Diffuse alveolar hemorrhage in inflammatory rheumatic diseases
Authors:
P.. Bradna
Authors‘ workplace:
II. interní gastroenterologická klinika LF UK a FN, Hradec Králové
Published in:
Čes. Revmatol., 31, 2023, No. 4, p. 190-196.
Category:
Review Article
Overview
Diffuse alveolar hemorrhage (DAH) is a life-threatening complication, especially of systemic vasculitis, but also of other connective tissue diseases. The trick is the fact that for some patients, a DAH attack is the first manifestation of the disease. A typical triad of symptoms includes rapidly developing dyspnea, anemization, and the presence of diffuse alveolar hemorrhage on bronchoalveolar lavage examination. Hemoptysis is not a reliable indicator, it occurs only in a third to a half of patients. HRCT will show extensive clouding of the lung fields, usually bilateral. The acute prognosis depends on the timeliness of the diagnosis or initiation of intensive immunomodulating therapy. Urgent determination of the presence of ANCA antibodies (immediately) can be of significant help. Acute bronchoscopy with BAL is an essential part of the diagnosis.
Treatment of an acute attack of DAH consists of i.v. administration of pharmacological doses of methylprednisolone and early initiation of i.v. cyclophosphamide or rituximab therapy according to usual schemes. Concomitant rapidly progressing anuric renal failure occurs in approximately half of patients with a severe course and requires the use of elimination methods. Overcoming respiratory failure may require the temporary use of extracorporeal oxygenation (ECMO). If the acute phase of DAH can be managed, the restitution of lung and renal functions is significant, and chronic dialysis is required less often. In the further course, the prognosis of the patient probably does not differ significantly from the prognosis of the underlying disease.
Due to the significant number of patients in whom the attack of DAH is the first manifestation of a systemic disease, the possibility of DAH should be considered in any case of rapidly developing severe respiratory insufficiency without an obvious previous cause.
Keywords:
Systemic lupus erythematosus – glucocorticoids – granulomatosis with polyangiitis – diffuse alveolar hemorrhage – cyclophosphamide, rituximab – bronchoalveolar la-vage
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