Adult Pulmonary-Renal Syndrome in a Military Centre: A 19-Year Retrospective Review

Abstract

Pulmonary Renal Syndrome (PRS) is a rare entity, defined by the association of intra-alveolar hemorrhage and rapidly progressive glomerulonephritis, which histologically translates to extracapillary glomerulonephritis. It requires emergency diagnosis and treatment, and its etiologies are mostly ANCA vasculitis. The objective of this study was to describe the characteristics of renal and pulmonary involvement during PRS, its etiologies and evolution, and to study the overall survival of the patients. This is a descriptive and analytical study that includes all patients admitted for PRS and is analyzed retrospectively from January 2005 to March 2024. There were 18 patients with an average age of 45 years [32;64] and a sex ratio of 1. Regarding renal manifestations, 16 patients out of 18 showed rapidly progressive renal failure, requiring the use of dialysis in the majority of cases. The average serum creatinine level of the patients included was 751 μmol/l, the average proteinuria was 3.2 g/24h, and 15 patients had hematuria. Regarding pulmonary manifestations, 13 patients presented with hemoptysis, 15 patients had intra-alveolar hemorrhage confirmed by chest CT scan, and half of them required mechanical ventilation. The median follow-up of the patients was 38 months [15;137]. The treatment response consisted of pulmonary remission in all patients versus renal remission in only 6 patients, with 3 patients having come off dialysis. Twelve patients progressed to chronic renal failure while 11 patients were still relying on dialysis; there were 6 relapses, mainly pulmonary, and 2 deaths. There was no statistically significant difference in the overall survival of patients who relapsed or not (p = 0.67). In conclusion, PRS is a life-threatening emergency in both the short and long term. In our series, renal involvement was as severe as pulmonary involvement, with progression to chronic renal failure in two-thirds of patients and significant dependence on dialysis.