pulmonary renal syndrome

pulmonary renal syndrome

Nephrology An idiopathic condition characterized by pulmonary hemorrhage, immune crescent glomerulonephritis, and antineutrophil cytoplasmic antibodies; pulmonary-renal 'syndrome' may be defined as a heterogeneous group of multisystem diseases–eg, Goodpasture syndrome, Wegener's granulomatosus, collagen vascular disease–in particular SLE, polyarteritis nodosa, Henoch-Schönlein purpura, and various other conditions, which have prominent pulmonary and renal components and microangiopathic vasculitis Clinical Asymptomatic pulmonary infiltrates or pulmonary hemorrhage with episodic cough, hemoptysis, dyspnea and widespread alveolar infiltrates on CXR; renal involvement is characterized by microscopic hematuria, RBC casts, ↑ creatinine
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Keywords: Pulmonary renal syndrome (PRS), diffuse alveolar haemorrhages (DAH), acute kidney injury (AKI), Antineutrophil cytoplasmic autoantibody (ANCA) associated vasculitis, anti-glomerular basement membrane (GBM) disease, systemic lupus erythematosus (SLE).
The term pulmonary renal syndrome (PRS) was first described by Good pasture in 1919.
Chung et al., "Malignant hypertension with an unusual presentation mimicking the immune mediated pulmonary renal syndrome," Yonsei Medical Journal, vol.
Pulmonary renal syndrome in a patient with vasculitis: Case report and review of literature.
Pulmonary renal syndrome (PRS) hemorrhage is a well-known but lethal complication.
Bronchoscopy was not performed in this case, as the radiological, clinical, and pathological changes were attributed to a pulmonary renal syndrome (Fig.
GPS as a cause of pulmonary renal syndrome in childhood remains extremely rare.
Jayne, "Pulmonary renal syndrome: a 4-year, single-center experience," American Journal of Kidney Diseases, vol.
Clinically it can involve multiple systems, commonly pulmonary in 78%, upper respiratory tract in 86% and kidney in 61% of cases.1 Most of the patients (90%) seek medical attention for nasal and sinus symptoms with or without involvement of lower respiratory tract.2 Lower respiratory tract presents as hemorrhagic infiltrates and cavitating lesions leading to haemoptysis and respiratory failure in 45% of cases.1 Renal manifestations are haematuria, proteinuria and RPGN in 50% of cases while 25% of cases may reach end stage renal disease (ESRD) in 3-4 years.3 Our patient presented predominantly with pulmonary renal syndrome as lower respiratory tract involvement and RPGN.
Pulmonary capillaritis has been reported as a manifestation of Wegener granulomatosis, microscopic polyangiitis, systemic lupus erythematosus, Goodpasture syndrome, idiopathic pulmonary renal syndrome, Behcet syndrome, Henoch-Schonlein purpura, immunoglobulin A nephropathy, antiphospholipid syndrome, progressive systemic sclerosis, and diphenylhydantoin use.
Pulmonary renal syndromes encompass a wide variety of pathophysiologic entities, including antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), anti-glomerular basement membrane (GBM) disease, and other connective tissue diseases such as systemic lupus erythematosus.
Multiple diseases may produce pulmonary renal syndromes and renal failure, such as Wegener's granulomatosis, Henoch-Schonlein purpura, lupus nephritis, and polyarteritis nodosa.