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PO.7.154 Jaccoud’s rheumatism during systemic lupus: a case report
  1. A Kella,
  2. N Bouziani,
  3. A Belabbas,
  4. M Derder and
  5. D Hakem
  1. Internal Medicine, Universitary Hospital Center ~ Mostaganem ~ Algeria

Abstract

Introduction Jaccoud’s rheumatism (JR) is a chronic, deforming and non-erosive arthropathy which preferentially affects the hands giving an aspect close to rheumatoid arthritis (RA). It’ is distinguished from this connective tissue by the initially reducible character of the deformations and the absence of erosions on standard radiography.

Observation BR, 34 years old, who with inflammatory arthralgia and a dry syndrome. The immunological assessment showed positive AAN 1/320, anti-SSA, anti-SSB and proteinuria for 24 hours at 4 g/24 hours. The accessory salivary gland biopsy showed Chisholm grade 4. The diagnosis of systemic lupus erythematosus (SLE) associated with secondary Sjogren’s syndrome (SSj) was retained. The nephropathy had benefited boluses of cyclophosphamide presuming an active and severe lesion. The corticosteroids and immunosuppressants had been continued for 2 years and for the joint damage which appeared 2years ago and a rhulupus had been diagnosed and benefited from treatment with hydroxychloroquine and methotrexate. The physical examination found a deformation of the hands type deviation in ulnar wind. The joint deformity was reducible, painless and the radiographs did not show any erosions allowing the diagnosis of JR be retained. The biological assessment found an accelerated ESR at 94 mm and a CRP at 67 mg/l, and the immunological assessment of AAN 1/320, SSA + and SSB suggesting a lupus inflammatory flare-up. Anti-CCP rheumatoid serology was negative. The patient also presented with autoimmune thyroiditis and heterozygous β-thalassemia.

Discussion The physiopathological mechanism of JR remains imperfectly elucidated. Its diagnostic criteria are not validated and its therapeutic management poorly codified. There are different severity scores for the evaluation of RJ. The most commonly used is the Jaccoud arthropathy (JA) index, used by Spronk and Al, which takes into account the number of fingers affected and the type of deformities visualized (ulnar deviation sup 20°, swan neck deformities, limitation of Metacarpophalangeal extension, buttonhole deformation, Z deformation.). The pathologies most frequently associated with JR are mainly SLE with a prevalence of 10–35%, more rarely SSj 2%. The clinical and serological factors correlated to JR are a longer duration of evolution of SLE, the presence of arthritis of the hands and wrists (rhulupus) and biologically, a higher CRP and the presence of anti-DNA antibodies. native or anti-phospholipid antibodies. RJ deformities tend to worsen over time, causing functional disability and impaired quality of life, and no treatment has been proven to be effective. Rehabilitation and the wearing of orthoses remain the usual modest techniques.

Conclusion JR is classically associated with SLE. A JR that has been evolving for several years can lead to degenerative damage and lead to irreducible deformities whose pathophysiology deserves to be better elucidate in order to determine the optimal therapeutic strategy. Therefore, it is important not to ignore this complication, which is the cause of a functional and unsightly disability that alters the quality of life.

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