Pre-existing chronic interstitial pneumonia is a poor prognostic factor of Goodpasture’s syndrome: a case report and review of the literature
© The Author(s). 2017
Received: 9 September 2016
Accepted: 23 March 2017
Published: 13 April 2017
Goodpasture’s syndrome is a rare disease that is characterized by rapidly progressive glomerulonephritis and diffuse alveolar hemorrhage.
A 71-year-old Japanese man who had chronic interstitial pneumonia was diagnosed as having Goodpasture’s syndrome. Both anti-glomerular basement membrane antibody and myeloperoxidase anti-neutrophil cytoplasmic antibody were increased. Despite intensive treatments, including mechanical ventilation, he died from respiratory failure. Pathological findings at autopsy showed rapidly progressive glomerulonephritis in his kidneys, diffuse alveolar hemorrhage, hyaline membranes, and fibroblastic foci in his lungs. The cause of death was diagnosed as respiratory failure as a result of diffuse alveolar damage induced by a combination of diffuse alveolar hemorrhage and exacerbation of interstitial pneumonia.
We report a case of Goodpasture’s syndrome complicated with pre-existing chronic interstitial pneumonia and positive myeloperoxidase anti-neutrophil cytoplasmic antibody. We reviewed six similar cases reported in the literature and concluded that Goodpasture’s syndrome with pre-existing interstitial pneumonia and myeloperoxidase anti-neutrophil cytoplasmic antibody is related to a poor prognosis.
KeywordsGoodpasture’s syndrome Interstitial pneumonia Anti-glomerular basement membrane antibody Myeloperoxidase anti-neutrophil cytoplasmic antibody
Goodpasture’s syndrome (GPS) is a rare autoimmune disease that is related to anti-glomerular basement membrane (anti-GBM) antibodies . Clinical manifestations are characterized by rapidly progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH) . The incidence of GPS is estimated to be one case per million per year . The pathogenesis of GPS is thought to be related to circulating anti-GBM antibodies inducing RPGN and DAH; however, the mechanism of anti-GBM antibody production is not fully understood . Although treatments with plasmapheresis, corticosteroids, and immunosuppressive agents have improved the prognosis, some patients still die as the disease progresses . Poor prognostic factors related to GPS have been reported to be positive myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and elevated serum creatinine levels; however, others have not been clearly elucidated [6, 7].
Here we report a rare case of GPS complicated with pre-existing chronic interstitial pneumonia. We reviewed six similar cases reported previously and estimated that GPS with pre-existing interstitial pneumonia and positive MPO-ANCA are related to poor clinical outcomes.
GPS is a lung-specific and kidney-specific autoimmune disease that is related to anti-GBM antibodies . Clinical manifestations include acute renal failure caused by RPGN and acute respiratory failure caused by DAH . GPS complicated with pre-existing chronic interstitial pneumonia, as seen in our case, is certainly rare.
Characteristics of patients with Goodpasture’s syndrome complicated with pre-existing interstitial pneumonia
Smoking (pack year)
Type of interstitial pneumonia
Duration from onset of IP to GPS
Cause of death
CS, PE, HD, MV
CS, PE, HD
CS, CY, PE, HD, MV
CS, PE, HD, MV
The pathogenesis of GPS is not fully understood. The target GBM antigen molecule was subsequently identified as the noncollagenous-1 (NC1) domain of the α3 chain of collagen IV . In addition, environmental factors are also thought to increase the risk of the disease, for instance, respiratory infection by influenza virus, exposure to hydrocarbon fumes or metallic dust, and tobacco smoking .
Of interest, almost all of the seven cases we reviewed were positive for serum MPO-ANCA and six of the seven patients had a severe tobacco smoking habit. Pre-existing chronic interstitial pneumonia might be a trigger for the production of MPO-ANCA because MPO-ANCA is known to be positive prior to the development of microscopic polyangiitis (MPA) . In addition, MPO-ANCA-positive interstitial pneumonia and cigarette smoking might be a factor associated with the induction of positive anti-GBM antibody. Moreover, RPGN and positive anti-GBM antibody is reported to be a poor prognostic factor in patients with MPA [18, 19]. According to these findings, we propose that MPO-ANCA-positive interstitial pneumonia is the related lung manifestation of GPS, not just a coexisting disease, and both MPO-ANCA and pre-existing interstitial pneumonia are related to poor clinical outcomes for patients with GPS.
We report a case of GPS complicated with pre-existing chronic interstitial pneumonia and positive MPO-ANCA. The cause of death was diagnosed as respiratory failure as a result of diffuse alveolar damage induced by a combination of DAH and exacerbation of interstitial pneumonia. We conclude that pre-existing interstitial pneumonia and a positive MPO-ANCA are important prognostic factors in the clinical outcomes of patients with GPS, and chronic interstitial pneumonia with MPO-ANCA may be related to the pathogenesis of GPS.
Diffuse alveolar hemorrhage
Glomerular basement membrane
- HCO3 :
Myeloperoxidase anti-neutrophil cytoplasmic antibody
- pCO2 :
Partial pressure of carbon dioxide
- pO2 :
Partial pressure of oxygen
Rapidly progressive glomerulonephritis
Usual interstitial pneumonia
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Data sharing is not applicable to this article as no datasets were generated.
HT was responsible for data collection, interpretation, and drafting of the manuscript. KT drafted and revised the manuscript. YI revised the manuscript. SU revised the manuscript. MF revised the manuscript. MM revised the manuscript. SK revised the manuscript. NSA reviewed and revised the manuscript. All authors read and approved the final manuscript.
The authors declare that they have no competing interests.
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