Interstitial Lung Disease in Inflammatory Myopathies
Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF
Arthritis Rheum. 2011;63:3439-3447
Interstitial lung disease (ILD) is a known comorbidity of both polymyositis (PM) and dermatomyositis (DM) and has been associated with increased mortality in these diseases. To enhance the understanding of the clinical impact of ILD in PM/DM, in this retrospective study conducted in France, Marie and colleagues evaluated the clinical characteristics and outcomes of a series of patients with ILD and PM or DM and evaluated potential predictive factors for worse outcomes of ILD.
Through chart evaluations of 348 consecutive patients at 4 centers, the investigators identified 107 patients with DM or PM who had ILD identified by the patients' clinic providers. PM/DM was determined on the basis of fulfillment of the Bohan and Peter criteria. Study personnel reviewed medical charts to determine the patients' clinical characteristics at the time of diagnosis of ILD, including results of pulmonary function testing (PFT), high-resolution computed tomographic (HRCT) lung imaging, and clinical outcomes of ILD, which were designated as resolution (normalization of symptoms, PFT, and HRCT findings), improvement (without resolution), or deterioration. The median duration of follow-up of these patients after their diagnosis of ILD was 34 months (range, 4-372 months).
Of these 107 patients, 20 (19%) had symptomatic acute onset of ILD, 55 (51%) had symptomatic progressive onset of ILD, and 32 (30%) were asymptomatic. The latter group had findings consistent with ILD on PFT and HRCT, although it is not clear why this group of patients underwent pulmonary evaluation.
ILD resolved in 35 patients (33%), remained stable or improved in 55 patients (51%), and deteriorated in 17 patients (16%). A total of 8 patients died, all in the group that had ILD deterioration, and 7 of these deaths were from complications related to ILD. Factors present at the time of diagnosis associated with ILD deterioration over time included older age, dyspnea, cough, acute onset of ILD, and worse PFT. Additionally, an HRCT finding of usual interstitial pneumonia was associated with ILD deterioration, although not significantly (P = .07). Lung tissue was obtained from 41/107 of patients (38%), and biopsy findings of usual interstitial pneumonia were significantly associated with ILD deterioration. Conversely, biopsy findings of cryptogenic organizing pneumonia and nonspecific interstitial pneumonia were associated with improved outcomes. Factors not associated with worsening ILD were PM/DM subset, creatine kinase levels, esophageal impairment, a concomitant diagnosis of cancer, mechanic's hands, and autoantibodies (in particular antinuclear antibodies, anti-Jo-1, and anti-PM-Scl). Of interest, 29 patients (27%) were positive for anti-SSA antibodies, raising the question of possible overlap syndromes. The relationship between anti-SSA positive status and ILD outcomes was not discussed. Also of note, ILD preceded a diagnosis of PM/DM in 20/107 patients (19%), although in most patients (64%), ILD was identified at the same time that the patients presented with PM/DM.
A variety of therapies were used to treat the PM/DM and ILD. In descriptive analyses, several treatments resulted in improvement of ILD, although these same treatments were also used in the patients who did not improve. These therapies included steroids alone or steroids in combination with azathioprine, cyclophosphamide (typically monthly at a dose of 0.5-0.8 g/m for 3-6 months), and other agents, such as mycophenolate. In particular, of those treated initially with steroids and azathioprine, 20/25 (80%) had either resolution or significant improvement of their ILD. Of patients with ILD treated initially with steroids and cyclophosphamide, 16/25 (64%) had resolution or significant improvement.
Short-term and Long-term Outcomes of Interstitial Lung Disease in Polymyositis and Dermatomyositis: A Series of 107 Patients
Marie I, Hatron PY, Dominique S, Cherin P, Mouthon L, Menard JF
Arthritis Rheum. 2011;63:3439-3447
Interstitial lung disease (ILD) is a known comorbidity of both polymyositis (PM) and dermatomyositis (DM) and has been associated with increased mortality in these diseases. To enhance the understanding of the clinical impact of ILD in PM/DM, in this retrospective study conducted in France, Marie and colleagues evaluated the clinical characteristics and outcomes of a series of patients with ILD and PM or DM and evaluated potential predictive factors for worse outcomes of ILD.
Study Summary
Through chart evaluations of 348 consecutive patients at 4 centers, the investigators identified 107 patients with DM or PM who had ILD identified by the patients' clinic providers. PM/DM was determined on the basis of fulfillment of the Bohan and Peter criteria. Study personnel reviewed medical charts to determine the patients' clinical characteristics at the time of diagnosis of ILD, including results of pulmonary function testing (PFT), high-resolution computed tomographic (HRCT) lung imaging, and clinical outcomes of ILD, which were designated as resolution (normalization of symptoms, PFT, and HRCT findings), improvement (without resolution), or deterioration. The median duration of follow-up of these patients after their diagnosis of ILD was 34 months (range, 4-372 months).
Of these 107 patients, 20 (19%) had symptomatic acute onset of ILD, 55 (51%) had symptomatic progressive onset of ILD, and 32 (30%) were asymptomatic. The latter group had findings consistent with ILD on PFT and HRCT, although it is not clear why this group of patients underwent pulmonary evaluation.
ILD resolved in 35 patients (33%), remained stable or improved in 55 patients (51%), and deteriorated in 17 patients (16%). A total of 8 patients died, all in the group that had ILD deterioration, and 7 of these deaths were from complications related to ILD. Factors present at the time of diagnosis associated with ILD deterioration over time included older age, dyspnea, cough, acute onset of ILD, and worse PFT. Additionally, an HRCT finding of usual interstitial pneumonia was associated with ILD deterioration, although not significantly (P = .07). Lung tissue was obtained from 41/107 of patients (38%), and biopsy findings of usual interstitial pneumonia were significantly associated with ILD deterioration. Conversely, biopsy findings of cryptogenic organizing pneumonia and nonspecific interstitial pneumonia were associated with improved outcomes. Factors not associated with worsening ILD were PM/DM subset, creatine kinase levels, esophageal impairment, a concomitant diagnosis of cancer, mechanic's hands, and autoantibodies (in particular antinuclear antibodies, anti-Jo-1, and anti-PM-Scl). Of interest, 29 patients (27%) were positive for anti-SSA antibodies, raising the question of possible overlap syndromes. The relationship between anti-SSA positive status and ILD outcomes was not discussed. Also of note, ILD preceded a diagnosis of PM/DM in 20/107 patients (19%), although in most patients (64%), ILD was identified at the same time that the patients presented with PM/DM.
A variety of therapies were used to treat the PM/DM and ILD. In descriptive analyses, several treatments resulted in improvement of ILD, although these same treatments were also used in the patients who did not improve. These therapies included steroids alone or steroids in combination with azathioprine, cyclophosphamide (typically monthly at a dose of 0.5-0.8 g/m for 3-6 months), and other agents, such as mycophenolate. In particular, of those treated initially with steroids and azathioprine, 20/25 (80%) had either resolution or significant improvement of their ILD. Of patients with ILD treated initially with steroids and cyclophosphamide, 16/25 (64%) had resolution or significant improvement.