Review article: Thoracic involvement in systemic primary vasculitis: radiological patterns and follow-up

Serra, Gloria; Al, Brun; Tolédano, Dan; Cluzel, Philippe; Pa, Grenier

doi:10.5334/jbr-btr.36

Cited by 1 publication

(1 citation statement)

References 23 publications

(18 reference statements)

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“…As hemorrhage is resorbed in the pulmonary interstitium, later imaging may show reticular or nodular appearances. 68 Bronchoalveolar lavage: Acute alveolar hemorrhage may be indicated by the finding of hemorrhagic bronchoalveolar lavage fluid, classically with increasing blood content on successive washes. After 2 to 3 days, alveolar macrophages convert hemoglobin to hemosiderin, and these hemosiderinladen cells may persist in the lung for several weeks, being evident on Perls staining of bronchoalveolar samples.…”

Section: Diagnosis Of Alveolar Hemorrhagementioning

confidence: 99%

Antiglomerular Basement Membrane Disease

McAdoo

Pusey

2018

Semin Respir Crit Care Med

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Antiglomerular basement membrane (anti-GBM) disease is a rare but life-threatening autoimmune vasculitis that is characterized by the development of pathogenic autoantibodies to type IV collagen antigens expressed in the glomerular and alveolar basement membranes. Once deposited in tissue, these autoantibodies incite a local capillaritis which manifests as rapidly progressive glomerulonephritis (GN) in 80 to 90% of patients, and with concurrent alveolar hemorrhage in ∼50%. A small proportion of cases may present with pulmonary disease in isolation. Serological testing for anti-GBM antibodies may facilitate rapid diagnosis, though renal biopsy is often required to confirm the presence of necrotizing or crescentic GN and linear deposition of autoantibody on the glomerular basement membrane. Alveolar hemorrhage may be evident clinically, or detected on imaging, pulmonary function testing, or bronchoscopy. Prompt treatment with plasmapheresis, cyclophosphamide, and steroids is usually indicated to remove pathogenic autoantibodies, to prevent their ongoing production, and to ameliorate end-organ inflammation. Alveolar hemorrhage is usually responsive to this treatment, and long-term respiratory sequelae are uncommon. Renal prognosis is more variable, though with aggressive treatment, independent renal function is maintained at 1 year in more than 80% of patients not requiring renal replacement therapy at presentation. Relapse in uncommon in anti-GBM disease, unless there is a concomitant antineutrophil cytoplasm antibody (present in 30–40%), in which case maintenance immunosuppression is recommended.

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Section: Diagnosis Of Alveolar Hemorrhagementioning

confidence: 99%