1. Private Practice, Kalamata, Greece
2. Division of Rheumatology, Department of Internal Medicine, Patras, University Hospital, University of Patras Medical School, Patras, Greece
3. Rheumatology Unit, Alexandra General Hospital, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece
Volume 35, Issue 1, March 2024
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1980892708
Mediterr J Rheumatol 2022;33(2):259-60
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Hands Deformity in a Patient with IgA Vasculitis
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CASE PRESENTATION
A 74-year-old female patient diagnosed with IgA vasculitis (formerly known as Henoch–Schönlein purpura) was referred by her general practitioner for a rheumatologic evaluation. The diagnosis has been made 12 years ago and was based upon her typical clinical manifestations (purpura, gastrointestinal bleeding, glomerulonephritis) and the typical findings in kidney biopsy (dense deposits of IgA immunocomplexes and C3 complement).1 The patient had a chronic disease with frequent flares and was under chronic immunosuppressive treatment with steroids and azathioprine by her attending nephrologists. Arthritic complaints were minimal and she has never been referred to a rheumatologist.
Main laboratory findings included anaemia (Haematocrit 33%, Hemoglobulin 10.9 gr/dl), elevated serum creatinine (1.72 gr/dl), haematuria (100 red cells per high power field), ANA(-), ANCA(-), RF(-) and anti-CCP(-) . Physical examination revealed arthritis in both hands and chronic swan-neck type deformities. The deformities were reversible. Radiographic findings included ulnar deviation, sublaxation of the 1st MCP joints in both hands and absence of joint erosions. This clinical image is typical of Jaccoud’s arthropathy.
Jaccoud’s arthropathy is mainly observed in Rheumatic Fever, Systemic Lupus Erythematosus and Sjogren’s syndrome but has been associated with several clinical entities.2 It is characterised by reversible deformities, usually affecting the MCP and MTP joints. The pathogenesis of the arthropathy is mostly attributed to capsule fibrosis and relaxation of tendons and ligaments. The most characteristic finding in X-rays is the absence of joint erosions, despite the presence of ulnar deviation and swan-neck deformities. This is the major clue for the differential diagnosis between Jaccoud's arthropathy and rheumatoid arthritis. Treatment is mainly conservative and focuses on the underlying disease. To our knowledge this is the first case of Jaccoud’s arthropathy associated with IgA vasculitis in literature.
Figure 1. Arthritis of the right hand with ulnar deviation, swan-neck type deformities in the index and middle finger.
Figure 2. X-Ray shows ulnar deviation of both hands, subluxations of the 1st and 5th MCP joints (white arrows), soft tissue enlargement (green arrow) and absence of bone erosions.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
FUNDING SUPPORT/FINANCIAL BENEFITS
None received in relation to this manuscript.References
- Audemard-Verger A, Pillebout E, Guillevin L, Thervet E, Terrier B. IgA vasculitis (Henoch-Shönlein purpura) in adults: Diagnostic and therapeutic aspects. Autoimmun Rev 2015;14:579-85.
- Santiago MB. Jaccoud’s arthropathy. Best Pract Res Clin Rheumatol 2011;25:715-25.
