Goodpasture's disease: A report of ten cases and a review of the literature

Dammacco, Franco; Battaglia, Stefano; Gesualdo, Loreto; Racanelli, Vito

doi:10.1016/j.autrev.2013.06.014

Cited by 63 publications

(58 citation statements)

References 70 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Antineutrophil cytoplasmic antibodies (ANCAs) are often associated with small vessel vasculitis and are directed against two abundant neutrophil proteins: MPO and proteinase 3. Although ANCA is defined by lack of immune deposits (pauci-immune) in target organs, ANCA antibodies may also copresent in immune complex diseases including anti-glomerular basement membrane (GBM) disease, lupus, drug-induced vasculitis, and rheumatoid arthritis (Short et al, 1995;Bart unková et al, 2003;Yang et al, 2007;Dammacco et al, 2013).…”

Section: Introductionmentioning

confidence: 99%

PF-1355, a Mechanism-Based Myeloperoxidase Inhibitor, Prevents Immune Complex Vasculitis and Anti–Glomerular Basement Membrane Glomerulonephritis

Zheng

Warner

Ruggeri

et al. 2015

J Pharmacol Exp Ther

View full text Add to dashboard Cite

Small vessel vasculitis is a life-threatening condition and patients typically present with renal and pulmonary injury. Disease pathogenesis is associated with neutrophil accumulation, activation, and oxidative damage, the latter being driven in large part by myeloperoxidase (MPO), which generates hypochlorous acid among other oxidants. MPO has been associated with vasculitis, disseminated vascular inflammation typically involving pulmonary and renal microvasculature and often resulting in critical consequences. MPO contributes to vascular injury by 1) catabolizing nitric oxide, impairing vasomotor function; 2) causing oxidative damage to lipoproteins and endothelial cells, leading to atherosclerosis; and 3) stimulating formation of neutrophil extracellular traps, resulting in vessel occlusion and thrombosis. Here we report a selective 2-thiouracil mechanism-based MPO inhibitor (PF-1355 [2-(6-(2,5-dimethoxyphenyl)-4-oxo-2-thioxo-3,4-dihydropyrimidin-1(2H)-yl)acetamide) and demonstrate that MPO is a critical mediator of vasculitis in mouse disease models. A pharmacokinetic/pharmacodynamic response model of PF-1355 exposure in relation with MPO activity was derived from mouse peritonitis. The contribution of MPO activity to vasculitis was then examined in an immune complex model of pulmonary disease. Oral administration of PF-1355 reduced plasma MPO activity, vascular edema, neutrophil recruitment, and elevated circulating cytokines. In a model of anti-glomerular basement membrane disease, formerly known as Goodpasture disease, albuminuria and chronic renal dysfunction were completely suppressed by PF-1355 treatment. This study shows that MPO activity is critical in driving immune complex vasculitis and provides confidence in testing the hypothesis that MPO inhibition will provide benefit in treating human vasculitic diseases.

show abstract

Section: Introductionmentioning

confidence: 99%

PF-1355, a Mechanism-Based Myeloperoxidase Inhibitor, Prevents Immune Complex Vasculitis and Anti–Glomerular Basement Membrane Glomerulonephritis

Zheng

Warner

Ruggeri

et al. 2015

J Pharmacol Exp Ther

View full text Add to dashboard Cite

show abstract

“…Symptoms may either start slowly, gradually affecting kidneys and lungs, or they may progress rapidly, and become severe in a few days [8]. There are essential variations in the clinical manifestations of patients with anti-GBM disease, with 60-80% having clinically apparent pulmonary and renal involvement, 20-40% having renal disease only, and less than 10% having only pulmonary manifestation [9].…”

Section: Discussionmentioning

confidence: 99%

Goodpasture’s Syndrome With Ambiguous Serology: A Case Report

Szczykowska

Brzósko

Rakowska

et al. 2017

World J Nephrol Urol

View full text Add to dashboard Cite

Pulmonary-renal syndrome is a rare clinical syndrome defined by a combination of diffuse alveolar hemorrhage and rapidly progressive crescentic glomerulonephritis. It is not a single entity, but is caused by a wide variety of diseases including Goodpasture's syndrome associated with autoantibodies against glomerular and alveolar basement membrane (anti-glomerular basement membrane (anti-GBM)). Anti-GBM disease is an autoimmune disorder, estimated to occur in less than one case per million population. A 59-year-old previously healthy man presented with acute kidney injury (AKI, stage 3, KDIGO) with signs of nephritic syndrome, progressive oliguria, dyspnea with mild hemoptysis, cruses edema and elevated blood pressure. Serologic results were all negative except very low titer of anti-proteinase 3 antibodies. Chest radiograph revealed diffuse pulmonary infiltrations. Renal biopsy demonstrated necrotizing crescentic glomerulonephritis with linear pattern for immunoglobulin G along the entire GBM in immunofluorescent staining. Based on whole clinical picture, the diagnosis of Goodpasture's syndrome was done and the intensive treatment, including plasmapheresis, cyclophosphamide and prednisone, along with hemodialysis was provided. Improvement of patient's general condition was achieved. Despite recovery of diuresis, attempt to discontinue hemodialysis was found unsuccessful. The case proves the importance of diagnostic vigilance in patients presenting with AKI. Although supportive, in many cases, serology tests can be negative or ambiguous and should not prevent profound diagnostic process including kidney biopsy. Late diagnosis in the course of anti-GBM disease, in spite of proper treatment, correlates with less favorable kidney outcomes.

show abstract

“…(12,13) The characteristic butterfly rash or other skin lesions such as alopecia in SLE may also be found. (14) If autoimmune disease is suspected, the patient should be referred to a specialist centre for further evaluation.…”

Section: Autoimmune Causesmentioning

confidence: 99%