Diffuse Alveolar Haemorrhage

OVERVIEW

Diffuse alveolar hemorrhage is an acute, life-threatening event, and repeated episodes can lead to organizing pneumonia, collagen deposition in small airways, and, ultimately, fibrosis

  • difficult to diagnose and uncommon, requiring a high index of suspicion

CAUSES

There are 3 general patterns of diffuse alveolar hemorrhage

Vasculitis or capillaritis

  • Granulomatosis with polyangiitis (GPA)
  • Microscopic polyangiitis
  • Goodpasture syndrome
  • Isolated pauci-immune pulmonary capillaritis
  • Henoch-Schönlein purpura
  • immunoglobulin A nephropathy
  • Pauci-immune glomerulonephritis
  • immune complex-associated glomerulonephritis
  • Urticaria-vasculitis syndrome
  • Connective tissue disorders
  • Antiphospholipid antibody syndrome
  • Cryoglobulinemia
  • Behçet syndrome
  • Acute lung-graft rejection
  • Thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura

Bland’ pulmonary hemorrhage (ie, without capillaritis or vasculitis)

  • Anticoagulants, antiplatelet agents, or thrombolytics; disseminated intravascular coagulation
  • Mitral stenosis and mitral regurgitation
  • Pulmonary veno-occlusive disease
  • Infection: human immunodeficiency virus infection, infective endocarditis
  • Toxins: trimellitic anhydride, isocyanates, crack cocaine, pesticides, detergents
  • Drugs: propylthiouracil, diphenylhydantoin, amiodarone, mitomycin, D-penicillamine, sirolimus, methotrexate, haloperidol, nitrofurantoin, gold, all-trans-retinoic acid , bleomycin (especially with high oxygen concentrations), montelukast, zafirlukast, infliximab
  • Idiopathic pulmonary hemosiderosis

Alveolar bleeding associated with another process or condition

  • Diffuse alveolar damage
  • Pulmonary embolism
  • Sarcoidosis
  • High-altitude pulmonary edema, barotrauma
  • Infection: invasive aspergillosis, CMV infection, legionellosis, herpes simplex virus infection, mycoplasmosis, hantavirus infection, leptospirosis, other bacterial pneumoniae
  • Malignant conditions (pulmonary angiosarcoma, Kaposi sarcoma, multiple myeloma, acute promyelocytic leukemia) Lymphangioleiomyomatosis
  • Tuberous sclerosis
  • Pulmonary capillary hemangiomatosis
  • Lymphangiography

CLINICAL FEATURES

  • alveolar bleeding alone +/- features of the underlying cause (e.g. rash, purpura, eye lesions, hepatosplenomegaly, clubbing)
  • dyspnea, cough, and fever usually acute/ subacute (<1 week)
  • may cause severe acute respiratory distress requiring mechanical ventilation
  • haemoptysis (absent in a third due to large alveolar volume)

INVESTIGATIONS

Bedside

Laboratory

  • Acute or chronic anemia
  • Leukocytosis
  • Elevated ESR and CRP
  • UEC and urinalysis – ? pulmonary-renal syndrome e.g. Henoch-Schönlein Purpura, Goodpastures, GPA
  • autoimmune screen
  • biopsies e.g. lung (e.g. MPO-ANCA positive or PR3-ANCA positive) , renal

Imaging

  • CXR – patchy alveolar opacification (may appear normal!)
  • CT chest – areas of consolidation and ground glass changes interspersed with ‘normal’ areas

Special tests

  • bronchoscopy – BAL, document alveolar haemorhage (frank blood, >5% haemosiderin-laden macrophages), exclude airway source, exclude infection
  • Pulmonary function tests – high DLCO, usually restrictive > obstructive pattern, low exhaled NO

MANAGEMENT

  • ABCs
  • treat underlying cause
  • stop suspected contributory medications
  • often treated with corticosteroids e.g. IV methylprednisolone 500mg q6h for 5 days followed by tapered oral course)
  • immunosuppresants used as second line or where indicated for select conditions (e.g. cyclophosphamide 2mg/kg/d)
  • consider plasmapheresis if Ig-mediated (e.g. Goodpastures)
  • Factor VII needs further evaluation

COMPLICATIONS

  • death
  • side-effects of treatment
  • organising pneumonia
  • pulmonary fibrosis

PROGNOSIS

  • varies according to underlying cause
  • 2 year survival ranges from 20 to 90% depending on the cause

References and Links

Journal articles and textbooks

  • Ioachimescu OC, Stoller JK. Diffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med. 2008 Apr;75(4):258, 260, 264-5 passim. Review. PubMed PMID: 18491433.[Free Fulltext]
  • West S, Arulkumaran N, Ind PW, Pusey CD. Diffuse alveolar haemorrhage inANCA-associated vasculitis. Intern Med. 2013;52(1):5-13. Epub 2013 Jan 1. Review.PubMed PMID: 23291668. [Free Full Text]

CCC 700 6

Critical Care

Compendium

Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also the Innovation Lead for the Australian Centre for Health Innovation at Alfred Health, a Clinical Adjunct Associate Professor at Monash University, and the Chair of the Australian and New Zealand Intensive Care Society (ANZICS) Education Committee. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.

After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.

He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE.  He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.

His one great achievement is being the father of two amazing children.

On Twitter, he is @precordialthump.

| INTENSIVE | RAGE | Resuscitology | SMACC

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