When pulmorenalen syndrome (PRS) often diffuse alveolar hemorrhage with glomerulonephritis occurs simultaneously. The cause is almost always an autoimmune disease. The diagnosis is made by serology and sometimes lung and kidney biopsy. Treatment usually includes immunosuppression with corticosteroids and cytotoxic drugs.
PRS is not a specific entity but a syndrome suggesting a differential diagnosis and a specific test sequence.
When pulmorenalen syndrome (PRS) often diffuse alveolar hemorrhage with glomerulonephritis occurs simultaneously. The cause is almost always an autoimmune disease. The diagnosis is made by serology and sometimes lung and kidney biopsy. Treatment usually includes immunosuppression with corticosteroids and cytotoxic drugs. PRS is not a specific entity but a syndrome suggesting a differential diagnosis and a specific test sequence. Pulmonary pathology is a vasculitis of small vessels, arterioles, venules and often affects alveolar capillaries. Renal Pathology] is a vasculitis of the small vessels, which leads to a form of focal segmental glomerulonephritis. Etiology PRS is almost always the manifestation of an underlying autoimmune disease. The classic cause is a Goodpasture’s syndrome. However, PRS can also be SLE, granulomatosis with polyangiitis, microscopic polyangiitis and rarely by other vasculitis, rheumatoid connective tissue disorders and substance-induced vasculitis: caused (such as propylthiouracil-see Table causes of pulmorenalen syndrome.). More rarely, the PRS is being built on the ground IgA-mediated diseases such as IgA nephropathy and IgA-associated vasculitis or immune complex mediated kidney diseases such as essential mixed cryoglobulinemia. Rarely has a rapidly progressive glomerulonephritis can cause pathological mechanisms by kidney failure, volume overload and pulmonary edema with hemoptysis own a PRS. Causes of pulmorenalen syndrome disease Examples connective tissue disorders dermatomyositis polymyositis progressive systemic sclerosis RA SLE Goodpasture’s syndrome – kidney disease Idiopathic immune complex glomerulonephritis, IgA nephropathy Rapidly progressive glomerulonephritis with heart failure Systemic vasculitis Behcet’s disease Cryoglobulinemia eosinophils granulomatosis microscopic polyangiitis with granulomatosis with polyangiitis IgA-associated vasculitis Polyarteritis Other substances (eg. As propylthiouracil) heart failure symptoms and discomfort symptoms and signs typically include dyspnoea cough fever hemoptysis Peripheral edema hematuria patients, other signs of glomerulonephritis have. Lung and kidney manifestations may be weeks or months apart. Tips risks we Pulmorenales syndrome should be considered in patients with findings that are compatible with alveolar hemorrhage and glomerulonephritis into consideration, even if the lung and kidney findings occur at different times. Diagnosis Serological tests Sometimes lung and kidney biopsies A PRS is suspected in patients with hemoptysis, which are not allocated to any other obvious cause (eg. As pneumonia, cancer or bronchiectasis), especially if the hemoptysis accompanied by diffuse parenchymal infiltrates and the findings kidney disease suggest. The initial diagnosis comprises a urine test for hematuria and cylinder of red blood cell (suggest glomerulonephritis), serum creatinine for detection of renal function and a blood image to the diagnosis of anemia. Provided that he is not yet available, a chest x-ray is taken. Antibody tests in serum can help to distinguish some causes, such as the following: antiglomerular basement membrane antibodies: Goodpasture’s syndrome antibodies against ds-DNA and reduction of Komplementlevels: SLE Antineutrophil cytoplasmic antibodies (ANCA), which (against proteinase 3 PR3 ANCA or cytoplasmic ANCA [c-ANCA]) are directed: granulomatosis with polyangiitis. ACNA to myeloperoxidase (MPO-ANCA or perinuclear ANCA [p-ANCA]): Microscopic polyangiitis The definitive diagnosis requires a lung biopsy with the finding of a small vessel vasculitis or renal biopsy finding of glomerulonephritis with or without antibody-Able Gerung. Pulmonary function tests and bronchoalveolar lavage are not a PRS diagnosis, but can be used to confirm a diffuse alveolar hemorrhage in patients with glomerulonephritis and pulmonary infiltrates without hemoptysis. Lavage fluid, which is bloody in successive lavages proves esp. With decreasing hematocrit, diffuse alveolar hemorrhage. Sometimes treatment corticosteroids cyclophosphamide plasma exchange The cornerstone of treatment is immunosuppression. A standard induction remission therapy includes pulse i.v. Methylprednisolone (500-1000 mg iv once a day for 3-5 days). When the life-threatening characteristics subside, the corticosteroid dose can be reduced. 1 mg / kg of prednisone (or equivalent) p.o. is given in the first month once a day, then tapered off over the next 3-4 months. Cyclophosphamide should be attached to the corticosteroid therapy for very sick patients with generalized disease at a dose of 0.5 to 1 g / m2 iv pulsates once a month or orally (1-2 mg / kg once daily). A plasma exchange is also used frequently, especially at Goodpasture syndrome and certain vasculitis. A transition to a maintenance therapy can use 6-12 months after the initiation of induction therapy or clinical remission. Maintenance therapy comprising low-dose corticosteroids coupled with cytotoxic substances. However, there can be relapses despite ongoing treatment. Summary The most striking indication PRS is often that patients have both unexplained lung and kidney symptoms, even if these symptoms occur at different times, routine laboratory tests (including urinalysis and chest X-ray) and autoantibody tests are performed. If necessary, the diagnosis is confirmed with a lung and kidney biopsy. The autoimmune diseases underlying is treated.