Pulmonary-renal syndrome

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Pulmonary-renal syndrome (PRS) is a rare medical syndrome in which respiratory failure involving bleeding in the lungs and kidney failure (glomerulonephritis) occur. [1] PRS is associated with a high rate of morbidity and death. [1] The term was first used by Goodpasture in 1919 to describe the association of respiratory and kidney failure. [1]

Contents

Causes

Pulmonary-renal syndromes are most commonly caused by an underlying autoimmune disease. PRS is most commonly due to ANCA-associated vasculitides (e.g., granulomatosis with polyangiitis) or due to anti-basement membrane diseases (e.g., Goodpasture's syndrome). Granulomatosis with polyangiitis usually presents with nasopharyngeal involvement as well, whereas Goodpasture's will not. Microscopic polyangiitis is the most common cause of pulmonary-renal syndrome.[ citation needed ]

Other causes include systemic lupus erythematosus, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, dermatomyositis, polymyositis, mixed connective tissue disease, poststreptococcal glomerulonephritis, rheumatoid arthritis, and systemic sclerosis. [1] Less common causes also include IgA vasculitis and cryoglobulinemic vasculitis.

Other etiologies include toxic injury such as paraquat poisoning, infection with hantavirus, leptospirosis, or legionella, or vascular, as seen in nephrotic syndrome when a renal vein thrombosis embolizes to the lungs.

Diagnosis

Differential diagnosis

Cardiogenic shock can mimic a pulmonary renal syndrome and lead to coughing up blood due to pulmonary edema and kidney failure from inadequate blood flow. [1]

Treatment

Treatment is primarily by corticosteroids and immunosuppressive medications like cyclophosphamide, methotrexate, and azathioprine. Plasmapheresis can be used in some circumstances.[ citation needed ]

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<span class="mw-page-title-main">Polyarteritis nodosa</span> Medical condition

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<span class="mw-page-title-main">Respiratory disease</span> Disease of the respiratory system

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<span class="mw-page-title-main">Rapidly progressive glomerulonephritis</span> Medical condition

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The following outline is provided as an overview of and topical guide to cardiology, the branch of medicine dealing with disorders of the human heart. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in cardiology are called cardiologists.

Pauci-immune vasculitis is a form of vasculitis that is associated with minimal evidence of hypersensitivity upon immunofluorescent staining for IgG. Often, this is discovered in the setting of the kidney.

Necrotizing vasculitis, also called systemic necrotizing vasculitus, is a category of vasculitis, comprising vasculitides that present with necrosis. Examples include giant cell arteritis, microscopic polyangiitis, and granulomatosis with polyangiitis. ICD-10 uses the variant "necrotizing vasculopathy". ICD-9, while classifying these conditions together, does not use a dedicated phrase, instead calling them "polyarteritis nodosa and allied conditions".

References

  1. 1 2 3 4 5 West, SC; Arulkumaran, N; Ind, PW; Pusey, CD (May 2013). "Pulmonary-renal syndrome: a life threatening but treatable condition". Postgraduate Medical Journal (Review). 89 (1051): 274–83. doi:10.1136/postgradmedj-2012-131416. PMID   23349383. S2CID   22646727.