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A 60-year-old woman with limited cutaneous systemic sclerosis presents with progressive shortness of breath, pleuritic chest pain, gross hematuria, and hemoptysis. She has elevated creatinine, proteinuria, and new oxygen needs. Initial labs find anemia, RBC casts, and positive MPO-ANCA. How should clinicians approach the next diagnostic step for acute pulmonary-renal syndrome in this complex patient? What clues help distinguish critical etiologies in this scenario? VIDEO INFO Category: Therapeutic Immunosuppression, 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: Diagnostic Step Case Type: Tricky Findings Explore more ways to learn on this and other topics by going to https://endlessmedical.academy/auth?h... QUESTION A 60-year-old female graduate student with limited cutaneous systemic sclerosis (scleroderma) presents with 3 days of progressive dyspnea, pleuritic chest pain, new gross hematuria, and fatigue. She reports two episodes of epistaxis and a transient, non-productive cough with scant hemoptysis this morning. She denies fever and has had poor oral intake. She has no history of chronic kidney disease.... OPTIONS A. Perform an urgent percutaneous native-kidney biopsy with light microscopy, immunofluorescence, and electron microscopy to confirm pauci-immune necrotizing crescentic GN versus scleroderma-associated TMA, without delaying stabilization, while serologies (including anti-GBM) continue to return. B. Measure plasma renin activity and start captopril for presumed scleroderma renal crisis, deferring kidney biopsy until after a week of ACE-inhibitor titration and blood pressure control. C. Repeat high-resolution CT of the chest and bronchoscopy with lavage to further characterize alveolar hemorrhage before considering any renal tissue diagnosis. D. Rely on ANCA titers and complement levels alone to infer pauci-immune GN and begin pulse methylprednisolone immediately without histologic confirmation. CORRECT ANSWER A. Perform an urgent percutaneous native-kidney biopsy with light microscopy, immunofluorescence, and electron microscopy to confirm pauci-immune necrotizing crescentic GN versus scleroderma-associated TMA, without delaying stabilization, while serologies (including anti-GBM) continue to return. EXPLANATION The single best next diagnostic step is tissue confirmation of the renal lesion to discriminate pauci-immune necrotizing crescentic GN from scleroderma-associated thrombotic microangiopathy or other mimics. Perform an urgent percutaneous native-kidney biopsy with light microscopy, immunofluorescence, and electron microscopy to confirm pauci-immune necrotizing crescentic GN versus scleroderma-associated TMA, without delaying stabilization, while serologies (including anti-GBM) continue to return. directly accomplishes this. The patient has systemic sclerosis, hypoxemia with bilateral ground glass, active urine sediment with dysmorphic RBCs and casts, rapid creatinine rise, normal complements, and MPO-ANCA positivity-all features consistent with a pulmonary-renal syndrome in which immediate histologic typing dictates treatment and steroid strategy. KDIGO 2024 and the 2021 ACR/VF guideline emphasize the role of biopsy in rapidly progressive GN to distinguish AAV from mimics and to guide immunosuppression. Alternatives are inferior or unsafe.... 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. May contain errors. -------------