Nephrotic syndrome heralding Scleroderma

Résumé

Background
Systemic Sclerosis (SSc) is a multisystem connective tissue disorder of unknown aetiology characterized chronic inflammation, deposition of collagen and other macromolecules in extracellular matrix of tissues. Renal affectation in SSc is usually rare. In most cases Scleroderma precedes the renal disease. The finding of Nephrotic syndrome in Scleroderma is most often due to complication of drug management or amyloidosis. We hereby present a case of nephrotic syndrome preceding diffuse cutaneous systemic sclerosis.
Case
MB is a 17-year-old female who presented with 3 months history of generalized body swelling, a week after a febrile illness. She had taken Artemisinin combination therapy, otherwise no other drug history. She had no other systemic symptoms and her past medical history was unremarkable. Examination revealed mainly facial and lower limb oedema, otherwise no other significant finding. Her chemistry revealed 24-hour urine protein 6.1g, Serum albumin 26g/L, and dyslipidaemia. Her renal biopsy result showed features of Minimal Change Disease. She had 6 months course of Prednisolone and remitted. Seven months post treatment, she developed features of diffuse cutaneous systemic sclerosis, and has been treatment till date. No other renal involvement.
Conclusion
Most cases of Nephrotic syndrome associated with Systemic Sclerosis occurred as complication after frank features of SSc have emerged unlike the index patient. Hence the need for high index of suspicion