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A 36-year-old man with prior hypersensitivity pneumonitis, glomerulosclerosis in remission, and a history of gallbladder cancer presents with sudden onset pleuritic chest pain, hemoptysis, and acute kidney injury. Exam shows hypoxemia, crackles, and an active urine sediment on labs. Imaging and bronchoscopy reveal alveolar hemorrhage. How would you approach the clinical evaluation, and what recent evidence informs diagnostic and management decisions in this pulmonary-renal syndrome? VIDEO INFO Category: Pulmonary-Renal Syndrome, Pauci-Immune Glomerulonephritis (GN), Glomerulonephritis: Causes, Diagnosis, and Management, Nephrology: Kidney Disease Diagnosis and Management Difficulty: Expert - Expert level - For those seeking deep understanding Question Type: Recent Changes Case Type: Complicated Condition Explore more ways to learn on this and other topics by going to https://endlessmedical.academy/auth?h... QUESTION A 36-year-old man with hypersensitivity pneumonitis diagnosed 4 years ago (reduced TLC, previously normal DLCO), biopsy-proven focal segmental glomerulosclerosis in remission since adolescence, and resected gallbladder adenocarcinoma (T1bN0) 3 years ago presents with 5 days of pleuritic chest pain, several teaspoons of hemoptysis daily, and oliguria.... OPTIONS A. Begin B-cell depletion with rituximab 1 g IV on days 1 and 15 in an inpatient infusion-capable setting, add avacopan 30 mg orally twice daily with a reduced-dose oral glucocorticoid taper, and avoid plasma exchange without dialysis requirement or anti-GBM positivity. B. Start rituximab 1 g IV on days 1 and 15 in a monitored infusion setting with a conventional high-dose prednisone taper and add therapeutic plasma exchange for diffuse alveolar hemorrhage despite negative anti-GBM testing and no dialysis requirement. C. Use rituximab 375 mg/m2 IV weekly for four doses in an inpatient infusion-capable setting with a conventional high-dose oral prednisone taper and omit avacopan; defer plasma exchange because anti-GBM is negative and kidney function does not require dialysis. D. Initiate off-label obinutuzumab 1,000 mg IV on days 1 and 15 as first-line induction in an inpatient infusion-capable setting with avacopan 30 mg twice daily and a reduced-dose prednisone regimen, foregoing plasma exchange given negative anti-GBM and stable oxygenation. CORRECT ANSWER A. Begin B-cell depletion with rituximab 1 g IV on days 1 and 15 in an inpatient infusion-capable setting, add avacopan 30 mg orally twice daily with a reduced-dose oral glucocorticoid taper, and avoid plasma exchange without dialysis requirement or anti-GBM positivity. EXPLANATION Begin B-cell depletion with rituximab 1 g IV on days 1 and 15 in an inpatient infusion-capable setting, add avacopan 30 mg orally twice daily with a reduced-dose oral glucocorticoid taper, and avoid plasma exchange without dialysis requirement or anti-GBM positivity. The foundational principle for organ-threatening ANCA-associated vasculitis with DAH and rapidly progressive pauci-immune GN is prompt induction with an effective remission agent plus minimized steroid exposure. Per KDIGO 2024 and the 2025 British Society for Rheumatology recommendations, rituximab is an accepted first-line induction alternative to cyclophosphamide. Avacopan, a C5a receptor inhibitor, enables a reduced-dose glucocorticoid regimen and was noninferior to standard prednisone for remission while improving kidney outcomes in ADVOCATE; guidelines now endorse avacopan to decrease glucocorticoid toxicity.... Further reading: Links to sources are provided for optional further reading only. The questions and explanations are independently authored and do not reproduce or adapt any specific third-party text or content. --------------------------------------------------- Our cases and questions come from the https://EndlessMedical.Academy quiz engine - multi-model platform. Each question and explanation is forged by consensus between multiple top AI models (i.e. Open AI GPT, Claude, Grok, etc.), with automated web searches for the latest research and verified references. Calculations (e.g. eGFR, dosages) are checked via code execution to eliminate errors, and all references are reviewed by several AIs to minimize hallucinations. Important note: This material is entirely AI-generated and has not been verified by human experts; despite stringent consensus checks, perfect accuracy cannot be guaranteed. Exercise caution - always corroborate the content with trusted references or qualified professionals, and never apply information from this content to patient care or clinical decisions without independent verification. Clinicians already rely on AI and online tools - myself included - so treat this content as an additional focused aid, not a replacement for proper medical education. Visit https://endlessmedical.academy for more AI-supported resources and cases. This material can not be treated as medical advice.