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PubMedDate not supplied

Fatal rapidly progressive interstitial pneumonitis associated with amyopathic dermatomyositis and CD8 T lymphocytes.

A patient with amyopathic dermatomyositis associated with fatal rapidly progressive interstitial pneumonitis resistant to therapy is described. Pathologic examination of a transbronchial lung biopsy specimen showed diffuse alveolar damage and nonspecific interstitial pneumonia-organizing pneumonia-like findings. Bronchoalveolar lavage fluid contained many CD8+ lymphocytes, considered to be cytotoxic T cells. Analysis of bronchoalveolar lavage fluid in this case may provide prognostically and pathogenetically important information.

Bronchoalveolar Lavage Fluid↗
PubMed2006

Malignant lymphoma in patients with systemic rheumatic disease (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, and dermatomyositis): a clinicopathologic study of 24 Japanese cases.

We conducted clinicopathologic and immunohistochemical analyses of the prevalence of Epstein-Barr virus (EBV) among 24 patients with malignant lymphoma complicating systemic rheumatic diseases. (SRD) These 24 patients included 17 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 2 with systemic sclerosis (SS), and 2 with dermatomyositis (DM). There were 2 men and 22 women ranging in age from 30 to 86 years (mean: 64 years). The interval between the onset of rheumatic disease and that of malignant lymphomas ranged from 3 months to 35 years (mean: 142 months). The use of immunosuppressive drugs before the onset of malignant lymphoma was recorded in 15 patients. Among them, 5 patients received methotrexate (MTX) therapy. Malignant lymphomas were found at extranodal sites in 9 patients, and the disease was in the advanced stage in 17 patients. Histologic and immunohistochemical studies demonstrated that 18 cases (75%) were B-cell lymphoma (RA=12, SLE=2, SS=2, DM=2), 3 (12.5%) were peripheral T-cell lymphoma (RA=3), and 3 (12.5%) were classical Hodgkin lymphoma (RA=2, SLE=1). As in previous reports, there was an increased frequency of diffuse large B-cell lymphoma (50%) in the present series. Moreover, a majority of the diffuse large B-cell lymphomas exhibited activated B-cell phenotype. EBV-encoded small RNAs (Epstein-Barr early region [EBER]-) and/or LMP-1+tumor cells were identified in only 3 cases of classical Hodgkin lymphomas. Our findings suggested EBV-associated lymphoma comprised only a small fraction of all non-Hodgkin's lymphomas in the general SRD patient population.

Adult↗
PubMed2006

Mycophenolate mofetil in dermatomyositis: is it safe?

The authors report 10 patients with idiopathic dermatomyositis treated with mycophenolate mofetil in combination with corticosteroids. Successful steroid taper without disease relapse was achieved in six patients; however, in three patients, treatment was associated with opportunistic infections, leading to death in one patient. The disproportionately high rate of opportunistic infections in this group is considered.

Adult↗
PubMed1976

The childhood type of dermatomyositis.

The childhood type fo dermatomyositis, which occurs in children and young adults, shows a specific constellation of pathologic changes in muscle. Capillary necrosis leads to capillary loss, generally starting on the periphery of muscle fascicles. Electron microscopy discloses undulating tubules in endothelial cells, lymphocytes, pericytes, and pseudosatellite cells. The muscle fiber damage is coextensive with capillary damage and probably results from progressive ischemia. The muscle cells, before atrophying, show mitochondrial elongation, Z disk streaming, focal myofibrillary loss, and occassionally selective thick filament loss. Muscle cell necrosis is rare and limited to infarctlike lesions. Inflammatory infiltrates, if present, occur only in connective tissue septa. The cause of the capillary damage has not been determined.

Adult↗
PubMed1980

Sarcoid myopathy with typical rash of dermatomyositis.

A 46-year-old woman had heliotrope erythema, poikiloderma, and proximal muscle weakness. Histologic examinations of the skin were consistent with dermatomyositis. Muscle biopsy revealed numerous noncaseating granulomatous lesions within the muscle fasciculi, confirming the diagnosis of sarcoid myopathy. No granulomatous lesion could be detected clinically in other organs. A combination of this skin and muscle disorder is rare and has not been reported previously.

Biopsy↗
PubMed1982

Ipecac-induced myopathy simulating dermatomyositis.

We studied a young woman with an eating disorder. To induce vomiting, she took syrup of ipecac daily for 2 years, and then developed insidious, progressive muscle weakness. Skin findings were similar to those of dermatomyositis. Muscle biopsy, however, was similar to experimental emetine myopathy and lacked inflammatory features. Upon cessation of ipecac abuse, strength returned. We believe that this patient had ipecac-induced muscle weakness.

Adult↗
PubMed1996

Elevated serum levels of manganese superoxide dismutase in polymyositis and dermatomyositis.

We studied serum concentrations of manganese superoxide dismutase (Mn SOD) and copper-zinc superoxide dismutase (Cu/Zn SOD) in 22 patients with polymyositis and dermatomyositis (PM/DM), 87 patients with four types of muscular dystrophy, 20 patients with amyotrophic lateral sclerosis, and 15 patients with collagen vascular diseases (CVD). Serum levels of Mn SOD were increased only in the patients with PM/DM and CVD, and the elevation was more prominent in those with PM/DM. Levels of Cu/Zn SOD were slightly elevated in some patients with PM/DM and Duchenne muscular dystrophy. In patients with PM/DM, the change in Mn SOD levels corresponded to disease activity as closely as or more closely than those of creatine kinase. The results indicate that serum Mn SOD may be a useful clinical marker for PM/DM.

Adolescent↗
PubMed1996

Necrotizing myopathy with pipestem capillaries and minimal cellular infiltration: a case associated with cutaneous signs of dermatomyositis.

In 1991, Emslie-Smith and Engel described a distinct form of idiopathic inflammatory myopathy they called "necrotizing myopathy with pipestem capillaries, microvascular deposition of the complement membrane attack complex (MAC) and minimal cellular infiltration." We describe a patient with exercise-dependent painful myopathy related to a necrotizing myopathy with pipestem capillaries in whom mild cutaneous signs of dermatomyositis were detected 7 years after onset and who subsequently developed multiple cerebral infarcts.

Capillaries↗
PubMed1996

Sustained myoglobinuria: the presenting manifestation of dermatomyositis.

We describe two patients with sustained myoglobinuria that did not resolve with supportive treatment. After we established a diagnosis of dermatomyositis and started treatment with corticosteroids, there was rapid resolution of the myoglobinuria. Well-documented cases of inflammatory myopathy causing myoglobinuria are rare, but their prompt recognition has important therapeutic implications.

Adult↗
PubMed1998

Calciphylaxis mimicking dermatomyositis: ischemic myopathy complicating renal failure.

BACKGROUND: Among the complications of chronic renal failure is a syndrome of medial calcification of small- to medium-sized arteries associated with ischemic necrosis of the skin and other organ systems, leading to gangrene and a poor prognosis. The syndrome has been reviewed in the renal, dermatologic, and surgical literature under the term calciphylaxis, which describes a postulated pathogenetic mechanism whereby sensitization to an endogenous or exogenous substance (such as parathyroid hormone) predisposes to calcium deposition after exposure to a challenging agent. Myopathy has rarely been reported as the presenting feature, and the syndrome has not been discussed in the neurologic literature. METHODS: We report two patients with renal failure and systemic calciphylaxis who presented to our hospital with myopathic complaints and signs suggesting dermatomyositis. We also discuss possible disease mechanisms and treatment. CONCLUSIONS: Because early treatment (including aggressively lowering the calcium and phosphate levels and parathyroidectomy) may improve the outcome, early recognition of the syndrome of calciphylaxis is essential.

Adult↗
PubMed1998

Controlled studies with high-dose intravenous immunoglobulin in the treatment of dermatomyositis, inclusion body myositis, and polymyositis.

There are three major subsets of the inflammatory myopathies: polymyositis (PM), dermatomyositis (DM), and inclusion-body myositis (IBM). High-dose intravenous immunoglobulin (IVIg) has been tried in controlled clinical trials in patients with DM and IBM but not with PM. In patients with DM that is resistant or partially responsive to conventional therapies, IVIg was very effective. The treated patients experienced dramatic improvement not only in muscle strength but also of their skin rash. Repeated muscle biopsies with quantitative histologic studies showed the IVIg-treated patients had a statistically significant improvement of the muscle cytoarchitecture, with resolution of the aberrant immunopathologic parameters. In two controlled clinical trials conducted in IBM patients, IVIg showed marginal improvements in muscle strength which were nonsignificant. However, a few IBM patients had a definite clinical improvement with increased activities of daily living, but when analyzed within the entire IVIg-treated group, their total gains in muscle strength did not reach statistical significance compared to the placebo-treated group. Of interest is that certain muscle groups in the IVIg-treated patients, such as the muscles of swallowing, showed significant improvement compared to those of the placebo-treated patients, implying mild regional effects. In PM, uncontrolled trials have shown improvements in muscle strength, but the controlled clinical trial is still ongoing.

Dermatomyositis↗
PubMed2003

Nonspecific interstitial pneumonia associated with polymyositis and dermatomyositis: serial high-resolution CT findings and functional correlation.

STUDY OBJECTIVES: We described the features seen on serial high-resolution CT scans of nonspecific interstitial pneumonia (NSIP) that was associated with polymyositis (PM) and dermatomyositis (DM), and we correlated the changes in the CT scan findings with those of pulmonary function test results. DESIGN, SETTING AND PATIENTS: Serial CT scans of 14 patients with histologically proven NSIP and PM/DM from two university hospitals were evaluated retrospectively (follow-up period, 3 to 61 months; mean follow-up period, 27.6 months). Using initial and follow-up CT scan findings, the extent of each type of opacity and the total area of increased opacity were calculated and correlated with the results of pulmonary function tests. MEASUREMENTS AND RESULTS: The predominant findings on the initial CT scans were of reticular and/or ground-glass opacities with or without consolidation. Reticular and ground-glass opacities predominated in the lower zone of each lung, and consolidation predominated at the lung periphery. Thirteen patients showed significant improvement of the total area of increased opacity (p < 0.05), and this decrease in extent inversely correlated with changes in FVC (r = -0.650; p = 0.031). Ground-glass and reticular opacities also were improved significantly in 11 and 13 patients, respectively. The decrease in the extent of ground-glass opacity correlated inversely with the changes in FVC (r = -0.758; p = 0.0119) and diffusion capacity for carbon monoxide (r = -0.669; p = 0.0448). In one patient, ground-glass opacity progressed, and death occurred after 3 months. Traction bronchiectasis was seen in 12 patients, and it improved in four patients after treatment. Honeycomb lung was not noted in any patient during follow-up. CONCLUSIONS: With treatment, serial CT scans of PM/DM patients with NSIP showed significant improvement in the abnormal opacities, and radiographic progression of lung fibrosis was limited. The CT scan features and clinical course of NSIP in PM/DM patients were relatively uniform, and this constitutes a subset of NSIP.

Adult↗
PubMed2000

Diagnosis of dermatomyositis and polymyositis: a study of 102 cases.

UNLABELLED: Patients with dermatomyositis (DM) or polymyositis (PM) were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p <0.01]. DM patients complained more arthralgia [p <0.05], dysphagia [p <0.03] and weight loss [p <0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p <0.002]. PM presented more significant increase in creatine kinase (CK) [p <0.02] and in alanine aminotransferase (ALT) [p <0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early DM group [p <0.03]. CONCLUSION: A small association with connective-tissue diseases and neoplasms was found. DM and PM are clinically different. DM presents systemic involvement affecting the skin, developing more severe arthralgia, dysphagia and weight loss and presenting higher values of ESR. PM presents a restricted and more significant involvement of muscles generating more weakness complaints and higher levels of serum muscle enzymes.

Adolescent↗
PubMed2003

Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients.

This study was based on a prospective and a retrospective analysis of 35 patients who met Bohan and Peter criteria for juvenile dermatomyositis diagnosis. The mean follow-up time was three years ten months. Calcinosis was present in five (14.28 %) patients, cutaneous ulcers in four (11.42%), and systemic involvement in nine (27.71%) patients. All patients presented alterations in the serum levels of muscle enzymes, and all of them were submitted to muscle biopsy as a diagnostic procedure. Nine (25.71%) patients received corticotherapy prior to and 26 (74.28%) after the muscle biopsy. Chloroquine, methotrexate, cyclosporine, cyclophosphamide and intravenous immunoglobulin were used in patients with poor response to corticotherapy. Continuation of cutaneous manifestations was observed in 4 (11.43%) patients, laboratorial activity in 1 (2.85%), cutaneous and laboratorial activities in 3 (8.57%). Ten (28.57%) patients were out of activity, and 17 (48.57%) in remission at study end-point, on March 2002. Two (5.71%) patients died.

Adrenal Cortex Hormones↗
PubMed2004

Myositis in mixed connective tissue disease: a unique syndrome characterized by immunohistopathologic elements of both polymyositis and dermatomyositis.

OBJECTIVE: To characterize the inflammatory cells, the expression pattern of adhesion molecules (ICAM-1 and VCAM-1), membrane attack complex (C5b-9), and major histocompatibility complex (MHC) antigens in muscle biopsy of mixed connective tissue disease (MCTD). METHOD: We studied 14 patients with MCTD, and compared to 8 polimyositis (PM) patients, 5 dermatomyositis (DM) and 4 dystrophies. Inflammatory cells were examined for CD4+, CD8+, memory and naive T cells, natural killer cells, and macrophages. Expression of MHC-I and -II, ICAM-1, VCAM-1 and C5b -9 were characterized on muscle fibers and vessels. RESULTS: Morphological analysis displayed a pattern of PM. Immunohistochemical study revealed a decreased number of capillaries, predominance of CD4+ and B cells in perivascular regions and predominance of CD8+ and CD45RO+ in endomysial regions. The expression of MHC-I on vessels and on degenerated muscle fibers, MHC-II expression on vessels and perifascicular muscle fibers, and the expression of ICAM-1 / VCAM-1 on endothelial cells indicated both vascular and cellular-immune mediated processes causing the muscular lesion. CONCLUSION: Our findings revealed a mixed mechanism in MCTD, both vascular involvement as DM, and cell-mediated like PM.

Adult↗
PubMed2005

Bone mineral density of Brazilian girls with juvenile dermatomyositis.

We measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.

Absorptiometry, Photon↗
PubMedDate not supplied

Ureteral calcinosis in juvenile dermatomyositis: successful precocious surgical management.

We report a successful surgical intervention to repair bilateral ureteral strictures in a child with juvenile dermatomyositis (JDM) and ureteral calcinosis. This is the fourth reported case in medical literature. A 9-year-old-girl with severe JDM, a rare connective tissue disease characterized by skin and muscles vasculitis, was under immunosuppressive therapy. In the course of the disease, she presented recurrent urinary tract infections. Bilateral ureteral dilation was detected by ultrasound (US) and intravenous pyelogram (IVP). CT scan showed bilateral ureteral calculus. Ureteroscopy revealed bilateral ureteral calcinosis, confirmed by histopathological analysis. Bilateral double-J stents were placed, resulting in transient improvement of ureteral dilation and infection, but only the surgical removal of abnormal ureteral portions was successful. In conclusion, endourological approach is recommended for diagnosis of urinary tract involvement by JDM because radiological evaluation can be misleading. The immunosuppressive treatment and the resection of damaged ureteral segments have allowed the control of urinary complications.

Calcinosis↗