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Objectives Pulmonary-renal syndrome is a rare but life-threatening manifestation of systemic lupus erythematosus (SLE), characterized by the simultaneous occurrence of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. This condition presents a major diagnostic and therapeutic challenge due to its acute onset, variable presentation, and high mortality. Early recognition, prompt immunologic evaluation, and aggressive immunosuppressive therapy are essential to prevent irreversible organ damage and improve survival.Methods Case ReportResults A 31-year-old man presented with fever, sore throat, a maculopapular trunk rash, and a malar rash. Laboratory findings showed normocytic anemia, elevated inflammatory markers, acute kidney injury with nephritic urine sediment, nephrotic-range proteinuria (20 g/day), and hypocomplementemia. The chest X-ray was initially normal. Immunologic testing revealed positive homogeneous ANA and high anti-dsDNA titers, while ANCA, anti-GBM, and anti-PLA2R antibodies were negative.During hospitalization, respiratory failure with hemoptysis developed. Chest CT demonstrated ground-glass opacities consistent with pulmonary hemorrhage. Kidney biopsy confirmed class IV lupus nephritis with rapidly progressive glomerulonephritis (>50% cellular and fibrocellular crescents) and marked tubulointerstitial inflammation.Treatment included high-dose intravenous methylprednisolone pulses, six plasmapheresis sessions, and two cycles of cyclophosphamide. Due to pulmonary deterioration, additional steroid pulses, rituximab, and mycophenolate mofetil were administered. The course was complicated by Enterococcus faecalis bacteremia and cytomegalovirus (CMV) reactivation, which was successfully treated with valganciclovir. Trimethoprim-sulfamethoxazole was started for Pneumocystis jirovecii prophylaxis. Supportive therapy included empagliflozin, telmisartan, and atorvastatin.After three months, renal function improved (eGFR >60 ml/min/1.73 m2), though proteinuria persisted (16.9 g/day), prompting initiation of voclosporin. After six months of combined therapy with voclosporin, mycophenolate, hydroxychloroquine, and low-dose prednisone, proteinuria decreased to 1.1 g/day with stable renal function. During follow-up, there was no evidence of disease exacerbation or recurrence of pulmonary or renal manifestations.Conclusions This case highlights the fulminant course of pulmonary-renal syndrome in SLE and the importance of early diagnosis and multimodal immunosuppressive therapy. Initial treatment with glucocorticoid pulses, rituximab, and plasmapheresis, followed by maintenance therapy with mycophenolate mofetil and voclosporin, can result in significant renal and pulmonary recovery. Continuous monitoring for infectious complications and appropriate prophylaxis are essential during intensive immunosuppression.