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Rheumatology 2001; 40: 594-595
© 2001 British Society for Rheumatology
Letters to the Editor |
Systemic sclerosis with pencil-in-cup deformity
Department of Medicine, K. G. Medical College, Lucknow 226003, India
SIR, We report a 16-yr-old female suffering from systemic sclerosis (SSc) who presented with deforming symmetrical polyarthritis which she had had for the past 1 yr, associated with a history of Raynaud's phenomenon and thickening of the skin, initially involving the hand and then becoming generalized to involve the feet, face and trunk. The patient had scleroderma facies, thickened skin over the face, extremities and trunk, healed fingertip infarcts, resorption of the pulp of the terminal pad of the fingers, flexion contractures of the fingers, and swelling and tenderness of the metacarpophalangeal (MCP) and proximal (PIP) and distal (DIP) interphalangeal joints of both hands. There was no telangiectasia, calcinosis or friction rub over the involved joints on movement. Raised erythrocyte sedimentation rate (ESR, Westergren; 40 mm/h), negativity for rheumatoid factor (20 IU/ml), positivity for anti-Scl-70 (41.390 EU/ml; cut-off >25 EU/ml) by double-sandwich bichromatic solid-phase enzyme-linked immune assay, and negativity for antinuclear antibodies (ANA) and anticentromere antibodies were the important investigatory findings. X-ray of the hands showed acro-osteolysis, flexion deformity of the fingers, erosive and destructive changes in the carpometacarpal (CMC), MCP, PIP and DIP joints with relative sparing of the CMC of the thumb, soft tissue atrophy, and whittling of the head of the proximal phalanx with broadening of the base of the middle phalanx, resulting in the characteristic pencil-in-cup deformity of the left 5th PIP joint (Fig. 1
). There was no radiological evidence of calcinosis. X-ray of the chest was within normal limits. Pulmonary function tests showed a FVC of 1.62 (76% predicted), FEV1/FVC of 100 and FEF25–75 of 3.9 (143% of predicted value). On questioning, the patient denied a history of psoriasis in family members, and examination of available family members failed to reveal psoriasis.
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The patient was classified as having diffuse SSc according to the 1980 ACR classification criteria [1]. Erosive arthritis in systemic sclerosis can occur with associated rheumatoid arthritis (RA). Horiki et al. [2] reviewed the literature and found 15 patients in whom SSc overlapped with RA. They compared the clinical and laboratory features of these patients, and suggested that SSc–RA overlap may be a distinct entity based on the characteristic features observed in these patients, which include generalized skin sclerosis (12 out of 14 patients), severe seropositive erosive polyarthritis (15 out of 15), pulmonary fibrosis (14 out of 15), anti-topoisomerase I antibodies (eight out of 10 examined) and HLA haplotypes DR4, 53; DQA1*0301; DBQ1*04 (in five cases examined). None of the reported patients of SSc–RA overlap had destructive DIP involvement [2]. Unlike the earlier reported cases, our patient was negative for rheumatoid factor, and had prominent destructive changes also involving DIP and an almost simultaneous onset of arthritis, skin thickening and Raynaud's phenomenon. Thickened skin of the trunk, destructive arthropathy, prominent DIP involvement, seronegativity and the absence of systemic features suggest that the patient did not have mixed connective tissue disease either.
Non-rheumatoid arthritis in SSc usually presents as rheumatoid-like symmetrical polyarthritis with predominant hand involvement, and may be erosive in as many as 40% of patients, but is less destructive [3]. Unlike RA, DIP and the first CMC joints may be involved [3, 4]. The radiological features are usually limited to mild joint-space narrowing, osteoporosis and small discrete erosions at periarticular margins [3]. Patients who have SSc with arthritis are reported to have predominantly limited skin involvement [5] and calcinosis [3]. They are also more likely to be positive for rheumatoid factor (80%) [5] and anticentromere [5], antinuclear and anti-double-stranded DNA antibodies [6]. However, the above-mentioned features were not observed in this patient.
Even though DIP involvement is reported frequently in SSc with arthritis [3], the pencil-in-cup deformity is considered to be relatively specific for psoriatic arthropathy. To the best of our knowledge it has not been reported in SSc or in SSc–RA overlap. Although psoriasis has been reported previously in association with SSc [7], there was no evidence initially of the skin or nail changes of psoriasis in our patient, and none had appeared at the 3-month follow-up. A family history of psoriasis was also lacking. Significant DIP involvement and the pencil-in-cup deformity in this patient suggest that arthritis in SSc can mimic the hand-joint involvement of psoriatic arthropathy. Fischer [8] also pointed out that erosions in SSc occur at the entheses of the hand. It has been suggested that soft-tissue calcification occurring at the insertions of ligaments into the phalanges could lead to erosions of the neighbouring bone during absorption of the calcifications [9]. The involvement of the DIP and a pencil-in-cup deformity (psoriatic arthropathy-like illness) in SSc suggests the role of enthesitis in the causation of erosions.
Notes
References
- Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee: Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum1980;23:581–90.[Web of Science][Medline]
- Horiki T, Moriuchi J, Takaya M et al. The coexistence of systemic sclerosis and rheumatoid arthritis in five patients. Clinical and immunogenetic features suggest a distinct entity. Arthritis Rheum1996;39:152–6.[Web of Science][Medline]
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Baron M, Lee P, Keystone EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis1982;41:147–52.
[Abstract/Free Full Text] - Catoggio LJ, Evison G, Harkness JA, Maddison PJ. The arthropathy of systemic sclerosis (scleroderma): comparison with mixed connective tissue disease. Clin Exp Rheumatol1983;1:101–12.[Medline]
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Misra R, Darton K, Jewkes RF, Black CM, Maini RN. Arthritis in scleroderma. Br J Rheumatol1995;34:831–7.
[Abstract/Free Full Text] - Serup J, Staun-Olsen P. Antinuclear antibodies and anti-DNA antibodies in scleroderma. A possible relationship between joint manifestations and increased antibodies in localized scleroderma. Allergy1986;41:452–6.[Medline]
- Korotkii NG, Sharanova G, Udzhukhu V, Antonova NA, Panchenko MA. A case of severe form of psoriasis and systemic scleroderma [Russian]. Vestn Dermato Venerol1984;(5):48–9.
- Fischer F. Enthetic reactions of the hand in progressive scleroderma [German]. Z Rheumatol1986;45:255–9.[Medline]
- Fischer F. Development of erosions in the finger phalanges during absorption of periosseous calcification [German]. ROFO: Fortschr Geb Rontgenstr Nuklearmed1984;141:87–91.
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