CASE REPORT Clinical and Experimental Rheumatology 1999; 17: 730-732. Three cases of ABSTRACT naud’s phenomenon as the initial mani- This report describes three cases of festation of their disease. Disease of the osteonecrosis of the osteonecrosis of the lunate bone of the medium and small arteries and arterioles wrist in patients with systemic sclerosis causes local tissue ischemia that is asso- lunate bone of the presenting with wrist pain. All three ciated with tissue fibrosis and dysfunc- patients had limited skin scleroderma tion of major internal organs (5). Osteo- wrist in scleroderma but severe Raynaud’s phenomenon. necrosis in association with scleroderma Two patients never received cortico- is rare. One report describes two cases A.K Matsumoto1, steroids and one patient received only of osteonecrosis of the femoral head; 2 3 low doses for a brief period. None of only one patient had received prednisone R. Moore , P. Alli , the patients had other definable risk therapy (6). Two prior reports exist of a F.M. Wigley1 factors for osteonecrosis. Two patients patient with scleroderma and osteonecro- underwent vascular bone grafting with sis of the wrist (7, 8). The first patient 1Division of Rheumatology, Department improvement in symptoms. Osteo- had osteonecrosis of the wrist, but had of Medicine, and 2Department of Ortho- necrosis may represent an under- received 25 grams of prednisone over pedics, Johns Hopkins University School of recognized cause of wrist pain in eight years prior to the event (7). The Medicine; 3Department of Pathology, scleroderma patients. second patient had evidence of an in- Johns Hopkins Hospital, Baltimore, flammatory vasculitis with vasculitic Maryland, USA. Introduction ulcerations, digital loss, and an elevated Please address correspondence and reprint Osteonecrosis (avascular necrosis, ische- sedimentation rate and circulating im- requests to: Alan K. Matsumoto, M D. mic necrosis) of bone is a condition char- mune complexes (8). Johns Hopkins Division of Rheumatology acterized by death of a segment of bone 5501 Hopkins Bayview Circle - 3B.35 We present three cases of osteonecrosis Baltimore, MD 21224, USA. caused by the interruption of blood flow of the lunate in patients with sclero- E-mail: [email protected] to the bone marrow and trabecular ele- derma, two without prior corticosteroid Received on January 11, 1999; ment (1). Traumatic fracture and dislo- therapy, and one with only low dose accepted in revised form on June 16, 1999. cation resulting in disruptions in the mi- prednisone therapy prior to the diagno- crocirculation in the bone is the most sis of osteonecrosis (Table I). © Copyright CLINICAL AND common cause of osteonecrosis (2). EXPERIMENTAL RHEUMATOLOGY 1999. Atraumatic osteonecrosis is associated Case 1 with conditions that cause circulatory Key words: The patient is a 49-year-old woman with disturbance by thrombosis, embolization Scleroderma, osteonecrosis. scleroderma who presented with left or vascular injury (3). Included in this wrist pain in December 1997. She was category are hypercoaguable states, ni- diagnosed with scleroderma in 1992 af- trogen bubbles, Caisson’s disease, sickle ter a 10-year history of Raynaud’s phe- cell disease, radiation injury to the vas- nomenon and the subsequent develop- culature, and systemic vasculitis (3). Al- ment of sclerodactyly, facial telangec- cohol and corticosteroid use are predis- tasias and gastroesophageal reflux. Ma- posing factors for osteonecrosis (2). jor depression and a modest reduction Vascular disturbances are a well-recog- in single breath diffusing capacity on pul- nized part of the disease process in sys- monary function testing have also com- temic sclerosis (scleroderma) (4). Most plicated her clinical course. scleroderma patients experience Ray- She presented to the emergency room

Table I. Clinical and serologic features.

Case 1 Case 2 Case 3

Age 49 years 30 years 46 years Sex Female Female Male Skin involvement Sclerodactyly Sclerodactyly Sclerodactyly Prednisone use None 10 mg daily None ANA 1: 640 1: 1280 1: 1280 Anti-topoisomerase I Positive Positive Negative Anti-centromere Negative Negative Negative Russell viper venom time Normal Normal Normal Anti-cardiolipin IgG, IgM, IgA Negative Negative Negative

730 Osteonecrosis of the lunate bone in scleroderma / A.K. Matsumoto et al. CASE REPORT

Fig. 1. Wrist radiographs of Case 1 demonstrating sclerosis of both lunate bones. The rest of the carpal bones appear normal. after minor trauma to the left wrist. Ex- presenting with diffuse swelling of the have loss of full range of motion of the amination of her right wrist showed 60 fingers. She was diagnosed with fibro- wrist but only minor discomfort. degrees of extension and 70 degrees of sing alveolitis and was placed on cyclo- flexion. Her left wrist showed 45 degrees phosphamide 100 mg daily and predni- Case 3 of both extension and flexion. Radio- sone 10 mg daily in August 1992. Cyclo- The patient is a 46-year-old gentleman graphs of her wrists demonstrated bilat- phosphamide was discontinued in May who presented in March 1991 with left eral sclerosis of the lunate (Fig. 1). Mag- 1993 but prednisone 10 mg daily was wrist swelling and pain. He was diag- netic resonance imaging demonstrated continued until 1994. nosed with scleroderma at age 26 after loss of signal intensity in both her left Examination of her left wrist in January presenting with severe Raynaud’s phe- and right lunate consistent with bilateral 1994 demonstrated a restricted range of nomenon and sclerodactyly. The patient osteonecrosis of the lunate (Fig. 2). She motion with flexion to 30 degrees and has antibodies to U170Kda. His skin in- underwent a vascularized bone graft into extension to 45 degrees. There was pain volvement has remained limited to scle- the lunate in April 1998. Since then she on range of motion. Radiographs dem- rodactyly and telangectasias. Recurrent has done well, but continues to have right onstrated sclerosis of the left lunate and digital ulcerations, impotence and gas- wrist pain after activity. magnetic resonance imaging demon- troesophageal reflux disease has compli- strated loss of signal intensity at the left cated his clinical course. Case 2 lunate consistent with avascular necro- Examination of his wrist in 1991 showed The patient is a 39-year-old woman with sis. Because of progressive pain, the pa- the presence of swelling and decreased scleroderma who presented in January tient underwent a proximal row carpal- range of motion of the left wrist, with 1994 with left wrist pain. There was no ectomy in 1994 with complete resolu- 30° of flexion and extension. Radio- history of trauma. She first noted Ray- tion of her wrist pain. Bone biopsy dem- graphs of the left wrist revealed sclero- naud’s phenomenon in 1987 and was di- onstrated changes typical of osteonecro- sis of the lunate. He was treated con- agnosed with scleroderma in 1992 after sis (Fig. 3). Since then she continues to servatively with splinting and was lost

Fig. 2. Magnetic resonance images of both wrists of Case 1 demonstrating loss of signal intensity in both lunate bones consistent with osteonecrosis of bone.

731 CASE REPORT Osteonecrosis of the lunate bone in scleroderma / A.K. Matsumoto et al. assumed to be secondary to tendinitis or arthritis. Therefore, they may not be ap- propriately investigated for osteonecro- sis. Although we have not exhaustively excluded all other secondary causes of osteonecrosis, none was clinically evi- dent. The finding of three cases in this limited cohort of scleroderma patients supports an association of scleroderma and osteonecrosis of the lunate. We be- lieve that these cases represent an un- der-recognized complication of the vas- cular disturbance in scleroderma and rec- ommend that osteonecrosis be consid- ered in scleroderma patients presenting with wrist pain. Fig. 3. Histopathologic examination of the lunate bone of the patient pre- sented in Case 2, revealing osteonecro- Acknowledgements sis with necrotic cancellous bone ex- We thank Drs. Livia Casciola-Rosen and emplified by empty lacunae within the trabeculae. The surrounding marrow Antony Rosen for their assistance with space elements show diffuse necrosis the serologic typing. (Hematoxylin-eosin, x20). References to follow-up until December 1997 when positive and none had anti-centromere 1. CLIMCHER MJ, KENZORA JE: The biology of his wrist was pain-free, without signifi- antibody, despite the clinical expression osteonecrosis of the human femoral head and cant swelling but continued decreased of limited scleroderma. None of the pa- its clinical implications. II. The pathologic chan- ges in the femoral head as an organ and in the range of motion. tients exhibited serologic or clinical evi- hip joint. Clin Orthop 1979; 139: 283-312. dence of antiphopholipid antibodies. The 2. COOGAN PG, URBANIAK JR: Osteonecrosis, In: Discussion lack of other definable risk factors for Textbook of Rheumatology, 5th ed., W.B. Saun- We describe three patients with sclero- osteonecrosis in two of these three pa- ders, Philadelphia, 1997. 3. MANKIN HJ: Nontraumatic necrosis of bone (os- derma and osteonecrosis of the lunate tients and the involvement of the lunate, teonecrosis). N Engl J Med 1992;326:1473-9. without other conditions associated with a bone that is not commonly affected by 4. CAMPBELL PM, LEROY EC: Pathogenesis of osteonecrosis. Two of the patients had osteonecrosis, suggests that scleroderma systemic sclerosis: A vascular hypothesis. Semin never received corticosteroids and the vascular disease may have caused the Arthritis Rheum 1975; 4: 351-68. 5. RODNAN GP, MYEROWITZ RL, JUSTH GO: third had received low dose prednisone circulatory impairment resulting in the Morphologic changes in the digital arteries of 10 mg daily for only 18 months prior to bone injury. patients with progressive systemic sclerosis the diagnosis of osteonecrosis. Osteo- The prevalence of osteonecrosis of the (scleroderma) and Raynaud’s phenomenon. necrosis of the lunate (Kienbock’s dis- wrist is unknown in scleroderma. These Medicine 1980; 59: 393-408. 6. RODNAN GP, MEDSGER TA: Rheumatic mani- ease) is rare in the absence of trauma (9). three cases reflect the experience with festations of progressive systemic sclerosis. The first patient in this report had minor 650 patients seen at the Johns Hopkins Clin Orthop 1968; 57: 81. trauma to one wrist but bilateral disease and University of Maryland Scleroder- 7. KAWAI H, TSUYUGUCHI Y, YONENOBU K, was found when she was evaluated. This ma Center between 1991-1997. Given INCUE A, TADA K: Avascular necrosis of the carpal scaphoid associated with systemic scle- makes it unlikely that a traumatic insult the referral nature of the center, it is im- rosis. Hand 1983; 15: 270-3. was the sole etiology of her osteonecro- possible to estimate the true prevalence 8. AGUS B: Bilateral aseptic necrosis ofthe lunate sis. Interestingly, all three patients had of this problem. in systemic sclerosis. Clin Exp Rheumatol 1987; limited skin involvement and severe, However, the prevalence is likely to be 5: 155-7. 9. WRIGHT PE: Keinbock’s disease. In CANALE longstanding Raynaud’s phenomenon. underestimated because patients with ST (Ed.): Campbell’s Operative Orthopedics, St. Two were anti-topoisomerse I antibody scleroderma often have joint discomfort Louis, Mosby 1998: 3469-72.

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