[Post-infectious systemic vasculitis: recovery without corticotherapy].

Abstract

UNLABELLED: In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic. CONCLUSION: In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.