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Acute myocarditis is extremely rare in patients with Behçet disease. Here we report a case of a 32-year-old man with a history of oral ulcerations who was admitted for fever and chest pain. On examination, he had bipolar ulcers with pseudofolliculitis and a positive pathergy test. Electrocardiogram and transthoracic echocardiography were normal. Cardiac magnetic resonance imaging revealed left ventricular inferior and lateral wall late gadolinium enhancement. A diagnosis of acute myocarditis revealing Behçet disease was made, and the patient was treated with colchicine and pulses of methylprednisolone, and cyclophosphamide. A 7-month follow-up magnetic resonance image showed a substantial decrease in the late gadolinium enhancement area.

Background: Autoimmune diseases result from the interplay of cellular effectors like T and B cells, regulatory cells in addition to molecular factors like cytokines and regulatory molecules. Methods: Different electronic databases were searched in a non-systematic way to find out the literature of interest. Results: Pathogenesis of autoimmune diseases involves typical factors such as genetic background including HLA and non HLA system genes, environmental factors such as infectious agents and inflammatory cells mainly T and B lymphocytes abnormally activated leading to immune dysfunction. Other recently reported less typical factors such as micro-RNAs, circular RNAs, myeloperoxidase, vimentine and microbiome dysbiosis seem to be potential target therapies. Conclusion: We aimed in this manuscript to review common factors in the pathogenesis of autoimmune diseases.

A 66-year-old woman presented with slowly progressive cognitive decline. MRI showed bi-frontotemporal atrophy, but the 14.3.3 protein was not present in cerebrospinal fluid. Electroencephalogram was irrelevant. Interview with relatives led doctors to search for a genetic cause and confirmed the diagnosis of Gerstmann–Sträussler–Scheinker syndrome.