D.: 2222 Sierra Blvd.. Suite E26, Sacramento, California Address reprint requests to Donald Resnick. M.D., Department of Radiology, Veterans Administration Hospital La Jolla Village Drive, San Diego, California Submitted for publication June 20, 1977; accepted in revised form August 8, metrical involvement of joints particularly in the hands, wrists, and feet. The involvement characteristic of RA includes regional or periarticular osteoporosis, early symmetrical articular space loss, and marginal and interior joint erosions. These features are particularly evident in women with RA: however, they may be somewhat modified in men with the disease (1,2). One modified pattern is the robust type of RA (3). In these patients, continued or exaggerated physical activity has been hypothesized to lead to elevated intraarticular pressure and accelerated pannus formation (46). These patients may also have asymmetrical involvement of the dominant side, characterized by relative preservation of joint space, discrete punched out erosions, and lack of osteoporosis. The appearance may simulate gout. In this report we describe 8 patients with clinical RA who also manifested the characteristic findings of diffuse idiopathic skeletal hyperostosis (DISH). DISH (7,8) is a common ossifying diathesis occurring in middleaged and elderly patients resulting in proliferative bone abnormalities at sites of ligament and tendon attachment to bone. The patients described in this report demonstrated another modified pattern of articular abnormality in RA in that they had some roentgen features that were atypical for RA and typical for DISH. These features included the absence of osteoporosis, and the presence of bone eburnation and proliferation about osseous erosions, osteophytosis, and intraarticular bony ankylosis. This report summarizes our observations in these patients with RA DISH. Arthritis and Rheumatism, Vol. 21, No. 1 (JanuaryFebruary 1978)

2 2 RESNICK ET AL MATERIALS AND METHODS The records and radiographs of eight patients recently seen with RA DISH were reviewed. All patients had been evaluated by rheurnatologists for polyarthritis with complete history and physical examinations. In the course of their evaluation and treatment, roentgenograms of all peripheral joints and the entire spine were obtained. All eight patients met the American Rheumatism Association criteria for rheumatoid arthritis and the radiographic criteria for DISH (8). These latter criteria include: I. The presence of flowing calcification and ossification along the anterolateral aspects of at least four contiguous vertebral bodies with or without associated localized pointed excrescences at the intervening vertebral bodydisc junctions. 2. A relative preservation of disc height in the involved areas and the absence of extensive radiographic changes of degenerative disc disease, including vacuum phenomena and vertebral body marginal sclerosis. 3. Absence of apophyseal joint bony ankylosis and sacroiliac joint erosion, sclerosis, or bony fusion. RESULTS A summary of the clinical and laboratory findings observed in these patients is presented in Table I. Notably, all patients except 1 were men. This male predominence may reflect the patient population seen at the Veterans Administration Hospital. The patients averaged 65 years of age (range: 5578 years) and had a history of longstanding arthritis (duration: 540 years). The initial impression of the rheumatologist evaluating the patient was definite RA in 6 of the 8 patients, probable RA in I patient. and possible RA in 1 patient. On initial physical examination all patients except 1 were thought to have active synovitis varying from mild to severe in degree: subsequently, all patients developed synovitis in one or more joints. Four patients had subcutaneous nodules, 2 had hypergarnmaglobulinemia, 1 had Sjogren s syndrome, and 1 had interstitial lung disease. All were seropositive (latex fixation), although the titer Table 1. Summary of Clinical Findings Duration Severe of Rheumatoid Sub Restriction Age Disease Factor ES R cutaneous of Active Associated Patient Sex (years) (years) (titer) ANA (mm/hr) Anemia Nodules Vasculitis Motion Synovitis Disorders WG M 64 5 RA M ER M FG M HL M WN M 62 6 ES M CT F ( I /640) ( I /40) ( I /320) (I /40) (I /go) (l/5 120) (I / 1280) * = mild: = moderate: = severe t 33 R elbow L wrist None Elbows R knee Elbows Wrists Subtalars Elbows Wrists Elbows Elbows Ankles K nees None * Lung disease Psoriasis Hypergammaglobulins Hypergammaglobulins Pleural effusions CH F CHF Tendinitis

RADIOGRAPHIC ABNORMALITIES IN RA PATIENTS 3 ditionally had RA. In all 8 patients the diagnosis of DISH was established radiographically, usually to the surprise of the attending rheumatologist.

A feature commonly noted on physical examination was decreased range of motion and/or flexion contractures of large joints.

3 RADIOGRAPHIC ABNORMALITIES IN RA PATIENTS 3 ditionally had RA. In all 8 patients the diagnosis of DISH was established radiographically, usually to the surprise of the attending rheumatologist. Only 1 patient had symptoms referable to the axial skeleton and his cervical spine radiographs revealed atlantoaxial changes consistent with RA. A feature commonly noted on physical examination was decreased range of motion and/or flexion contractures of large joints. Six patients had flexion contractures of their elbows, wrists, ankles, or knees. The articular distribution of radiographic abnormalities in these patients was typical for RA and demonstrated common and symmetrical involvement of the proximal interphalangeal and metacarpophalangeal joints of the hand, all of the compartments of the wrist, and the metatarsophalangeal joints of the foot. Extensive alterations of all joints were observed in 3 individuals. Abnormalities of DISH were also typical and included widespread osteophytosis and ligament Figure I. Patieni FG. DISH. Observe linear JIowing ossification along rhe anterior aspecr of the vertebral column resulting in a bumpy spinal coniour. ranged from 1/40 to 1/5120. Five of 7 patients had positive fluorescent antinuclear antibody tests. One pa Figure 2. Patient FG. Roentgen.findings include lack of osteoporosis and the presence of bone sclerosis and proliferation, and inrraarticular bone tient had psoriasis; however, his joint examination, posi ankylosis,..mushrooming~~ of distal (arrowhead) and tive Serum rheumatoid factor, hypergammaglobulin carpal ankylosis. Superficial bone erosions appear well marginated and emia, and interstitial lung disease indicated he ad defined (arrows).

4 RESNICK ET AL 3. Osteophytes (Figure 3). Degenerative changes about involved articulations were common and resulted in osteophyte formation. 4. Bony ankylosis (Figures 2 and 4).

4 4 RESNICK ET AL 3. Osteophytes (Figure 3). Degenerative changes about involved articulations were common and resulted in osteophyte formation. 4. Bony ankylosis (Figures 2 and 4). Partial or complete carpal and tarsal ankylosis was observed. Bony ankylosis extended to involve carpometacarpal and tarsometatarsal articulations in 3 patients. Figure 3. Patient RA. Bone sclerosis appears about an erosion on the medial aspect of the proximal phalanx of the large toe (arrowhead). Note also the osteophytosis of the terminal phalanx (arrow). ossification of the spine (Figure 1 ), bony proliferation or whiskering about the pelvis, paraarticular osteophytes, ligament and tendon calcification and ossification, and spur formation on the calcaneus and ulnar olecranon. Atypical radiographic features of RA, evident to some degree in all of these patients, included the following: 1. Lack of osteoporosis (Figure 2). Patients failed to demonstrate periarticular demineralization. 2. Bony sclerosis and proliferation about erosions (Figures 2 and 3). Typical marginal erosions were surrounded by bone sclerosis and proliferation. Proliferative changes resulted in frayed irregular bone contours, spiculation, angular bony protuberances, and osseous enlargement with mushrooming of protruded surfaces. DISCUSSION Each of the above atypical radiographic features of RA, when taken alone, is occasionally observed in the disease. That is, it is not uncommon occasionally to note sclerosis about osseous erosion, minor degrees of carpal and tarsal ankylosis, and even secondary osteoarthritis about rheumatoid articulations. These findings are particularly prevalent in male patients with RA, and 7 of these 8 patients were men. The startling observation in these cases, however, was the widespread distribution and prominence of the productive osseous alterations. All involved joints were commonly atypical demonstrating bony eburnation or sclerosis, osteophytosis, and enlargement. Furthermore, the degree of bone ankylosis, which was considerable, resulted in complete carpal and tarsal masses with further extension to involve metacarpal and metatarsal bases. The resulting roentgen features simulated those of rheumatoid variant disorders, particularly ankylosing spondylitis, although there was no evidence of any of these diseases. Clinically, these patients with RADISH had a typical spectrum of seropositive RA except that they consisted primarily of older men with longstanding disease. The clinical signs and symptoms appeared to correlate with the activity of their RA, although the frequency of flexion contractures and marked limitation of motion in the elbows, wrists, knees, and ankles may have related to the coexistence of RA with DISH. Large irregular paraarticular osteophytes in several joints appeared mechanically to block full range of motion. It appears reasonable to speculate that bone production might occur about involved joints in patients with RAoDISH. The latter disorder is characterized by bone proliferation, particularly at sites of ligament and tendon attachment to bone both in the axial and extraaxial skeleton. This proliferation may result from normal or abnormal stress at tendoosseous junctions. Furthermore, heterotopic bone formation is also observed about prostheses following total joint replacements in

RADIOGRAPHIC ABNORMALITIES IN RA PATIENTS 5 Figure 4. Patient ES. On an anteroposterior radiograph of the midfoot, complete ankylosis is seen in the intertarsal and tarsometatarsal joints.

5 RADIOGRAPHIC ABNORMALITIES IN RA PATIENTS 5 Figure 4. Patient ES. On an anteroposterior radiograph of the midfoot, complete ankylosis is seen in the intertarsal and tarsometatarsal joints. patients with DISH (9). Similar bone formation in patients with DISH might occur about rheumatoid erosions as the osseous surface attempts to heal in an exaggerated fashion. These observations are of importance to radiologists who are reviewing roentgenograms showing osseous erosions in a typical distribution for RA with associated extensive bone proliferation. The latter finding should not mislead them into suggesting alternative diagnoses, particularly if radiographs of the spine demonstrate the characteristic pattern of DISH. REFERENCES Rheumatoid arthritis of the robust reaction type. Ann Rheum Dis 33:8185, Castillo BA, ElSallab RA, Scott JT: Physical activity, cystic erosions and osteoporosis in rheumatoid arthritis. Ann Rheum Dis , Jayson MIV, Rubenstein D, St J Dixon A: Intraarticular pressure and rheumatoid geodes (bone cysts ). Ann Rheum Dis 29:496502, Magyar E, Talerman A, Feher M, Wouters HW: The pathogenesis of the subchondral pseudocyst in rheumatoid arthritis. Clin Orthop 100:341344, Resnick D, Shad S, Robins J: Diffuse idiopathic skeletal hyperostosis: extraspinal manifestations of Forestier s disease. Radiology , Resnick D, Niwayama G: Radiographic and pathologic features of spinal involvement in diffuse idiopathic skel Rapporport AS, Sosman JL, Weissman BN: Lesions resembling gout in patients with rheumatoid arthritis. Amer eta1 hyperostosis (DISH). Radiology 1 19:559568, 1976 J Roentg 126:4145, Resnick D, Linovitz RL, Feingold ML: Postoperative het Resnick D: Goutlike lesions in rheumatoid arthritis. Am J erotopic ossification in patients with ankylosing hyper Roentgen01 127:1062, 1976 ostosis of the spine (Forestier s disease). J Rheum De Haas, WHD, DeBoer W, Griffioen F, Oostenelst P: 3: , 1976

HYPEROSTOSIS AND OSSIFICATION IN THE CERVICAL SPINE

564 RADIOLOGIC VIGNETTE HYPEROSTOSIS AND OSSIFICATION IN THE CERVICAL SPINE DONALD RESNICK A variety of diseases can produce hyperostosis or ossification in the cervical spine. Included among these are