Interstitial lung disease in dermatomyositis and polymyositis: Treatment
- Paul F Dellaripa, MD
Paul F Dellaripa, MD
- Associate Professor of Medicine
- Harvard Medical School
- Marc L Miller, MD
Marc L Miller, MD
- Clinical Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Kevin R Flaherty, MD, MS
Kevin R Flaherty, MD, MS
- Section Editor — Interstitial Lung Disease
- Associate Professor of Medicine
- University of Michigan Health System
- Deputy Editors
- Helen Hollingsworth, MD
Helen Hollingsworth, MD
- Deputy Editor — Pulmonary, Critical Care, and Sleep Medicine
- Associate Professor of Medicine
- Boston University School of Medicine
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
Interstitial lung disease (ILD) is a major cause of morbidity and mortality in dermatomyositis (DM) and polymyositis (PM). The clinical features and histopathologic appearance of ILD in DM and PM reflect the patterns of lung pathology associated with the idiopathic interstitial pneumonias, including nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, and acute interstitial pneumonia. (See "Idiopathic interstitial pneumonias: Clinical manifestations and pathology".)
The treatment of interstitial lung disease associated with DM and PM will be reviewed here. The clinical manifestations, diagnosis, and differential diagnosis of interstitial lung disease in DM and PM and the management of myositis in DM and PM are discussed separately. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)
APPROACH TO THERAPY
When assessing the need for treatment in patients with interstitial lung disease (ILD) due to polymyositis (PM) or dermatomyositis (DM), we review the type of underlying ILD and assess the severity of respiratory impairment and the rate of progression.
Overview — Not every patient with ILD due to PM or DM requires treatment of the ILD as most patients follow a chronic, slowly-progressive course . While formal guidelines are not available, we observe patients without treatment in the setting of the following characteristics: mild dyspnea on exertion, <10 percent involvement on high resolution computed tomography, forced vital capacity >75 percent of predicted, and/or diffusing capacity for carbon monoxide (DLCO) >65 percent of predicted . However, for other patients with a greater degree of respiratory impairment and more pronounced abnormalities on imaging and pulmonary function tests, immunosuppressive therapy for ILD is generally indicated.
For patients with DM or PM who are receiving immunosuppressive therapy for their myositis, it is essential to exclude infection or drug-induced pulmonary toxicity as causes of lung disease. In addition, patients with DM and PM are at increased risk of malignancy, including cancers involving the lungs . Thus, it is prudent to ascertain that the patient has undergone appropriate cancer screening. (See "Interstitial lung disease in dermatomyositis and polymyositis: Clinical manifestations and diagnosis", section on 'Differential diagnosis' and "Malignancy in dermatomyositis and polymyositis", section on 'Approach to screening'.)
- Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest 2013; 143:814.
- Gutsche M, Rosen GD, Swigris JJ. Connective Tissue Disease-associated Interstitial Lung Disease: A review. Curr Respir Care Rep 2012; 1:224.
- Tiniakou E, Mammen AL. Idiopathic Inflammatory Myopathies and Malignancy: a Comprehensive Review. Clin Rev Allergy Immunol 2017; 52:20.
- Cottin V, Thivolet-Béjui F, Reynaud-Gaubert M, et al. Interstitial lung disease in amyopathic dermatomyositis, dermatomyositis and polymyositis. Eur Respir J 2003; 22:245.
- Koreeda Y, Higashimoto I, Yamamoto M, et al. Clinical and pathological findings of interstitial lung disease patients with anti-aminoacyl-tRNA synthetase autoantibodies. Intern Med 2010; 49:361.
- Kang EH, Lee EB, Shin KC, et al. Interstitial lung disease in patients with polymyositis, dermatomyositis and amyopathic dermatomyositis. Rheumatology (Oxford) 2005; 44:1282.
- Marie I, Hachulla E, Chérin P, et al. Interstitial lung disease in polymyositis and dermatomyositis. Arthritis Rheum 2002; 47:614.
- Dickey BF, Myers AR. Pulmonary disease in polymyositis/dermatomyositis. Semin Arthritis Rheum 1984; 14:60.
- Schwarz MI, Matthay RA, Sahn SA, et al. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature. Medicine (Baltimore) 1976; 55:89.
- Fujisawa T, Suda T, Nakamura Y, et al. Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol 2005; 32:58.
- Tazelaar HD, Viggiano RW, Pickersgill J, Colby TV. Interstitial lung disease in polymyositis and dermatomyositis. Clinical features and prognosis as correlated with histologic findings. Am Rev Respir Dis 1990; 141:727.
- Wells AU, du Bois RM. Bronchiolitis in association with connective tissue disorders. Clin Chest Med 1993; 14:655.
- Nawata Y, Kurasawa K, Takabayashi K, et al. Corticosteroid resistant interstitial pneumonitis in dermatomyositis/polymyositis: prediction and treatment with cyclosporine. J Rheumatol 1999; 26:1527.
- Salmeron G, Greenberg SD, Lidsky MD. Polymyositis and diffuse interstitial lung disease. A review of the pulmonary histopathologic findings. Arch Intern Med 1981; 141:1005.
- Bonnefoy O, Ferretti G, Calaque O, et al. Serial chest CT findings in interstitial lung disease associated with polymyositis-dermatomyositis. Eur J Radiol 2004; 49:235.
- Douglas WW, Tazelaar HD, Hartman TE, et al. Polymyositis-dermatomyositis-associated interstitial lung disease. Am J Respir Crit Care Med 2001; 164:1182.
- Takada K, Kishi J, Miyasaka N. Step-up versus primary intensive approach to the treatment of interstitial pneumonia associated with dermatomyositis/polymyositis: a retrospective study. Mod Rheumatol 2007; 17:123.
- Bradley B, Branley HM, Egan JJ, et al. Interstitial lung disease guideline: the British Thoracic Society in collaboration with the Thoracic Society of Australia and New Zealand and the Irish Thoracic Society. Thorax 2008; 63 Suppl 5:v1.
- Labirua-Iturburu A, Selva-O'Callaghan A, Martínez-Gómez X, et al. Calcineurin inhibitors in a cohort of patients with antisynthetase-associated interstitial lung disease. Clin Exp Rheumatol 2013; 31:436.
- Saketkoo LA, Espinoza LR. Experience of mycophenolate mofetil in 10 patients with autoimmune-related interstitial lung disease demonstrates promising effects. Am J Med Sci 2009; 337:329.
- Swigris JJ, Olson AL, Fischer A, et al. Mycophenolate mofetil is safe, well tolerated, and preserves lung function in patients with connective tissue disease-related interstitial lung disease. Chest 2006; 130:30.
- Morganroth PA, Kreider ME, Werth VP. Mycophenolate mofetil for interstitial lung disease in dermatomyositis. Arthritis Care Res (Hoboken) 2010; 62:1496.
- Fischer A, Brown KK, Du Bois RM, et al. Mycophenolate mofetil improves lung function in connective tissue disease-associated interstitial lung disease. J Rheumatol 2013; 40:640.
- Tsuchiya H, Tsuno H, Inoue M, et al. Mycophenolate mofetil therapy for rapidly progressive interstitial lung disease in a patient with clinically amyopathic dermatomyositis. Mod Rheumatol 2014; 24:694.
- Mira-Avendano IC, Parambil JG, Yadav R, et al. A retrospective review of clinical features and treatment outcomes in steroid-resistant interstitial lung disease from polymyositis/dermatomyositis. Respir Med 2013; 107:890.
- Wilkes MR, Sereika SM, Fertig N, et al. Treatment of antisynthetase-associated interstitial lung disease with tacrolimus. Arthritis Rheum 2005; 52:2439.
- Ochi S, Nanki T, Takada K, et al. Favorable outcomes with tacrolimus in two patients with refractory interstitial lung disease associated with polymyositis/dermatomyositis. Clin Exp Rheumatol 2005; 23:707.
- Takada K, Nagasaka K, Miyasaka N. Polymyositis/dermatomyositis and interstitial lung disease: a new therapeutic approach with T-cell-specific immunosuppressants. Autoimmunity 2005; 38:383.
- Qushmaq KA, Chalmers A, Esdaile JM. Cyclosporin A in the treatment of refractory adult polymyositis/dermatomyositis: population based experience in 6 patients and literature review. J Rheumatol 2000; 27:2855.
- Maeda K, Kimura R, Komuta K, Igarashi T. Cyclosporine treatment for polymyositis/dermatomyositis: is it possible to rescue the deteriorating cases with interstitial pneumonitis? Scand J Rheumatol 1997; 26:24.
- Cavagna L, Caporali R, Abdì-Alì L, et al. Cyclosporine in anti-Jo1-positive patients with corticosteroid-refractory interstitial lung disease. J Rheumatol 2013; 40:484.
- Kurita T, Yasuda S, Oba K, et al. The efficacy of tacrolimus in patients with interstitial lung diseases complicated with polymyositis or dermatomyositis. Rheumatology (Oxford) 2015; 54:39.
- Saravanan V, Kelly CA. Reducing the risk of methotrexate pneumonitis in rheumatoid arthritis. Rheumatology (Oxford) 2004; 43:143.
- Lambotte O, Kotb R, Maigne G, et al. Efficacy of rituximab in refractory polymyositis. J Rheumatol 2005; 32:1369.
- Brulhart L, Waldburger JM, Gabay C. Rituximab in the treatment of antisynthetase syndrome. Ann Rheum Dis 2006; 65:974.
- Andersson H, Sem M, Lund MB, et al. Long-term experience with rituximab in anti-synthetase syndrome-related interstitial lung disease. Rheumatology (Oxford) 2015; 54:1420.
- Zappa MC, Trequattrini T, Mattioli F, et al. Rituximab treatment in a case of antisynthetase syndrome with severe interstitial lung disease and acute respiratory failure. Multidiscip Respir Med 2011; 6:183.
- Vandenbroucke E, Grutters JC, Altenburg J, et al. Rituximab in life threatening antisynthetase syndrome. Rheumatol Int 2009; 29:1499.
- Dasa O, Ruzieh M, Oraibi O. Successful Treatment of Life-Threatening Interstitial Lung Disease Secondary to Antisynthetase Syndrome Using Rituximab: A Case Report and Review of the Literature. Am J Ther 2016; 23:e639.
- Marie I, Dominique S, Janvresse A, et al. Rituximab therapy for refractory interstitial lung disease related to antisynthetase syndrome. Respir Med 2012; 106:581.
- Keir GJ, Maher TM, Ming D, et al. Rituximab in severe, treatment-refractory interstitial lung disease. Respirology 2014; 19:353.
- Sharp C, McCabe M, Dodds N, et al. Rituximab in autoimmune connective tissue disease-associated interstitial lung disease. Rheumatology (Oxford) 2016; 55:1318.
- Oddis CV, Reed AM, Aggarwal R, et al. Rituximab in the treatment of refractory adult and juvenile dermatomyositis and adult polymyositis: a randomized, placebo-phase trial. Arthritis Rheum 2013; 65:314.
- Kameda H, Nagasawa H, Ogawa H, et al. Combination therapy with corticosteroids, cyclosporin A, and intravenous pulse cyclophosphamide for acute/subacute interstitial pneumonia in patients with dermatomyositis. J Rheumatol 2005; 32:1719.
- Kawasumi H, Gono T, Kawaguchi Y, Yamanaka H. Recent Treatment of Interstitial Lung Disease with Idiopathic Inflammatory Myopathies. Clin Med Insights Circ Respir Pulm Med 2015; 9:9.
- Kurita T, Yasuda S, Amengual O, Atsumi T. The efficacy of calcineurin inhibitors for the treatment of interstitial lung disease associated with polymyositis/dermatomyositis. Lupus 2015; 24:3.
- Suzuki Y, Hayakawa H, Miwa S, et al. Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung 2009; 187:201.
- Bakewell CJ, Raghu G. Polymyositis associated with severe interstitial lung disease: remission after three doses of IV immunoglobulin. Chest 2011; 139:441.
- Yamasaki Y, Yamada H, Yamasaki M, et al. Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis. Rheumatology (Oxford) 2007; 46:124.
- al-Janadi M, Smith CD, Karsh J. Cyclophosphamide treatment of interstitial pulmonary fibrosis in polymyositis/dermatomyositis. J Rheumatol 1989; 16:1592.
- Mok CC, To CH, Szeto ML. Successful treatment of dermatomyositis-related rapidly progressive interstitial pneumonitis with sequential oral cyclophosphamide and azathioprine. Scand J Rheumatol 2003; 32:181.
- Zou J, Li T, Huang X, et al. Basiliximab may improve the survival rate of rapidly progressive interstitial pneumonia in patients with clinically amyopathic dermatomyositis with anti-MDA5 antibody. Ann Rheum Dis 2014; 73:1591.
- Ameye H, Ruttens D, Benveniste O, et al. Is lung transplantation a valuable therapeutic option for patients with pulmonary polymyositis? Experiences from the Leuven transplant cohort. Transplant Proc 2014; 46:3147.
- Arboleda R, Gonzalez O, Cortes M, Perez-Cerda F. Recurrent polymyositis-associated lung disease after lung transplantation. Interact Cardiovasc Thorac Surg 2015; 20:560.
- Shoji T, Bando T, Fujinaga T, et al. Living-donor lobar lung transplantation for rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis: report of a case. Gen Thorac Cardiovasc Surg 2013; 61:32.
- Caceres M, Ali SZ, Braud R, et al. Spontaneous pneumomediastinum: a comparative study and review of the literature. Ann Thorac Surg 2008; 86:962.
- Le Goff B, Chérin P, Cantagrel A, et al. Pneumomediastinum in interstitial lung disease associated with dermatomyositis and polymyositis. Arthritis Rheum 2009; 61:108.
- Barvaux VA, Van Mullem X, Pieters TH, Houssiau FA. Persistent pneumomediastinum and dermatomyositis: a case report and review of the literature. Clin Rheumatol 2001; 20:359.
- Viguier M, Fouéré S, de la Salmonière P, et al. Peripheral blood lymphocyte subset counts in patients with dermatomyositis: clinical correlations and changes following therapy. Medicine (Baltimore) 2003; 82:82.
- Marie I, Hatron PY, Dominique S, et al. Short-term and long-term outcomes of interstitial lung disease in polymyositis and dermatomyositis: a series of 107 patients. Arthritis Rheum 2011; 63:3439.
- Tillie-Leblond I, Wislez M, Valeyre D, et al. Interstitial lung disease and anti-Jo-1 antibodies: difference between acute and gradual onset. Thorax 2008; 63:53.
- Mukae H, Ishimoto H, Sakamoto N, et al. Clinical differences between interstitial lung disease associated with clinically amyopathic dermatomyositis and classic dermatomyositis. Chest 2009; 136:1341.
- Ikeda S, Arita M, Misaki K, et al. Incidence and impact of interstitial lung disease and malignancy in patients with polymyositis, dermatomyositis, and clinically amyopathic dermatomyositis: a retrospective cohort study. Springerplus 2015; 4:240.
- APPROACH TO THERAPY
- Nonspecific interstitial pneumonia
- Organizing pneumonia
- Other types of ILD
- INITIAL GLUCOCORTICOID THERAPY FOR NSIP
- ADDING A SECOND AGENT
- Choice of agent
- - Azathioprine
- - Mycophenolate mofetil
- - Calcineurin inhibitors
- - Methotrexate
- REFRACTORY DISEASE
- Combination therapy
- Intravenous immune globulin
- LUNG TRANSPLANTATION
- ASSESSING THE RESPONSE AND DURATION OF THERAPY
- Spontaneous pneumomediastinum
- Prevention of treatment related complications
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS