A pulmonary renal syndrome is diagnosed when the combination of a rapid progressive glomerulonephritis (RPGN) and diffuse pulmonary hemorrhage (DAH) are present. Both are severe disease manifestations that are associated with a considerable morbidity and mortality. Most frequently, the underlying cause is a small vessel vasculitis due to anti-neutrophil cytoplasmatic antibody (ANCA) associated vasculitis (AAV) or anti-glomerular basement membrane disease. Other vasculitis’s and connective tissue diseases such as Systemic Lupus Erythematosus and Rheumatoid Arthritis present the more rare causes of this syndrome.
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Published online: February 24, 2023
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