Reno pulmonary syndrome due to systemic lupus erythematosus associated with ANCA p: A case report

Abstract

Introduction: The term pulmonary-renal syndrome (PRS) is defined as pulmonary and renal failure caused by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Its pathogenesis is due to a variety of immune-mediated mechanisms which is related to the deposition of antiglomerular basement membrane antibodies, antineutrophil cytoplasmic antibodies, immune complexes, and thrombotic microangiopathy. Since the syndrome is characterized by a fulminant course if left untreated, early diagnosis, exclusion of infection, close monitoring, and timely initiation of treatment are crucial for patient outcome. Treatment consists of ventilatory support, replacement of renal function, high-dose corticosteroids, and cytotoxic agents. Kidney transplantation is the only alternative in end-stage renal disease. The association of Systemic Lupus Erythematosus (SLE) with results of Antibody Neutrophil Cytoplasm (ANCA) does not necessarily indicate that it is also a Vasculitis if criteria for the latter are lacking, but if they confer a worse prognosis. Objective: To present the case of a patient with SPR due to SLE plus the presence of Anca p from Hospital Regional Ambato. Methodology: Data were collected by direct interview and supplemented with review of the patient's physical medical history with the due informed consent. Results: Updated review of the pathogenesis, diagnostic approach and treatment of PRS.

Introduction: The term pulmonary-renal syndrome (PRS) is defined as pulmonary and renal failure caused by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Its pathogenesis is due to a variety of immune-mediated mechanisms which is related to the deposition of antiglomerular basement membrane antibodies, antineutrophil cytoplasmic antibodies, immune complexes, and thrombotic microangiopathy. Since the syndrome is characterized by a fulminant course if left untreated, early diagnosis, exclusion of infection, close monitoring, and timely initiation of treatment are crucial for patient outcome. Treatment consists of ventilatory support, replacement of renal function, high-dose corticosteroids, and cytotoxic agents. Kidney transplantation is the only alternative in end-stage renal disease. The association of Systemic Lupus Erythematosus (SLE) with results of Antibody Neutrophil Cytoplasm (ANCA) does not necessarily indicate that it is also a Vasculitis if criteria for the latter are lacking, but if they confer a worse prognosis. Objective: To present the case of a patient with SPR due to SLE plus the presence of Anca p from Hospital Regional Ambato. Methodology: Data were collected by direct interview and supplemented with review of the patient's physical medical history with the due informed consent. Results: Updated review of the pathogenesis, diagnostic approach and treatment of PRS.