A 22-year-old female patient originated from Dakar, Senegal presented with
nephrotic syndrome and rapidly progressive
glomerulonephritis. On physical examination, we noticed hyperchromic diffuse punctilious
purpura skin lesions predominant on the trunk, the neck and the upper thigh. Immunology investigations revealed strongly positive anti SSA/Ro and anti-SSB.
Anti-neutrophil cytoplasmic antibodies had positive results with a peri-nuclear type fluorescence, specific to
myeloperoxidase. In optic microscopy, renal biopsy showed a crescentic
glomerulonephritis with circumferential cellular and fibrous proliferation affecting 85% of glomeruli. The diagnosis of microscopic polyangitis with renal and skin involvement was retained. The patient received
methylprednisolone and
cyclophosphamide 700 mg/m(2) every 15 days for the first 3 pulses and every 21 days thereafter. After the 5(th) month, she developed obnubilation,
fever and central
pancytopenia. Bone marrow aspiration was performed, which showed medullary invasion by macrophages with signs of hemophagocytosis. Diagnosis of
hemophagocytic syndrome complicating a microscopic polyangitis was retained and
methylprednisolone pulses started. The patient was under
hemodialysis after follow-up of about 9 months with stable clinical state.
CONCLUSIONS: The occurrence of SAM in pauci-autoimmune
vasculitis is rarely described, particularly in Africa. Our case is an illustration of the reality of this association.