Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “DERMATOMYOSITIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 847 records · Page 47Linked to original sources
PubMed1992

Pulmonary and cardiac manifestations of polymyositis-dermatomyositis.

Polymyositis-dermatomyositis (PM-DM) is an inflammatory disease of muscle and skin mediated by autoimmune and cellular events. Most typically, muscle weakness is the usual presentation. This review emphasizes that often the systemic components of this disease may mask the usual presentation and actually may be the presenting and only manifestations; more often than not they are the causes of increased morbidity and mortality. In particular, the cardiopulmonary manifestations may dominate the disease course. Cardiac complications include congestive heart failure resulting from a primary cardiomyopathy, disrhythmias and atrioventricular conduction disturbances, sick sinus syndrome, and cor pulmonale either secondary to interstitial lung disease (ILD) or primary pulmonary artery hypertension. Recurrent aspiration pneumonia results from pharyngeal muscle involvement by the myositic process. Several histologic patterns of ILD can emerge with varying outcomes and responses to immunosuppresive therapy. Involvement of the muscles of respiration can lead to hypercapnic respiratory failure, diaphragmatic dysfunction, hypostatic pneumonia, and restrictive lung disease.

Cardiomyopathies↗
PubMed1996

High-resolution CT findings of lung disease in patients with polymyositis and dermatomyositis.

The purpose of this study was to determine the type and distribution of interstitial lung disease seen in patients with polymyositis and dermatomyositis, using high-resolution computed tomography (HRCT). The presence and distribution of high-resolution CT findings were retrospectively evaluated in 25 patients with polymyositis/dermatiomyositis. In 14 patients, a pathological diagnosis of pulmonary disease was obtained. Three lung specimens were also studied. Results showed that 23 patients had abnormal HRCT showing the following abnormalities: ground glass opacities (92%), linear opacities (92%), irregular interfaces (88%), airspace consolidation (52%), parenchymal micronodules (28%), and honeycombing (16%). A relatively high prevalence of airspace consolidation (52%) and a low prevalence of honeycombing (16%) were observed. Two patients with extensive consolidation proved to have diffuse alveolar damage; eight patients with either subpleural band-like opacities (n = 5) and/or airspace consolidation (n = 7) had bronchiolitis obliterans organizing pneumonia; four patients with honeycombing had usual interstitial pneumonitis. We conclude that HRCT findings in patients with polymyositis/dermatomyositis are nonspecific. However, a high prevalence of airspace consolidation and a low prevalence of honeycombing were observed. Predominant HRCT patterns are suggestive of the pathologic processes occurring in polymyositis/dermatomyositis.

Adrenal Cortex Hormones↗
PubMed1976

Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature.

Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis, or may occur later in the evolution of the disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. The histopathologic features are those of organizing and interstitial pneumonitis and pleuritis, with variable fibrosis. In the present series, the patients with mixed alveolar and interstitial infiltrates on chest radiograph and organizing pneumonia and bronchiolitis obliterans in addition to interstitial pneumonitis. In one patient evolution from pulmonary inflammation to interstitial fibrosis was demonstrated. The etiology of primary lung disease in PM-DM is not known, but cell-mediated autoimmunity to an unidentified component of lung tissue is suggested. Including the present series, 50 percent of patients have responded favorably to corticosteroids with decreased dyspnea, clearing of the chest radiograph and improved pulmonary function tests.

Adult↗
PubMed1994

Ovarian cancer in patients with dermatomyositis.

A consensus regarding adequate screening to detect early malignancy in the setting of dermatomyositis (DMM) has yet to be reached. This issue is particularly relevant with regard to ovarian cancer, as early detection with routine examinations, ultrasound, and abdominal CT may not be successful. Four of 15 women diagnosed with and seen in our department for DMM between 1986 and 1993 were subsequently diagnosed with metastatic papillary serous ovarian carcinoma. One additional patient developed metastatic pelvic papillary adenocarcinoma, believed to be of ovarian origin. These diagnoses of advanced cancer were unexpected, as all women had undergone repeated cancer screenings beyond what is normally recommended for patients with DMM. The 5 women were strikingly similar in their initial presentations and subsequent courses. In each, the diagnosis of DMM was delayed from 2 to 10 months, as they were initially misdiagnosed with a photoinduced or contact dermatitis. All except 1 had severe, recalcitrant skin disease despite attempted therapy with antimalarial and immunosuppressive agents. All 4 patients who survived the postoperative period after tumor debulking showed either improvement or resolution of their DMM. It appears that women with DMM have an increased incidence of ovarian cancer, which is usually diagnosed months to a few years (range, 0 d to 6 y) after DMM has been diagnosed. Although recommendations have been made regarding cancer screening in these individuals, recommendations for initial and surveillance examinations vary from routine history and physical examination to evaluations including extensive radiologic evaluation.(ABSTRACT TRUNCATED AT 250 WORDS)

Age of Onset↗
PubMed1999

Influence of age on characteristics of polymyositis and dermatomyositis in adults.

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.

Activities of Daily Living↗
PubMed2003

Peripheral blood lymphocyte subset counts in patients with dermatomyositis: clinical correlations and changes following therapy.

Lymphocytopenia has been reported in patients with connective tissue diseases, including dermatomyositis (DM). However, the risk of infectious complications and the changes of lymphocytic subsets during treatment have been poorly investigated in these patients. We investigated the alterations of peripheral blood lymphocyte counts in patients with DM. A retrospective analysis was conducted in patients with an ascertained diagnosis of DM admitted from 1994 to 2000 in both departments of Dermatology of the Saint-Louis Hospital in Paris. All patients had a peripheral blood absolute lymphocyte count available before therapy. From an initial set of 63 patients, 47 were included in the study. The median absolute lymphocyte count was 888/mm(3) (range, 400-4,070). Low peripheral blood CD4+ and CD8+ T-cell and B-cell counts were consistent findings (median CD4+: 382/mm(3); CD8+: 211/mm(3); CD19+: 122/mm(3)). There was a significant increase in lymphocyte count after 1 month (p < 0.0001), 3-6 months (p = 0.001), and 6-12 months (p = 0.0005) of corticosteroid treatment. Infectious events, mainly pneumonia (PCP), occurred in 12 patients. Their initial lymphocyte count was lower than that of patients who did not develop infections (p = 0.0001). These results support the high prevalence of lymphocytopenia in patients with DM and emphasize the risk for opportunistic infections, mainly PCP, in these patients. Further studies are warranted to evaluate the risk/benefit balance of PCP prophylaxis in patients with DM and severe lymphocytopenia.

Adolescent↗
PubMed2004

Inflammatory disorders of muscle: progress in polymyositis, dermatomyositis and inclusion body myositis.

PURPOSE OF REVIEW: To provide an update on the major advances in inflammatory myopathies. RECENT FINDINGS: Polymyositis is an uncommon disorder that can be misdiagnosed when the old, and never validated, criteria of Bohan and Peter are used. New diagnostic criteria were recently introduced, in which the MHC/CD8 complex is considered a specific immunopathological marker because it distinguishes the antigen-driven inflammatory cells that characterize polymyositis and sporadic inclusion-body myositis from the non-specific, secondary inflammation seen in other disorders, such as dystrophies. In sporadic inclusion-body myositis the inflammatory cells invade non-vacuolated fibers, whereas the vacuolated fibers are not invaded by T cells, implying two independent processes, a primary immune process with antigen-driven T cells identical to polymyositis, and a degenerative process in which beta-amyloid and amyloid-related proteins participate in vacuolar degeneration. In polymyositis and sporadic inclusion-body myositis, antigen-specific and clonally expanded autoinvasive T cells persist for years, even in different muscles, as reconfirmed by proof-of-principle techniques involving CDR3 spectratyping combined with laser microdissected single-cell polymerase chain reaction of the T-cell receptor genes. The formation of immunological synapse between autoinvasive T cells and muscle fibers was recently strengthened by the upregulation of co-stimulatory molecules ICOS/ICOS-L and PD-L1. A new, distinct myopathy characterized by T-cell-triggered macrophage hyperactivation has now been recognized in patients with dermatomyositis-like disease. SUMMARY: Despite recent progress, the antigen(s) responsible for T-cell activation in polymyositis and sporadic inclusion-body myositis and the cause of vacuolar degeneration in sporadic inclusion-body myositis remain unclear. Newer, more aggressive immunotherapies may be encouraging, but control trials are needed to prove efficacy.

Amyloid↗
PubMed1992

Malignancy and interstitial pneumonitis as fatal complications in dermatomyositis.

By sending questionnaires to the major dermatological clinics in Japan, we examined the outcome and causes of death in 182 cases of dermatomyositis (DM) observed between 1983 and 1987. The mortality rate in adults was 26.7%. Associated malignancy occurred in 47 cases (25.8% of adults), and 16 of them died of neoplasms. In contrast to 43 carcinomas, only 1 patient died of lymphoma. Associated interstitial pneumonitis (IP) appeared in 31 cases. IP started simultaneously or within 6 months after the diagnosis of DM in the majority of these cases. Of the 13 fatal cases due to IP, 8 died of respiratory failure within two months of the onset of lung disease. In the malignancy group, periungual erythema was noted in a higher proportion. Raynaud's phenomenon was more frequently noted in the IP group. DM overlapped with other collagen diseases in 14 cases.

Adolescent↗
PubMed1992

A case of dermatomyositis associated with pseudomyxoma peritonei originating from mucinous adenocarcinoma of the appendix.

A 69-year-old Japanese woman with erythema, severe edema on the face, Gottron's papules and poikiloderma was diagnosed as having dermatomyositis. She also noticed muscle weakness in her extremities, although her electromyogram showed neurogenic patterns. Her levels of CA19-9 and CEA were elevated and a CT of her abdomen revealed a giant, multilobular, cystic lesion in the pelvis. This tumor was diagnosed as pseudomyxoma peritonei originating from a mucinous adenocarcinoma of the appendix.

Adenocarcinoma, Mucinous↗
PubMed1994

Dermatomyositis with multiple skin ulcers showing vasculitis and membrano-cystic lesion.

A 63-year-old woman with dermatomyositis (DM) developed multiple punched-out ulcers of the skin. She had not only a past history of gastric cancer 10 years prior to the onset of DM, but also a familial history of cancer. A skin biopsy from one of the ulcers revealed both vasculitis and membrano-cystic lesion in the same specimen. She died of interstitial pneumonitis one year after the onset of DM. Vasculitis and membrano-cystic lesion may be an important marker of a poor prognosis in DM.

Dermatomyositis↗
PubMed1996

Successful treatment of a therapy-resistant severely pruritic skin eruption of malignancy-associated dermatomyositis with high-dose intravenous immunoglobulin.

Malignancy-associated dermatomyositis developing in a middle-aged Japanese female was successfully treated by removal of her gastric cancer. However, five years later, concomitant with catching a cold, her severely pruritic skin lesions recurred on exposed areas. Six years after the start of corticosteroid therapy for her annoying skin lesion, we started to treat her with high-dose intravenous immunoglobulin (IVIG) infusion therapy because of the difficulty of reducing the dosage of oral prednisone, that had secondarily induced adverse effects. After five courses of the therapy, her recalcitrant, pruritic, erythematous skin lesions improved dramatically, enabling a satisfactory reduction in the dosage of oral prednisone. There were no significant adverse side effects with IVIG.

Dermatomyositis↗
PubMed1997

Pneumomediastinum and subcutaneous emphysema associated with fatal interstitial pneumonia in dermatomyositis.

We described a 65-year-old woman who died of acute interstitial pneumonia associated with dermatomyositis. Subcutaneous emphysema and pneumomediastinum simultaneously developed. The association of the pulmonary rupture with vasculitis has been assumed as the common cause in interstitial pneumonia. Diffuse alveolar damage, however, might have led to the pneumomediastinum and subcutaneous emphysema in our patient, who had no signs of cutaneous vasculitis.

Aged↗
PubMed2000

A case of dermatomyositis associated with mechanic's hand.

A 67-year-old man was referred to the Department of Internal Medicine at Tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palsm and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patient's chest, back, and the extensor surfaces of his arms. Electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanic's hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanic's hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanic's hand in Asians.

Adenocarcinoma↗
PubMed2004

Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.

Biopsy, Needle↗
PubMed1994

Juvenile dermatomyositis: treatment with intravenous gammaglobulin.

High-dose intravenous gammaglobulin (IVGG) has proved to be effective in the treatment of a number of immune disorders. We report two patients with juvenile dermatomyositis (DM) who improved with IVGG therapy. These patients had become refractory to corticosteroids and had developed unacceptable steroid toxicity. We suggest that IVGG can be useful in the treatment of juvenile DM, by reducing steroid requirements, and replacing immunosuppressive drugs.

Child↗
PubMed2004

Infiltrating cells, related cytokines and chemokine receptors in lesional skin of patients with dermatomyositis.

BACKGROUND: There have been only two reports on immunophenotypic characterization in the cutaneous lesions of dermatomyositis (DM) that emphasize the importance of the infiltrating CD4+ T lymphocytes. OBJECTIVES: To characterize the immunophenotype of the cells that infiltrate the lesional skin of DM and to evaluate the possible T-helper (Th) polarization Th1/Th2 through detection of specific cytokines, chemokine receptors and markers of cellular activation. METHODS: Skin biopsy specimens derived from pathognomonic lesions (Gottron's papules and Gottron's sign) of eight patients with DM were immunostained with a large panel of monoclonal antibodies to CD3, CD4, CD8, myeloperoxidase (MPO), eosinophil cationic protein, tryptase, CD40, CD40 ligand (CD40L), HLA-DR, interleukin (IL)-2, IL-4, IL-5, IL-13, interferon-gamma, tumour necrosis factor-alpha, receptor 3 for CXC chemokines (CXCR3) and receptor 3 for CC chemokines, using the alkaline phosphatase-antialkaline phosphatase method. Control specimens were obtained from five healthy subjects and from six patients with discoid lupus erythematosus. RESULTS: Activated CD4+ Th lymphocytes (HLA-DR+ CD40L+) were the principal infiltrating cells in the lesional skin of DM; the CD4/CD8 ratio was approximately 2.5. A mixed Th1/Th2 profile and higher Th1 cytokine production together with significant staining for CXCR3 were detected. Neutrophil granulocytes were the second most abundant population; eosinophil granulocytes were very poorly represented. CONCLUSIONS: Activated CD4+ T cells presumably mediate the main pathogenetic mechanisms in pathognomonic skin lesions. The interaction between CD40 and CD40L could be an important mechanism of cellular activation in cutaneous immune-mediated inflammation by induction of secretion of proinflammatory cytokines and chemokines. Neither Th1 nor Th2 clear polarization was found, although there was a slight Th1 prevalence. There was a significant quantity of MPO+ cells (neutrophil granulocytes) in the inflamed tissue, and they might have a role in sustaining the chronic inflammation.

Adolescent↗