Chronic granulomatous disease (CGD) is an uncommon genetic immunodeficiency disorder affecting neutrophil function, characterized by recurrent bacterial and fungal infections. X-linked carriers of CGD have an increased risk of autoimmune disorders, in particular lupus like disorders. We describe the case of a 37 years old female carrier of X-linked CGD, who presented with clinical features and serology consistent with a definite diagnosis of Systemic lupus erythematosus (SLE), with rare immune mediated neurological manifestations including secondary central nervous system (CNS) vasculitis and Longitudinally extensive transverse myelitis (LETM), responsive to immunomodulation. These neurological manifestations have not been described previously in carriers of CGD. We recommend early diagnosis of these immune mechanisms, especially in X-linked carriers of CGD, and appropriate immunomodulation in order to improve life expectancy and improve neurological outcome.

Takayasu's arteritis exposes to complications of varying severity, such as arterial stenosis, thrombosis, and more rarely aneurysms. Aortic dissection is a rare complication of Takayasu's disease, reported in few times in the literature, only 7 of which concern the abdominal aorta. We report the case of a 41-year-old woman followed for Takayasu disease for 15 years, who presented an asymptomatic and chronic dissection of the abdominal subrenal aorta. The patient underwent conservative medical treatment. After a follow-up of 17 months, the aortic dissection was still asymptomatic, with a stable appearance on follow-up imaging. To our knowledge, this is the first case report of asymptomatic aortic dissection as a rare complication of Takayasu disease.

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Sofia
Journal Co-ordinator
Journal of Rare Disorders: Diagnosis & Therapy