A 51-year-old woman with advanced rheumatoid arthritis developed a Goodpasture’s syndrome during treatment with penicillamine and carbimazole. Circulating antiglomerular basement membrane antibodies (anti-GBM) were present. Renal biopsy showed focal necrotizing glomerulonephritis with crescents, and HLA typing showed the presence of DR3 and DR4. The patient responded dramatically to pulse methylprednisolone and cyclophosphamide, with both clinical remission and disappearance of anti-GBM antibodies.

A child developed steroid-responsive nephrotic syndrome at the age of 3 years. 6 years later, he developed insulin-dependent diabetes mellitus. At this time renal biopsy disclosed minimal-change disease. After multiple relapses requiring cyclophosphamide or repeated courses of steroid therapy, a second renal biopsy, 5 years after the first, revealed early diabetic changes with associated exudative lesions. The nephrotic syndrome remains responsive to steroids and cyclophosphamide, and the patient maintains an increased glomerular filtration rate and normal blood pressure 3.5 years afterwards. His HLA typing showed DR4 and DR7. Since DR4 and DR7 are associated with diabetes and minimal-change disease, respectively, we speculate that he could carry the genetic predisposition for the development of both diseases.

Systemic autoimmune diseases are characterized by a wide variety of clinical manifestations, including cutaneous lesions. Pyoderma gangrenosum (PG) and pyodermalike lesions can be associated with different rheumatic conditions, such as granulomatosis with polyangiitis (GPA). 1 Management of this pathology is based on local wound care, avoidance of trauma, and in some occasions, systemic therapy with systemic corticosteroids and cyclosporine being the mainstays of treatment. In recalcitrant PG, biologic medications, such as tumor necrosis factor-alpha (TNF-a) inhibitors, could be necessary. Actually, infliximab (IFX) is the only biologic that has shown efficacy in classic PG in a randomized, double-blind, controlled trial (level 1 evidence). 2 Rituximab (RTX) was approved for use in GPA by the Food and Drug Administration in April 2011. The use of RTX for cutaneous and subcutaneous GPA lesions has previously been reported in some case series and case reports. 3 In this article, we present the case of an antiphospholipid antibody (aPL)-positive female patient diagnosed with GPA who developed severe PG-like skin involvement that was responsive to RTX therapy.