Pachydermoperiostitis with Antisynthetase Syndrome

Abstract

Pachydermoperiostitis is a primary form of hypertrophic osteoarthropathy. Antisynthetase syndrome is primary autoimmune disease, associated with autoantibodies against one or more aminoacyl transport RNA synthetases, and development of interstitial lung disease, non-erosive arthritis, myositis, Raynaud phenomenon, fever of unknown origin and “mechanic’s hands”. We present a 36-years-old female patient admitted to Rheumatology clinic for diffuse joint pain and periodic edema of the knees, diffuse myalgia, whitening and blue discoloration of the fingers and toes with evening hyperpyrrhexia  of approximately 1 year duration. Subsequently, the patient developed photosensitive erythema on the face. The  angiopulmonography demonstrated pulmonary hypertension of venous type and mild splenoportal hypertension. The physical examination revealed Raynaud's syndrome, “clubbing fingers” and “hourglass nails”. The laboratory and immunological investigations showed normal acute phase reactants and muscle-related enzymes, elevated ANA, and high anti-PL-7 and anti-Ro-52 levels. The High-Resolution Computed Tomography of the lungs showed no pathological changes. The skin biopsy revealed IgM deposits at the dermoepidermal border. The functional examination of breathing showed isolated drop in TLCO, which could be explained by the pulmonary hypertension. The patient was diagnosed with Pachydermoperiostitis and Antisynthetase Syndrome. Corticosteroids, Plaquenil and Azathioprine were initiated and showed good therapeutic effect.