ABSTRACT
INTRODUCTION: We reported a case of hemophagocytic syndrome complicating microscopic polyangitis presented by crescentic glomerulonephritis. CASE PRESENTATION: 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.
ABSTRACT
UNLABELLED: Chronic hemodialysis in Rwanda is relatively recent and most of patients are treated with catheters. SUMMARY: Thirty-seven patients who require chronic hemodialysis with catheters were evaluated during a 3-years period in order to facilitate the creation of a permanent vascular access for hemodialysis (AVF). Patient selection were made during a multi-disciplinary consultation. The sex-ratio was 1.5 and the main cause of the nephropathy was arterial hypertension. RESULTS: Thirty-one patients benefited from the creation of an arterioveinous fistula. All of the interventions were performed using local or loco-regional anesthesia. Sixty percent of these AVF were radio-cephalic, 35.4% were humero-cephalic. Sixty-four percent of the operations were performed on ambulatory patients, with a primary function for 90% of them. CONCLUSION: This work proves the feasibility of the creation of AVF in Rwanda, thus allowing to preclude the various complications that arise with the prolonged use of a catheter. This experience was made possible by the pooling of the resources of 4 of Rwanda's leading hospitals. In an early future, the development of vascular surgery will assure the permanence of this care.
