Unusual presentation of systemic lupus erythematosus in a male child: a case presentation

Background Systemic lupus erythematosus is an autoimmune connective tissue disorder that is common among women of age group 15-40 years. The novelty in our case is owed to the deceptive demographic characteristics, not only the sex (F > > M) but also of the age profile (common in 15-44 years age group) along with deviation from typical disease presentation of skin rash/arthritis/nephritic syndrome. We aim to emphasize the importance of having a high index of suspicion in any child presenting with nephrotic range proteinuria in order to prevent delay in diagnosis.Case presentation Eight-year-old boy presented with generalized swelling, proteinuria, hypoalbuminemia and hypertriglyceridemia and was found to be unresponsive to systemic steroid therapy. Further testing revealed low complement levels(C3/C4) along with ANA positive, homogenous pattern (titre >= 1:80) and anti-dsDNA positive (titre 229:24) pointing towards the diagnosis of childhood SLE, which was made based on EULAR/ACR criteria. Subsequent renal biopsy was done in order to stage the disease and for initiation of appropriate treatment protocol.Conclusions SLE is a highly heterogenous disorder in terms of clinical presentation. All patients with steroid resistant nephrotic syndrome should undergo renal biopsy as a part of their workup. This case is a learning opportunity which demonstrates that even in absence of typical disease manifestations and demographic profile, a high index of suspicion will help in rapid diagnosis and prevention of complications. Knowledge about the varied presentations of renal lupus is of utmost importance for the same.