DOI: 10.36347/sjmcr.2024.v12i03.025

Pages: 336-338

Background: Pulmonary renal syndrome (PRS) is a diagnostic and therapeutic emergency defined by the association of alveolar haemorrhage and rapidly progressive glomerulonephritis. The primary causes of PRS are dominated by ANCA-associated vasculitis (60-70% of cases) and Goodpasture's syndrome. The pauci-immune variant of this syndrome (ANCA-negative) is rare, and its management lacking well-established guidelines. We herein present the case of a patient with renal and pulmonary manifestations of ANCA-negative vasculitis. Case presentation: A 42-year-old woman with no prior medical history presented with acute dyspnoea with hemoptysis associated with purpuric lesions on both lower limbs and macroscopic hematuria. Initial laboratory values revealed a normochromic normocytic anemia at 6.1 g/dL, CRP of 264 mg/L, renal failure with a GFR of 33 mL/min/1.73m², active urine sediment and 24-hour urine protein at 2.53g/24h. The anti-nuclear antibody was negative, and routine enzyme-linked immunosorbent assay (ELISA) were negative for MPO-ANCA, PR3-ANCA and anti-MBG. A thoracic angioscan revealed alveolar hemorrhage, while cardiac echocardiography indicated an acute cor pulmonale. Due to the patient's clinical instability, a kidney biopsy was not performed. The diagnosis of ANCA-negative vasculitis was suspected. Management of the patient required several stays in intensive care, including pulse therapy with a high dose of methylprednisolone at 1000 mg/daily for 3 days followed by oral prednisone 1 mg/kg per day, appropriate antibiothics, multiple transfusions, and plasma exchanges due to corticosteroid resistance. The patient’s clinical status showed an initial moderate respiratory improvement before succumbing to an acute respiratory failure. Conclusion: There is a pressing need to promptly establish a well-structured management approach to ensure a more favorable prognosis for this uncommon condition.