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Pulmonary-renal syndrome
Last reviewed: 23.04.2024
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Pulmonary-renal syndrome (LPS) is a combination of diffuse alveolar pulmonary hemorrhage and glomerulonephritis.
[What causes pulmonary-kidney syndrome?
Pulmonary-renal syndrome is always a manifestation of the main autoimmune disease, but it begins to be isolated into a separate nosological form, as it involves differential diagnosis and a certain sequence of research and treatment. Goodpasture syndrome is a classic variant, but pulmonary-renal syndrome can also be caused by systemic lupus erythematosus, Wegener's granulomatosis, microscopic polyangiitis and, more rarely, other vasculitis and systemic connective tissue diseases. The number of cases of pulmonary-kidney syndrome caused by these latter diseases is probably greater than that caused by Goodpasture's syndrome, but patients with these diseases often have other clinical manifestations; only a few have manifestations in the form of pulmonary-kidney syndrome.
Pulmonary-renal syndrome is less often the manifestation of IgA-nephropathy or purpura of Shenlaine-Genoch, as well as of essential mixed cryoglobulinemia, which is based on the damaging effect on the kidneys of IgA deposits. Rarely, the development of pulmonary-renal syndrome can lead to a rapidly progressive glomerulonephritis in itself. This is due to the mechanism of renal failure, volume overload and pulmonary edema with hemoptysis.
Symptoms of Pulmonary-Kidney Syndrome
Pulmonary-renal syndrome is suspected in patients with hemoptysis, apparently not associated with other causes (for example, pneumonia, cancer or bronchiectasis), especially when hemoptysis is combined with scattered parenchymal infiltrates.
Diagnosis of pulmonary-kidney syndrome
Initial studies include urine analysis to detect hematuria, serum creatinine to clarify renal function, and a clinical blood test to detect anemia. Lung function tests are not diagnostic, but the detection of an increased diffusion volume of carbon monoxide (DLCO) indicates pulmonary hemorrhage; this is due to the increased uptake of carbon monoxide by intra-alveolar hemoglobin.
Differential Diagnosis of Pulmonary-Kidney Syndrome
- Diseases of connective tissue
- Poliomyositis or dermatomyositis
- Progressive systemic sclerosis
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Goodpasture Syndrome
- Kidney Diseases
- Idiopathic Immunocomplex Glomerulonephritis
- lgA-nephropathy
- Rapidly progressive glomerulonephritis with heart failure
- Systemic vasculitis
- Behcet's Syndrome
- Czurdzha Strauss Syndrome
- Cryoglobulinemia
- Purple Shenlaine-Genocha
- Microscopic polyarteritis
- Wegener's granulomatosis
- Drugs (penicillamine)
- Heart failure
Identification of some reasons may contribute to the study of serum antibodies. Antibodies to the basal glomerular membrane (anti-EBM antibodies) are pathognomonic for Goodpasture's syndrome, although they are also found in patients with Alport syndrome after renal transplantation. Antibodies to double-stranded DNA and reduced levels of serum complement are typical for SLE. Antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase-3 (PR3-ANCA or cytoplasmic ANCA [c-ANCA]) are present in Wegener's granulomatosis. Antineutrophil cytoplasmic antibodies to myeloperoxidase (MPO-ANCA, or perinuclear ANCA [p-ANCA]) suggest the presence of microscopic polyangiitis.