MO324A RARE CASE OF GRANULOMATOSIS WITH POLYANGIITIS ASSOCIATED WITH FACTOR II DEFICIENCY

Abstract

Abstract Background and Aims Granulomatosis with polyangiitis (GPA) (formerly called Wegener's) is a systemic disorder that is characterized by necrotizing vasculitis of small arteries and veins. The kidney damage is severe. We report an observation of a young patient whose exploration of rapidly progressive renal failure revealed GPA with an original association with factor II deficiency. Method This a case report of a patient who was admitted to the nephrology, dialysis and transplantation in the sahloul university hospital in Tunisia. She was transferred from the regional hospital of sidi bouzid. Results This a case of a 19-year-old patient. She had the only a history of allergic rhinitis. She presented recently epistaxis and headache. The explorations reported a kidney failure with level of creatinine at 5.6 mg/dl. On examination: The blood pressure was normal (120/70 mmHg), she had signs of acute sinusitis and edema in both lower limbs and she developed anuria. For the biology: creatinine = 11.3 mg/dl (clearance = 4.6 ml/min), urea = 50 mmol/l, Proteinuria = 2.5 g/24h and Hematuria = 100/mm3. The cANCA type PR3 was positive. The diagnosis of rapidly progressive glomerulonephritis (RPGN) was suspected. She had microcytic hypochromic anemia of 6.9 g/dL and a low Prothrombin time (PT) of 54%. The exploration concluded to an isolated Factor II deficiency (59%). The Ear, Nose and throat (ENT) examination revealed nasal inflammation, sinusitis and nasal crusting. The kidney ultrasound showed two kidneys of normal size. The computed tomography showed bilateral maxillary sinusitis and there were no intra-alveolar hemorrhage. The Kidney biopsy could not be performed initially due to the risk of bleeding. On hospitalization, the patient presented a generalized tonic clonic seizures. The exploration by a cerebral Magnetic resonance imaging (MRI) showed signs of cerebral vasculitis and the electroencephalography (EEG) showed no focal abnormalities. The association of ENT involvement, the cerebrovascular disease, the rapid evolution of the creatinine, the positivity of the cANCA, the diagnosis of granulomatosis with polyangiitis was established. The therapeutic management was to start symptomatic treatment: diuretics, antibiotic therapy, transfusions, hemodialysis initially and antiepileptic therapy. The immunosuppressive treatment was based on pulses of methylprednisolone followed by full dose corticosteroid therapy, cyclophosphamide intravenous and sessions of plasma exchange. The evolution was characterized by clinical and biological improvement with creatinine = 1.3 mg/dl, a PT at 100% and a factor II at 70 % stable after stopping the plasma exchange in favor of the immunological cause of this deficit. Conclusion The early diagnosis and treatment of RPGN is important even if the biopsy cannot be performed. We reported a rare association between granulomatosis with polyangiitis and factor II deficiency. The normalization of the factor II after the treatment is in favor of the immunological cause of this deficit.