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Goodpasture syndrome (GS) is a rare clinical condition involving an autoimmune response targeting basement membranes, resulting in damage to renal glomeruli and pulmonary alveoli. Conventionally, the disease is marked by a rapidly progressive glomerulonephritis (RPGN) with acute deterioration. However, the case we present in this report challenges the typical course, as it describes a prolonged and unusual presentation of GS, extending over an 8-month period. The extended evolution and symptom similarity to other pulmonary and renal diseases posed significant diagnostic challenges. Treatment included hemodialysis, pulse therapy with corticosteroid and cyclophosphamide associated with plasmapheresis.
The development of this report involved analysis of the patients medical records, laboratory tests, imaging reports, renal biopsy results and prior medical history. The patient underwent interviews to gather information about his symptom evolution, previous treatments, environmental exposures and other factors relevant to diagnosis.
A review of medical literature related to GS was also carried out, in addition to investigation of previously published case studies and treatment guidelines. The findings were discussed and conducted to create a comprehensive report, accentuating the unique aspects of the case.
We report the case of a 23-year-old man who presented a prolonged prodromal condition of hemoptoic sputum accompanied by localized edema in both lower limbs, which began in February 2022. Covid tests were carried out, with negative results. Treatment for the virus was nevertheless realized. In September 2022, he was evaluated in his city health service and later transferred to a reference hospital center in Sorocaba due to laboratory results including hemoglobin of 4 g/dL, creatinine of 9 mg/dL and type I urine with nephrotic proteinuria and hematuria. He underwent renal biopsy that revealed a diagnosis of global and diffuse proliferative glomerulonephritis, with segmental sclerosis and crescents in 11 out of 17 glomeruli. The immunofluorescence results showed diffuse linear glomerular deposits of IgG (+++), Kappa (+++) and Lambda (+++), and trapping-type deposits of IgM, C1q and C3. The pattern was of immune-mediated glomerulopathy, consistent with anti-basement membrane antibody syndrome. The patient required hemodialysis treatment, which is currently being maintained, pulse therapy with corticosteroids and cyclophosphamide and plasmapheresis. He had an adverse skin reaction to the use of albumin in plasmapheresis, which was replaced with fresh plasma.
Conclusions
The presented report discusses an original case, revealing a significant deviation from the typical clinical profile of GS. The distinctive feature of this case is the prolonged duration of the patients clinical presentation which extended for over an 8-month period before actual diagnosis and treatment. The extension of the prodromal phase, often resembling that of other pulmonary and renal conditions, is a rare and under documented characteristic in existing literature. Consequently, knowledge of such atypical progressions can help with early interventions thar are essential for a favorable prognosis. This case was presented at the PUC-SP Academic Congress and the Paulist Congress of Nephrology, with authorization from both for international submission.