ICU · renal-metabolic
Pulmonary-Renal Syndrome in the ICU
Also known as Pulmonary renal syndrome · Diffuse alveolar haemorrhage · Rapidly progressive glomerulonephritis · ANCA-associated vasculitis · Anti-GBM disease · Goodpasture syndrome · Granulomatosis with polyangiitis · Microscopic polyangiitis · Crescentic glomerulonephritis · Pauci-immune glomerulonephritis · Pulmonary vasculitis with renal involvement
Pulmonary-renal syndrome is diffuse alveolar haemorrhage plus rapidly progressive glomerulonephritis. ANCA-associated vasculitis causes about 70 percent of cases and anti-GBM disease up to 20 percent. Diagnose clinically with ANCA, anti-GBM, complement and biopsy immunofluorescence (linear IgG vs pauci-immune vs granular). Treat with high-dose glucocorticoid plus rituximab or cyclophosphamide; plasma exchange is the anti-GBM gold standard and may be considered in ANCA RPGN after MEPEX and PEXIVAS.
Checked against its sources on 5 Sept 2026
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- Haemoptysis plus a rapidly rising creatinine is pulmonary-renal syndrome until ANCA and anti-GBM return
- Anti-GBM: plasma exchange is the gold-standard antibody-removal step — start with steroids and cyclophosphamide once the diagnosis is the working diagnosis; earlier exchange is associated with better renal recovery
- DAH can present without haemoptysis — up to 30 percent (Lara: up to one-third) — chase falling haemoglobin, bilateral alveolar filling, and sequential BAL
- Pauci-immune immunofluorescence is ANCA vasculitis, not anti-GBM — linear IgG on the GBM is anti-GBM
- DAH intubation is common (about half of DAH in the Boyle review) — suction-ready airway, not delayed serology
- Rituximab is non-inferior to cyclophosphamide in RAVE, with no significant difference in adverse-event rates in that trial; EULAR 2022 recommends high-dose glucocorticoid plus either agent for organ-threatening AAV
- Iodinated contrast is a nephrotoxic exposure in established RPGN/AKI — do not order it by default; Boyle notes CT angiography can still localise large-volume haemoptysis when that question changes management
Meet the patient
A 24-year-old male smoker turns up with two weeks of malaise and haemoptysis, now in type 1 respiratory failure. His creatinine has climbed from 120 to 340 micromol/L in a day, his haemoglobin has fallen from 132 to 88 g/L, and the chest X-ray is white-out bilateral. The urine dipstick reads blood 3+, protein 2+, and the anti-GBM titre returns strongly positive. [7]
The two questions that decide his next 24 hours are the two that decide every PRS: is this anti-GBM? (because plasma exchange is the gold-standard antibody-removal step, and earlier commencement is associated with better renal recovery) and how much kidney is left? (in the Hammersmith series every dialysis-dependent patient with 100 percent crescents on biopsy remained dialysis-dependent). Hold those two questions and the management below slots into place. [5][7]
References12ShowHide
- [1]Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis N Engl J Med, 2010.PMID 20647199
- [2]Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis N Engl J Med, 2010.PMID 20647198
- [3]Jayne DR, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis J Am Soc Nephrol, 2007.PMID 17582159
- [4]Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis N Engl J Med, 2020.PMID 32053298
- [5]Levy JB, Turner AN, Rees AJ, et al. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression Ann Intern Med, 2001.PMID 11388816
- [6]Lara AR, Schwarz MI Diffuse alveolar hemorrhage Chest, 2010.PMID 20442117
- [7]Boyle N, O'Callaghan M, Ataya A, et al. Pulmonary renal syndrome: a clinical review Breathe (Sheff), 2022.PMID 36865943
- [8]Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update Ann Rheum Dis, 2024.PMID 36927642
- [9]Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Arthritis Rheumatol, 2021.PMID 34235894
- [10]Tomelleri A, Dejaco C, Bond M To prophylax or not to prophylax? The role of trimethoprim/sulfamethoxazole as a prophylactic agent in systemic vasculitis Reumatismo, 2025.PMID 39945417
- [11]Rutgers A, Slot M, van Paassen P, et al. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis Am J Kidney Dis, 2005.PMID 16112043
- [12]Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis Ann Rheum Dis, 2022.PMID 35110334