title: Scleroderma renal crisis in a case of mixed connective tissue disease.
abstract: Mixed connective tissue disease (MCTD) is an overlap syndrome first defined in 1972 by Sharp et al. In this original study, the portrait emerged of a connective tissue disorder sharing features of systemic lupus erythematosus, systemic sclerosis (scleroderma) and polymyositis. Scleroderma renal crisis (SRC) is an extremely infrequent but serious complication that can occur in MCTD. The histologic picture of SRC is that of a thrombotic micro-angiopathic process. Renal biopsy plays an important role in confirming the clinical diagnosis, excluding overlapping/superimposed diseases that might lead to acute renal failure in MCTD patients, helping to predict the clinical outcome and optimizing patient management. We herewith report a rare case of SRC in a patient with MCTD and review the relevant literature.
authors: Department of Pathology, Global Health City, Chennai, Tamilnadu, India.
authors: Mukul
authors: M
authors: Vij
authors: Vinita
authors: V
authors: Agrawal
authors: Manoj
authors: M
authors: Jain
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology; D001706:Biopsy; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007668:Kidney / Q000473:pathology*; D007674:Kidney Diseases / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D008297:Male; D008947:Mixed Connective Tissue Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D010956:Plasmapheresis; D011237:Predictive Value of Tests; D006435:Renal Dialysis; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult