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Pulmonary-renal syndrome
Last reviewed: 12.07.2025
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Pulmonary-renal syndrome (PRS) is a combination of diffuse alveolar pulmonary hemorrhage and glomerulonephritis.
[ What causes pulmonary renal syndrome?
Pulmonary-renal syndrome is always a manifestation of the underlying autoimmune disease, but it is beginning to be identified as a separate nosological entity, since it requires differential diagnosis and a specific sequence of studies and treatment. Goodpasture's syndrome is the classic variant, but pulmonary-renal syndrome can also be caused by systemic lupus erythematosus, Wegener's granulomatosis, microscopic polyangiitis, and, less commonly, other vasculitides and systemic connective tissue diseases. The number of cases of pulmonary-renal syndrome caused by these latter diseases is probably greater than those caused by Goodpasture's syndrome, but patients with these diseases more often have other clinical manifestations; only a few have manifestations in the form of pulmonary-renal syndrome.
Pulmonary-renal syndrome is less often a manifestation of IgA nephropathy or Henoch-Schonlein purpura, as well as essential mixed cryoglobulinemia, which are based on the damaging effect of IgA deposits on the kidneys. Rarely, rapidly progressive glomerulonephritis itself can lead to the development of pulmonary-renal syndrome. This occurs due to the mechanism of renal failure, volume overload and pulmonary edema with hemoptysis.
Symptoms of pulmonary-renal syndrome
Pulmonary-renal syndrome is suspected in patients with hemoptysis apparently unrelated to other causes (eg, pneumonia, cancer, or bronchiectasis), especially when hemoptysis is associated with scattered parenchymal infiltrates.
Diagnosis of pulmonary-renal syndrome
Initial studies include urinalysis to detect hematuria, serum creatinine to evaluate renal function, and complete blood count to evaluate anemia. Pulmonary function tests are not diagnostic, but an elevated diffusing volume of carbon monoxide (DLCO) is suggestive of pulmonary hemorrhage; this is due to increased uptake of carbon monoxide by intraalveolar hemoglobin.
Differential diagnosis of pulmonary-renal syndrome
- Connective tissue diseases
- Polymyositis or dermatomyositis
- Progressive systemic sclerosis
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Goodpasture's syndrome
- Kidney diseases
- Idiopathic immune complex glomerulonephritis
- IgA nephropathy
- Rapidly progressive glomerulonephritis with heart failure
- Systemic vasculitis
- Behcet's syndrome
- Churg-Strauss syndrome
- Cryoglobulinemia
- Henoch-Schönlein purpura
- Microscopic polyarteritis
- Wegener's granulomatosis
- Medicines (penicillamine)
- Heart failure
Serum antibody testing may help identify some causes. Anti-glomerular basement membrane antibodies (anti-GBM antibodies) are pathognomonic of Goodpasture syndrome, although they also occur in patients with Alport syndrome after renal transplantation. Anti-double-stranded DNA antibodies and decreased serum complement levels are typical of SLE. Antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase-3 (PR3-ANCA or cytoplasmic ANCA [c-ANCA]) are present in Wegener granulomatosis. Antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA or perinuclear ANCA [p-ANCA]) suggest microscopic polyangiitis.