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Phys Topicsrespiratory

Phys · respiratory

Diffuse Alveolar Haemorrhage and Pulmonary-Renal Syndromes

Also known as DAH · diffuse alveolar hemorrhage · alveolar haemorrhage · pulmonary-renal syndrome · pulmonary renal syndrome · lung-kidney syndrome · Goodpasture syndrome · anti-GBM disease · pulmonary capillaritis · idiopathic pulmonary haemosiderosis

Consultant-physician-depth guide to diffuse alveolar haemorrhage — the haemoptysis-absent trap, capillaritis versus bland versus diffuse alveolar damage mechanisms, serial BAL diagnosis, the ANCA/anti-GBM/SLE pulmonary-renal differential, and evidence-based severe DAH management from pulse methylprednisolone to plasma exchange — structured for FRACP DWE and DCE preparation.

high19 referencesUpdated 17 July 202617 min readVerification in progress

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FRACP DWEFRACP DCEMRCP Part 2ABIM Internal Medicine

Red flags

  • Falling haemoglobin + new diffuse infiltrates + worsening hypoxaemia is DAH until proven otherwise — up to a third of patients have NO haemoptysis
  • Haemoptysis with haematuria or red-cell casts — pulmonary-renal syndrome: same-day ANCA, anti-GBM, ANA, complement and urine microscopy
  • Young smoker with DAH and acute kidney injury — anti-GBM disease: start plasma exchange on suspicion, not on biopsy
  • SLE with new infiltrates and falling Hb — lupus DAH carries high mortality; escalate early
  • New infiltrates in an anticoagulated patient — bland DAH from over-anticoagulation, not pneumonia
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FRACP DWEFRACP DCEMRCP Part 2ABIM Internal Medicine

Red flags

  • Falling haemoglobin + new diffuse infiltrates + worsening hypoxaemia is DAH until proven otherwise — up to a third of patients have NO haemoptysis
  • Haemoptysis with haematuria or red-cell casts — pulmonary-renal syndrome: same-day ANCA, anti-GBM, ANA, complement and urine microscopy
  • Young smoker with DAH and acute kidney injury — anti-GBM disease: start plasma exchange on suspicion, not on biopsy
  • SLE with new infiltrates and falling Hb — lupus DAH carries high mortality; escalate early
  • New infiltrates in an anticoagulated patient — bland DAH from over-anticoagulation, not pneumonia

Diffuse Alveolar Haemorrhage and Pulmonary-Renal Syndromes

The answer first

Diffuse alveolar haemorrhage (DAH) is bleeding into the alveolar spaces from the pulmonary microcirculation — a syndrome, not a diagnosis. The job is never just to recognise it; it is to find which of the immune, cardiac, toxic or coagulopathic causes is bleeding, because the treatments are opposites [1] [2]. Three rules carry you through the DWE and the DCE:

  1. Haemoptysis is not required. Up to a third of DAH episodes present without any haemoptysis. The real triad is a falling haemoglobin, new diffuse pulmonary infiltrates, and worsening hypoxaemia — in a ventilated ICU patient that combination, unexplained by pneumonia or pulmonary oedema, is DAH until bronchoscopy proves otherwise [1] [2].
  2. Bronchoscopy with serial lavage is the diagnostic move. Not another CT, not empiric antibiotics: bronchoalveolar lavage aliquots that become progressively bloodier, later confirmed by haemosiderin-laden macrophages [1].
  3. The urine finishes the examination. DAH plus an active urinary sediment (haematuria, red-cell casts, rising creatinine) is the pulmonary-renal syndrome — and the shortlist is ANCA-associated vasculitis, anti-GBM disease and SLE. Same-day serology decides the treatment: plasma exchange is the cornerstone for anti-GBM, while rituximab or cyclophosphamide anchors ANCA vasculitis — and after PEXIVAS, plasma exchange is no longer routine for ANCA disease [7] [12] [16].
The 30-second registrar answer

"Suspected DAH: falling haemoglobin, new infiltrates, hypoxaemia — haemoptysis optional. I confirm with serial BAL aliquots, send ANCA with MPO/PR3, anti-GBM, ANA, dsDNA and complement, dip and scope the urine, and echo for mitral stenosis. Severe disease gets ICU support, pulse methylprednisolone, and cause-specific therapy — plasma exchange for anti-GBM, rituximab or cyclophosphamide for ANCA vasculitis — while I correct coagulopathy and remove the trigger." [1] [16]


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References19ShowHide
  1. [1]Ioachimescu OC, Stoller JK Diffuse alveolar hemorrhage: diagnosing it and finding the cause Cleve Clin J Med, 2008.PMID 18491433
  2. [2]Lara AR, Schwarz MI Diffuse alveolar hemorrhage Chest, 2010.PMID 20442117
  3. [3]Hruskova Z, Casian AL, Konopasek P, et al. Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study Scand J Rheumatol, 2013.PMID 23374071
  4. [4]Kuang H, Jiang N, Jia XY, et al. Epidemiology, clinical features, risk factors, and outcomes in anti-glomerular basement membrane disease: A systematic review and meta-analysis Autoimmun Rev, 2024.PMID 38493958
  5. [5]Donaghy M, Rees AJ Cigarette smoking and lung haemorrhage in glomerulonephritis caused by autoantibodies to glomerular basement membrane Lancet, 1983.PMID 6140495
  6. [6]Johnson JP, Moore J Jr, Austin HA 3rd, et al. Therapy of anti-glomerular basement membrane antibody disease: analysis of prognostic significance of clinical, pathologic and treatment factors Medicine (Baltimore), 1985.PMID 3892220
  7. [7]Shin JI, Geetha D, Szpirt WM, et al. Anti-glomerular basement membrane disease (Goodpasture disease): From pathogenesis to plasma exchange to IdeS Ther Apher Dial, 2022.PMID 34339589
  8. [8]Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis N Engl J Med, 2010.PMID 20647199
  9. [9]Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis N Engl J Med, 2010.PMID 20647198
  10. [10]de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial Ann Intern Med, 2009.PMID 19451574
  11. [11]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
  12. [12]Walsh M, Merkel PA, Peh CA, et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis N Engl J Med, 2020.PMID 32053298
  13. [13]Jayne DRW, Merkel PA, Schall TJ, et al. Avacopan for the Treatment of ANCA-Associated Vasculitis N Engl J Med, 2021.PMID 33596356
  14. [14]Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis N Engl J Med, 2014.PMID 25372085
  15. [15]Smith RM, Jones RB, Specks U, et al. Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: an international randomised controlled trial Ann Rheum Dis, 2023.PMID 36958796
  16. [16]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
  17. [17]Zamora MR, Warner ML, Tuder R, et al. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome Medicine (Baltimore), 1997.PMID 9193454
  18. [18]Ioachimescu OC, Sieber S, Kotch A Idiopathic pulmonary haemosiderosis revisited Eur Respir J, 2004.PMID 15293620
  19. [19]Agrawal G, Agarwal R, Rohit MK, et al. Miliary nodules due to secondary pulmonary hemosiderosis in rheumatic heart disease World J Radiol, 2011.PMID 21390194

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