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A retrospective study of microscopic polyangiitis patients presenting with pulmonary fibrosis in China.

Huang H, Wang YX, Jiang CG, Liu J, Li J, Xu K, Xu ZJ - BMC Pulm Med (2014)

Bottom Line: One of the remaining patients progressed slowly, whereas six died.Most patients had a delayed positive ANCA.Corticosteroids plus cyclophosphamide was the remission-induction treatment scheme for all cases.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Beijing, Dongcheng District 100730, China. xuzj@hotmail.com.

ABSTRACT

Background: Pulmonary involvement is a common feature of MPA. Although alveolar hemorrhage is the most common pulmonary manifestation of MPA, a few recent studies have described instances of MPA patients with pulmonary fibrosis. Pulmonary fibrosis was seen to predate, be concomitant with, or occur after the diagnosis of MPA. The goal of this study was to describe the clinical features and prognosis of microscopic polyangiitis (MPA) patients whose initial respiratory presentation was pulmonary fibrosis.

Methods: We conducted a retrospective analysis of 19 MPA patients who presented with pulmonary fibrosis at Peking Union Medical College Hospital between 1990 and 2012.

Results: Of 67 total MPA cases, 19 patients presented with pulmonary fibrosis. There were 8 males and 11 females, with a median age of 63.6 years. Common clinical manifestations included fever (89.5%), cough (84.2%), dyspnea (78.9%) and velcro rales (84.2%). Eleven patients experienced weight loss, several had kidney involvement, and most had an increased erythrocyte sedimentation rate and C-reactive protein. All were positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA), with 6 patients being positive at the time of their initial diagnosis of pulmonary fibrosis. Every patient had typical features of usual interstitial pneumonia on High-resolution CT. All were treated with corticosteroids and cyclophosphamide, which lead to an improvement in twelve cases. One of the remaining patients progressed slowly, whereas six died.

Conclusions: Patients with MPA, who also presented with pulmonary fibrosis in our cohort, were more likely to be older, female, and have extrapulmonic involvement. Most patients had a delayed positive ANCA. Corticosteroids plus cyclophosphamide was the remission-induction treatment scheme for all cases. The current prognosis for MPA patients with pulmonary fibrosis appears to be poor, suggesting that they may be candidates for new therapies.

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Related in: MedlinePlus

Patient with pulmonary fibrosis had positive ANCA at her initial screening: A 64-year-old woman complained of cough and exertional dyspnea. She had UIP-pattern on chest CT: reticular opacities and honeycombing with basal and peripheral distribution. But her ANCA showed a myeloperoxidase of 196 EU and she was diagnosed with MPA. She was then treated with corticosteroids and cyclophosphamide and had a good outcome.
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Figure 1: Patient with pulmonary fibrosis had positive ANCA at her initial screening: A 64-year-old woman complained of cough and exertional dyspnea. She had UIP-pattern on chest CT: reticular opacities and honeycombing with basal and peripheral distribution. But her ANCA showed a myeloperoxidase of 196 EU and she was diagnosed with MPA. She was then treated with corticosteroids and cyclophosphamide and had a good outcome.

Mentions: Since all 19 patients were diagnosed with MPA after 2004, they all had high-resolution CT images (HRCT; FigureĀ 1 and 2) (Informed written consent was obtained for the publication of individual personal information from these two patients). The most common feature in each patient was reticular shadows, with approximately 40-70% of the whole lung involved. Additionally, some patients (10/19) had honeycomb lesions (approximately 5-31% of the whole lung), and 2 patients had minimal ground-glass opacities (both were less than 5%). All of the shadows were predominant in both lower lobes of the lungs and had outer zone predominance. Eleven patients received pulmonary function testing (PFT) upon the diagnosis of pulmonary fibrosis. Among them, PFT disclosed a restrictive pattern of ventilation and decreased gas diffusion, with a total lung capacity from 48.2% to 72.8% and a carbon monoxide transfer factor (DLCO) from 30% to 76%.


A retrospective study of microscopic polyangiitis patients presenting with pulmonary fibrosis in China.

Huang H, Wang YX, Jiang CG, Liu J, Li J, Xu K, Xu ZJ - BMC Pulm Med (2014)

Patient with pulmonary fibrosis had positive ANCA at her initial screening: A 64-year-old woman complained of cough and exertional dyspnea. She had UIP-pattern on chest CT: reticular opacities and honeycombing with basal and peripheral distribution. But her ANCA showed a myeloperoxidase of 196 EU and she was diagnosed with MPA. She was then treated with corticosteroids and cyclophosphamide and had a good outcome.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3914364&req=5

Figure 1: Patient with pulmonary fibrosis had positive ANCA at her initial screening: A 64-year-old woman complained of cough and exertional dyspnea. She had UIP-pattern on chest CT: reticular opacities and honeycombing with basal and peripheral distribution. But her ANCA showed a myeloperoxidase of 196 EU and she was diagnosed with MPA. She was then treated with corticosteroids and cyclophosphamide and had a good outcome.
Mentions: Since all 19 patients were diagnosed with MPA after 2004, they all had high-resolution CT images (HRCT; FigureĀ 1 and 2) (Informed written consent was obtained for the publication of individual personal information from these two patients). The most common feature in each patient was reticular shadows, with approximately 40-70% of the whole lung involved. Additionally, some patients (10/19) had honeycomb lesions (approximately 5-31% of the whole lung), and 2 patients had minimal ground-glass opacities (both were less than 5%). All of the shadows were predominant in both lower lobes of the lungs and had outer zone predominance. Eleven patients received pulmonary function testing (PFT) upon the diagnosis of pulmonary fibrosis. Among them, PFT disclosed a restrictive pattern of ventilation and decreased gas diffusion, with a total lung capacity from 48.2% to 72.8% and a carbon monoxide transfer factor (DLCO) from 30% to 76%.

Bottom Line: One of the remaining patients progressed slowly, whereas six died.Most patients had a delayed positive ANCA.Corticosteroids plus cyclophosphamide was the remission-induction treatment scheme for all cases.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, #1 Shuaifuyuan Street, Beijing, Dongcheng District 100730, China. xuzj@hotmail.com.

ABSTRACT

Background: Pulmonary involvement is a common feature of MPA. Although alveolar hemorrhage is the most common pulmonary manifestation of MPA, a few recent studies have described instances of MPA patients with pulmonary fibrosis. Pulmonary fibrosis was seen to predate, be concomitant with, or occur after the diagnosis of MPA. The goal of this study was to describe the clinical features and prognosis of microscopic polyangiitis (MPA) patients whose initial respiratory presentation was pulmonary fibrosis.

Methods: We conducted a retrospective analysis of 19 MPA patients who presented with pulmonary fibrosis at Peking Union Medical College Hospital between 1990 and 2012.

Results: Of 67 total MPA cases, 19 patients presented with pulmonary fibrosis. There were 8 males and 11 females, with a median age of 63.6 years. Common clinical manifestations included fever (89.5%), cough (84.2%), dyspnea (78.9%) and velcro rales (84.2%). Eleven patients experienced weight loss, several had kidney involvement, and most had an increased erythrocyte sedimentation rate and C-reactive protein. All were positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA), with 6 patients being positive at the time of their initial diagnosis of pulmonary fibrosis. Every patient had typical features of usual interstitial pneumonia on High-resolution CT. All were treated with corticosteroids and cyclophosphamide, which lead to an improvement in twelve cases. One of the remaining patients progressed slowly, whereas six died.

Conclusions: Patients with MPA, who also presented with pulmonary fibrosis in our cohort, were more likely to be older, female, and have extrapulmonic involvement. Most patients had a delayed positive ANCA. Corticosteroids plus cyclophosphamide was the remission-induction treatment scheme for all cases. The current prognosis for MPA patients with pulmonary fibrosis appears to be poor, suggesting that they may be candidates for new therapies.

Show MeSH
Related in: MedlinePlus