DOI: 10.36347/sasjm.2022.v08i10.020

Pages: 768-773

PAN is an inflammatory and necrotizing vasculitis affecting small and medium caliber arteries of the body, rarely described in children. Its pathophysiology is complex and remains poorly elucidated, involving the deposition of circulating immune complexes in the vascular wall, neutrophil anti-cytoplasmic antibodies, the role of cytotoxic lymphocytes, cytokines, environmental factors (infections), as well as genetic susceptibility (mutations in ADA 2 and the MEFV gene). There are two main forms: a classic systemic form with visceral involvement (60% of cases) and a cutaneous form with lesions limited to the skin and muscles (30%), which usually evolve less severely. Positive diagnosis is based on the Eular/Printo/Pres criteria. The treatment depends on the form of PAN, and relies essentially on NSAIDs, colchicine, corticotherapy and immunosuppressants: azathioprine and cyclophosphamide. Our work is a retrospective study of 5 cases of PAN, collected at the IV pediatric department and the pediatric rheumatology consultation of the children's hospital of Rabat between 2009 and 2014: The diagnosis was retained on the criteria of Eular/Printo/Pres. The clinical picture was dominated by skin and joint involvement. One of our patients presented an atypical picture of PAN associated with infective endocarditis and large vessel involvement. All of our patients received corticosteroid therapy, and one of our patients benefited́ in addition to colchicine.