Abstract:

Neonatal lupus is a rare congenital syndrome resulting from placental passage of maternal antibodies against SSA/Ro and/or SSB/La to the fetus. It is a generally benign condition, the cardiac involvement determining the prognosis. NNL is rarely manifested by isolated cardiomyopathy. The subsequent evolution towards systemic lupus is rare and unpredictable. We report the case of a daughter from a non-consanguineous marriage with healthy parents who presented a dilated cardiomyopathy in the first months of her life complicated by several outbreaks of heart failure. The spontaneous evolution was marked by the occurrence of skin lesions of the face and arms at the age of 10 years, then by the installation of episodes of hydrous retention at the age of 12 years complicated by an acute renal insufficiency with a rapid fatal outcome at the age of 15 years. ANA antibodies rate was at the threshold of positivity, antiDNA and ANCA were not detectable.C3 rate was low. Although a genetic cause can never be ruled out, the diagnosis was a systemic erythematous lupus SLE (an evolution of an eventual NNL or a childhood-onset systemic lupus). We propose a decision tree for the management of neonatal cardiomyopathy NCMP as a synthesis of the literature.The search for anti SSA/SSB must be part of the check-up usually requested at the time of pregnancy. In addition, we must strengthen the knowledge of the medical profession, especially in the proximity health centers, on rare diseases for a better management of patients.

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