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Correspondence |

IV Immunoglobulin Might Be Considered as a First-line Treatment of Severe Interstitial Lung Disease Associated With PolymyositisIV Immunoglobulin Treatment With Polymyositis FREE TO VIEW

Elisabeth Diot, MD, PhD; Delphine Carmier, MD; David Marquette, MD; Sylvain Marchand-Adam, MD, PhD; Patrice Diot, MD, PhD; Vincent Lesire, MD
Author and Funding Information

From the Service de médecine interne (Dr E. Diot) and the Service de pneumologie et exploration fonctionnelles respiratoires (Drs Carmier, Marquette, Marchand-Adam, and P. Diot), CHRU Tours, Hôpital Bretonneau; the Faculté de médecine (Drs E. Diot, Marchand-Adam, and P. Diot), Unité INSERM U618, Université François Rabelais; and the Service de médecine interne (Dr Lesire), Hôpital de Blois.

Correspondence to: Patrice Diot, MD, PhD, CHRU Tours, Hôpital Bretonneau, Service de pneumologie et exploration fonctionnelles respiratoires, 2 Boulevard Tonnelle, 37032 Tours, France; e-mail: diot@med.univ-tours.fr


Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2011 American College of Chest Physicians


Chest. 2011;140(2):562-563. doi:10.1378/chest.11-0492
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To the Editor:

We read with interest the article by Bakewell and Raghu1 in a recent issue of CHEST (February 2011), in which the authors relate the case of a patient with severe interstitial lung disease associated with polymyositis/dermatopolymyositis (ILD-PM/DM). The patient, remarkably, improved after three monthly doses of 2 g/kg IV immunoglobulin (Ig) without any other immunosuppressive agent, with sustained clinical remission after > 2 years. According to the authors, IV Ig has not yet been tested as a first-line treatment of this disease. Our recent observation of a patient who did not respond to steroids and worsened after one infusion of cyclophosphamide but did improve dramatically after IV Ig is consistent with Bakewell and Raghu,1 who suggest that IV Ig should be considered as a potential first-line therapy for ILD-PM/DM.

Our patient was a 47-year-old man with ILD-PM/DM in the context of dyspnea associated with severe weakness. Creatine kinase value was increased to 1,110 units/L, and antinuclear autoantibodies were positive without anti-Jo-1. The results of an electromyogram suggested a myopathy, and the results of a deltoid biopsy confirmed the diagnosis of polymyositis. Chest radiograph and CT scans revealed consolidation, with air bronchograms and ground-glass opacities in both lungs with nodular opacities. Total lung capacity (TLC) was 52% of predicted values, and vital capacity (VC) was 55%. Diffusion capacity of the lung for carbon monoxide (Dlco) was 61%. The results of BAL revealed mixed alveolitis with 22% lymphocytes and 10% neutrophils. Transbronchial biopsies were not performed because of poor tolerance for BAL. Prednisone was given at 80 mg/d (1 mg/kg) with little response in terms of muscle weakness and no respiratory improvement after 15 days. A bolus of 0.6 mg/m2 of cyclophosphamide was administered, as currently recommended for refractory cases.2-4 Reassessment before the second scheduled cyclophosphamide infusion revealed a dramatic deterioration of pulmonary function (TLC, 41%; VC, 51%; and Dlco, 32%). Cyclophosphamide treatment was stopped, and IV Ig at a dosage of 2 mg/kg was begun.

A marked clinical and biologic improvement (normalization of creatine kinase) was observed 21 days after the first infusion. After three monthly courses of IV Ig, the patient recovered normal muscle function and showed a marked improvement on pulmonary function test (TLC, 59%; VC, 63%; and Dlco, 51%) and a regression of fibrotic changes on CT scans. This observation lends support to the suggestion that IV Ig treatment might be considered as a treatment of ILD-PM/DM, especially for critical cases.

Bakewell CJ, Raghu G. Polymyositis associated with severe interstitial lung disease: remission after three doses of IV immunoglobulin. Chest. 2011;1392:441-443. [CrossRef] [PubMed]
 
Yamasaki Y, Yamada H, Yamasaki M, et al. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford). 2007;461:124-130. [CrossRef] [PubMed]
 
Conners GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;1386:1464-1474. [CrossRef] [PubMed]
 
Suzuki Y, Hayakawa H, Miwa S, et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung. 2009;1873:201-206. [CrossRef] [PubMed]
 

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References

Bakewell CJ, Raghu G. Polymyositis associated with severe interstitial lung disease: remission after three doses of IV immunoglobulin. Chest. 2011;1392:441-443. [CrossRef] [PubMed]
 
Yamasaki Y, Yamada H, Yamasaki M, et al. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford). 2007;461:124-130. [CrossRef] [PubMed]
 
Conners GR, Christopher-Stine L, Oddis CV, Danoff SK. Interstitial lung disease associated with the idiopathic inflammatory myopathies: what progress has been made in the past 35 years? Chest. 2010;1386:1464-1474. [CrossRef] [PubMed]
 
Suzuki Y, Hayakawa H, Miwa S, et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung. 2009;1873:201-206. [CrossRef] [PubMed]
 
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