Influence of Age on Characteristics of Polymyositis and Dermatomyositis in Adults

Abstract

In a retrospective study of 79 consecutive patients, we evaluated characteristics of polymyositis (PM) and dermatomyositis (DM) and compared clinical presentation, biochemical findings, histologic changes, evolution, complications, and mortality rate of elderly patients (aged > or = 65 yr) and younger patients (aged < or = 64 yr) at the onset of PM/DM. We found a high prevalence of PM/DM in elderly patients: 23 patients (29%) were aged 65 years or over. We also found that esophageal involvement (34.8% versus 16.1%, respectively) and bacterial pneumonia related to both ventilatory insufficiency and esophageal impairment (21.7% versus 5.4%, respectively) were more common in elderly patients compared with younger patients, resulting in increased morbidity and mortality rates. Moreover, malignancy frequency was higher in elderly patients compared with younger patients (47.8% versus 9.1%, respectively, p = 0.0001), particularly patients with DM (10/11). Fifty percent of malignancies were colon malignancies in elderly patients. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and ferritin levels were also higher in the elderly patient group compared with the younger group, and the presence of serum hypoprotidemia, hypoalbuminemia, and anemia was more frequent. Finally, PM/DM complete remission was less frequent (13.6% versus 41.1%) and the mortality rate (47.8% versus 7.3%) was higher in elderly patients than in younger patients. The main causes of death in elderly patients were bacterial pneumonia, due to ventilatory insufficiency and esophageal impairment, and malignancies. Our findings therefore indicate that PM/DM-related esophageal and lung involvement should be systematically searched for in elderly patients. Esophageal manometry and pulmonary function tests should become an integral part of initial evaluation in elderly patients for early detection of impairment. Moreover, as we observed a marked overrepresentation of colon cancer in elderly patients with DM, we suggest that the search for malignancies in elderly patients with DM should include lower gastrointestinal tract investigations.

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement: Table 1—

“…Pulmonary involvement in PM/DM may precede muscle and skin manifestations and includes respiratory muscle weakness, aspiration pneumonia, ILD, ventilatory insufficiency, infections and drug-induced pneumonia [114][115][116][117]. ILD, which develops in 23.1-65.0% of PM/DM patients [116,[118][119][120], is a major cause of death in this disease [116,119,[121][122][123].…”

“…ILD, which develops in 23.1-65.0% of PM/DM patients [116,[118][119][120], is a major cause of death in this disease [116,119,[121][122][123]. ILD leads to life-threatening complications, such as ventilatory failure, secondary pulmonary arterial hypertension and cor pulmonale [116,117,121,122,[124][125][126][127], so the early detection of ILD is a high priority in PM/DM patients. However, in PM/DM patients a careful search for underlying malignancies is strongly advocated.…”

Malignancy in patients with inflammatory myopathy

“…Case series were reported from Bulgaria (50 cases) [10], the United States (65 cases) [11], France (79 cases) [8,9], Switzerland (23 cases) [ Pautas et al [12] investigated the characteristics of PM and DM in the elderly including their frequency of cancer. They identified 200 patients in total who had been discharged between 1973 and 1996 with a diagnosis of PM or DM and arbitrarily defined two groups based on aged 65 years or over at onset to younger patients (minimum age 20 years).…”

“…Marie et al [9] also investigated the characteristics of IIM in the elderly by arbitrarily dividing 79 consecutive patients with DM and PM (Bohan and Peter [16] criteria, definite in 59 and probable in 20), from two hospital clinics in France between 1983 and 1997, into two groups according to age (less than 65 years, n = 23; 65 years or older, n = 56). It appears that all patients were examined and investigated in a similar method including examination for underlying malignancy.…”

Dermatomiosite: estudo de 109 pacientes avaliados no HCFMUSP

“…Houve maior mortalidade em adultos (16,3%), comparada a DMJ (8,7%), mostrando, assim, a influência da idade na evolução da DM (Marie et al, 1999;Marie et al, 2001a). As causas de óbito mais freqüentes coincidem com a literatura (Bohan et al, 1977;Benbassat et al, 1985;Basset-Seguin et al, 1990;Koh et al, 1993;Maugars et al, 1996;Marie et al, 2001a), sendo causa principal no nosso estudo a evolução das neoplasias associadas à DM (44%) e a pneumonia bacteriana (25%).…”