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PubMed2025

Alu Overexpression Leads to an Increased Double-Stranded RNA Signature in Dermatomyositis.

OBJECTIVE: Dermatomyositis is an autoimmune condition characterized by a high interferon signature of unknown etiology. Because coding sequences constitute <1.2% of our genomes, there is a need to explore the role of the noncoding genome in disease pathogenesis. Our genomes include roughly 1.2 million Alu elements occupying approximately 10% of the genome, which can form double-stranded (ds) RNA capable of triggering MDA5 leading to interferon production. METHODS: We aligned muscle biopsy RNA sequencing data to the telomere-to-telomere reference genome and quantified short interspersed elements including Alus. Because Alus have a propensity to form dsRNA and are the major targets of both adenosine deaminase RNA specific and MDA5, we quantified adenosine to inosine (A-to-I) RNA editing, which reflects dsRNA in vivo. RESULTS: Dermatomyositis muscle (n = 39) showed a global elevation in Alu expression (including inverted-repeat Alus with high potential to form dsRNA) as well as an increased expression of unique Alu elements (n = 557, q < 0.05) compared with healthy controls (n = 34), in a pattern not seen in other myositis types (n = 81). Most (75.3%) of these Alus originated from genomic regions outside genes. A cluster of the uniquely overexpressed Alus (n = 167) correlated with interferon-stimulated genes and markers of myositis activity. Additionally, we found a uniquely expanded Alu A-to-I editome in dermatomyositis, reflecting an increase in dsRNA. Edited Alus clustered on chromosome 19, which is known to have the highest concentration of dsRNA. CONCLUSION: We hypothesize that overexpressed Alus in dermatomyositis form endogenous dsRNA that exceeds the capacity of RNA editing enzymes and triggers dsRNA sensors leading to interferon production.

Humans↗

PubMed2004

Monocyte/macrophage differentiation in dermatomyositis and polymyositis.

Recent advances have revealed significant differences in the pathogenesis of inflammatory myopathies. To determine whether different patterns of macrophage differentiation are a useful tool to delineate the major groups of inflammatory myopathies, the muscle biopsies of 11 patients with dermatomyositis and 12 patients with polymyositis were studied using different macrophage markers. In polymyositis, the early-activation markers MRP14 and 27E10 stained the majority of macrophages, which were recognized by the pan-macrophage marker Ki-M1P and which were located primarily in the endomysium. In dermatomyositis, macrophages predominantly expressed the late-activation marker 25F9 and were found mainly in the perimysium. Thus, the location and presence of different subsets of macrophages distinguish dermatomyositis and polymyositis. The predominance of early-activated macrophages in polymyositis indicates a more acute disease process. The findings in dermatomyositis, by contrast, suggest a role of persistent monocytes/macrophages in the disease process.

Adult↗

PubMed1997

Serum CA-125 screening for ovarian cancer in patients with dermatomyositis.

Ovarian cancer is the most overrepresented malignancy diagnosed in women with dermatomyositis. Unfortunately, screening with pelvic examination rarely detects this cancer prior to the development of metastatic disease. Our objective was to examine the use of serum CA-125 antigen levels in screening patients with dermatomyositis for ovarian cancer. A single blinded, case-control study was conducted in our institution of CA-125 levels in 14 women diagnosed with dermatomyositis between 1986 and 1993, 4 of whom subsequently developed ovarian cancer. In the 4 patients who developed ovarian cancer ("cases"), CA-125 determinations were performed on serum stored 5 to 19 months prior to the diagnosis of ovarian cancer. In the remaining 10 patients ("controls"), serum was drawn for CA-125 level determination at the time of the study, and simultaneous gynecologic and endovaginal ultrasound examinations were performed to exclude clinical evidence of ovarian cancer. All CA-125 serum measurements were performed simultaneously by a technician blinded to disease status using one diagnostic kit. CA-125 was found to be elevated in 2 patients with ovarian cancer (on serum obtained 5 and 13 months prior to the date of diagnosis of ovarian cancer) and in none of the control patients without clinical or ultrasound evidence of ovarian cancer (relative risk = 20, 95% confidence interval = [0.64, 666]). In these 14 patients, the sensitivity of CA-125 elevation for detection of ovarian cancer was 50%, and specificity was 100%. Serum CA-125 screening for ovarian cancer in patients having dermatomyositis may be useful; however, prospective studies are needed to confirm this and to determine the effect of screening on cancer stage at diagnosis and long-term survival.

Aged↗

PubMed1993

Connection of atopic disease in Japanese patients with juvenile dermatomyositis based on serum IgE levels.

Serum IgE levels of 22 patients with juvenile dermatomyositis (JDMS), 44 normal children, and 43 patients with adult dermatomyositis were compared. The geometric mean of serum IgE levels was significantly higher in the juvenile patients when compared with normal children (p < 0.01) and adult patients (p < 0.01). Of the 22 patients with JDMS, 11 (50%) had elevated serum IgE levels accompanied by atopic disease. Dermatomyositis was accompanied by atopic dermatitis (AD) in 9 (41%) of these 22 patients, a high prevalence when compared with reports among the general population. Following the appearance of muscular symptoms in JDMS patients with AD, the ratio of OKT4 to OKT8 cells rose due to a reduction in the percentage of OKT8-positive cells, along with further elevations in serum IgE levels and intractable cutaneous manifestations of dermatomyositis. All this may be a result of an interaction between the immuno-mechanism of this disease and that of AD. We suspect that, in general, children with impaired cell-mediated immunity, presenting with such symptoms as AD, may have a higher tendency for developing JDMS.

Adolescent↗

PubMed2002

Peripheral T cell lymphoma presenting as dermatomyositis-like eruption.

BACKGROUND: There are several conditions reported to mimic the cutaneous manifestations of dermatomyositis, including lymphoproliferative disorders. OBJECTIVE: This case report presents an unusual case of peripheral T cell lymphoma mimicking dermatomyositis and discusses the clinical and pathologic features that distinguish it from dermatomyositis. METHODS AND RESULTS: A 62-year-old woman presented with a two-month history of a progressive painful cutaneous eruption and interstitial infiltrates on chest x-ray. Skin biopsy revealed peripheral T cell lymphoma. The diagnosis was confirmed by lung biopsy. CONCLUSIONS: Although rare, a lymphoproliferative disorder must be included in the differential diagnosis of a cutaneous dermatomyositis-like eruption.

Dermatomyositis↗

PubMed1982

The heart and cardiac conduction system in polymyositis-dermatomyositis: a clinicopathologic study of 16 autopsied patients.

We reviewed the clinical records of 16 patients with polymyositis-dermatomyositis syndromes autopsied at The Johns Hopkins Hospital to determine the nature and extent of cardiac involvement and its correlation with the severity of disease as a whole. The adult patients ranged in age from 32 to 84 years (average 56); the 2 children were aged 2 and 10 years. The duration of disease ranged from 1 to 72 months (average 21). Seven patients had dermatomyositis, 5 had dermatomyositis with malignancy, 2 had childhood dermatomyositis, and 2 had an overlap syndrome. Seven patients had clinical evidence of congestive heart failure, 4 of whom had microscopic evidence of myocarditis. Two patients had bundle branch block; in 1 there was direct involvement of the conduction system by myositis and contraction band necrosis. Evidence of active myocarditis was present in 4 patients (25%); all had congestive failure. Focal myocardial fibrosis was present in 4 patients. Vascular alterations were present in the coronary arteries in 5 patients (31%). Three had active vasculitis, 1 had intimal proliferation, and 1 had medical sclerosis with calcification. All patients with active myocarditis had skeletal muscle involvement. Nine patients had myositis without myocarditis. There was no correlation of overall severity of the disease with the presence or absence of active myocarditis. The present study shows that cardiac involvement may be common in polymyositis; congestive failure or conduction abnormalities arising in this setting may be indicative of myocarditis.

Adult↗

PubMed1991

Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients.

PURPOSE: Polymyositis and dermatomyositis are inflammatory muscular diseases of unknown cause. Many interventions are available to treat patients with these conditions including corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. However, these therapies are not always effective, and they may be associated with certain serious side effects. An attempt was made to evaluate the efficacy of polyvalent intravenous immunoglobulin (IVIG) in patients with polymyositis or dermatomyositis refractory to traditional treatment. PATIENTS AND METHODS: Twenty patients (16 women and 4 men; mean age 43 [16 SD] years), 14 with chronic refractory polymyositis and six with dermatomyositis, received high doses of IVIG because of the failure of traditional treatments (prednisone [19], methotrexate [10], azathioprine [6], cyclophosphamide [3], cyclosporine [3], chlorambucil [1], plasmapheresis [8], lymphopheresis [1], and total body irradiation [1]). In one patient with positive results on picornavirus serologic testing, IVIG was the first treatment choice. IVIG therapy was given with prednisone in 15 patients, with methotrexate in six patients, and with plasmapheresis in one patient. There were no changes in treatment in the 2 months before the introduction of IVIG therapy and no increases in dose during this treatment. Preparations of polyvalent human intravenous gammaglobulins with increased intact immunoglobulin G were used. Thirteen patients received 1 g/kg daily for 2 days each month, and seven patients received 0.4 g/kg daily for 5 days each month. The mean duration of treatment was 4 months. RESULTS: Clinical assessment, which consisted of the measurement of proximal muscle power, and biochemical studies were carried out before each treatment period. Significant clinical improvement was noted in 15 of the 20 patients. Mean muscle power estimated for the 20 patients before and after IVIG therapy was statistically significantly reduced (p less than 0.01). Eighteen patients showed biochemical improvement, and two patients with normal initial serum creatine kinase levels showed clinical improvement. Mean creatine kinase levels for the 20 patients during IVIG therapy showed a statistically significant decrease from the first IVIG perfusions (p less than 0.01). Side effects of IVIG therapy were noted in four patients; however, these effects were mild. During IVIG therapy, steroid doses were significantly reduced from the second or the third IVIG infusion (p less than 0.05). CONCLUSION: IVIG is an efficacious new therapy for polymyositis and dermatomyositis and should play a role in the treatment of these diseases, replacing or reducing steroid and immunosuppressive medications.

Adult↗

PubMed1991

Treatment of dermatomyositis with intravenous gammaglobulin.

PURPOSE: The mainstay of pharmacologic therapy in patients with dermatomyositis is corticosteroids. However, because patients sometimes become refractory to these drugs and because these drugs have potential short- and long-term toxicities, alternate therapy is highly desirable. Therefore, a pilot study was initiated using high-dose intravenous gammaglobulin (IVGG) in the treatment of dermatomyositis. PATIENTS AND METHODS: IVGG was administered to five patients with juvenile dermatomyositis. Prior to IVGG treatment, all patients had persistent muscle weakness despite daily corticosteroids and three patients had developed unacceptable steroid toxicity. Two of the patients had previously developed toxicity while receiving immunosuppressive therapy. RESULTS: IVGG therapy resulted in improved muscle strength and ameliorated skin rash in all patients. The percentage increase in muscle strength as measured by sphygmomanometry following the 9-month course of IVGG ranged from 56% to 606% in the proximal lower extremities and from 30% to 186% in the proximal upper extremities. Following IVGG therapy, prednisone could be discontinued or the dose reduced in all patients. CONCLUSION: This study suggests that IVGG may allow steroid sparing in dermatomyositis and may provide a safe alternative to cytotoxic therapy.

Adrenal Cortex Hormones↗

PubMed2005

Rapamycin (sirolimus) as a steroid-sparing agent in dermatomyositis.

Dermatomyositis is an autoimmune condition that results in significant morbidity and mortality through effects on muscle and skin. Corticosteroids are the mainstay of therapy of dermatomyositis, and severe morbidity and mortality occurs in part through the known long-term side effects of chronic steroid use. In addition, dermatomyositis is commonly associated with underlying malignancy, and high-dose steroids may adversely impair treatment of these malignancies. We describe the first use of rapamycin in a young patient with dermatomyositis.

Adult↗

PubMed2006

Genetically determined imbalance between serum levels of tumour necrosis factor (TNF) and interleukin (IL)-10 is associated with anti-Jo-1 and anti-Ro52 autoantibodies in patients with poly- and dermatomyositis.

Our aim was to investigate presence of tumour necrosis factor (TNF) and interleukin (IL)-10 in serum and their relation to different genotypes as well as to clinical and laboratory phenotypes in patients with polymyositis and dermatomyositis. In 65 patients with poly- or dermatomyositis the inflammatory cytokine balance was evaluated by the assessing absolute levels as well as the ratio between TNF and IL-10 in serum. These levels were correlated to the G-308A TNFA, G-1087A IL10 and G915C TGFB1 gene polymorphisms and haplotype frequencies, gender, autoantibody profiles and clinical manifestations. Increased serum levels of TNF and IL-10 were observed in patients compared to controls. A significantly higher TNF:IL-10 ratio was detected in female poly- and dermatomyositis patients carrying the TNF2 allele compared to female patients with the TNF1/TNF1 genotype (median+/-IQR 1.513+/-0.0.679 vs. 0.950+/-1.173, p=0.021). This ratio was also significantly higher in patients with the extended MICA5.1/TNF2/TNFa2/DRB1*03 haplotype compared to patients lacking this haplotype. A significantly higher TNF:IL-10 ratio was recorded in sera of patients with anti-Ro52 (1.513+/-1.275 and 1.276+/-0.671, positive vs. negative, p=0.010) antibodies and in women with anti-Jo-1 (1.919+/-0.918 and 1.281+/-0.790, positive vs. negative, p=0.041). Our data suggest that a genetically programmed cytokine imbalance exists in patients with poly- or dermatomyositis and that this imbalance is related to the presence of disease-associated autoantibodies.

Adult↗

PubMed2005

[Dermatomyositis with cutaneous necrosis revealing a fallopian tube carcinoma].

INTRODUCTION: Adult dermatomyositis is a rare inflammatory myopathy associated with typical cutaneous lesions and an increased incidence of internal malignancies, notably cancers of the female genital tract. Nevertheless, fallopian tube carcinoma is exceptionally associated with dermatomyositis. EXEGESIS: We report an unusual case of dermatomyositis because of cutaneous necrosis revealing a cancer of the fallopian tube. CONCLUSION: Predictive factors of cancer can improve prognosis of dermatomyositis due to earlier diagnosis of associated cancer. In our observation as in literature review, cutaneous necrosis lesions are highly predictive of an associated neoplasia even as rare as a fallopian tube carcinoma.

Carcinoma↗

PubMed1986

Polymyositis-dermatomyositis and malignant lesions: does an association exist?

To examine the purported association between polymyositis-dermatomyositis and malignant lesions, we identified all patients with histopathologically proven polymyositis-dermatomyositis examined at our institution from 1965 to 1974. For each patient, a control subject was selected, matched for age, sex, race, year and department of clinical evaluation, and geographic area of residence. Although the proportion of patients who ever had a diagnosis of malignant disease (25%) was greater than that for controls (17%), the difference was not statistically significant (P = 0.14). The slight excess of cancer seen among patients was contributed by the most distant referrals and may best be explained on the basis of referral bias. No clinical differences were found between patients with polymyositis-dermatomyositis who had a malignant neoplasm and those who did not, and intensive clinical investigation to exclude "occult" underlying malignant growths did not seem to be cost-effective. Although the present study does not prove that there is no association between polymyositis-dermatomyositis and malignant lesions, the existence of a clinically significant relationship seems unlikely.

Adolescent↗

PubMed1999

Dermatomyositis as a presenting symptom of ovarian cancer.

BACKGROUND: Among gynecologic malignancies, the coexistence of ovarian cancer and dermatomyositis is most frequent. CASE: A 75-year-old woman presented with dermatomyositis, and a search for underlying malignancy found an otherwise asymptomatic ovarian carcinoma with para-aortic lymph node metastases. After resection and chemotherapy, the muscle weakness and skin lesions relating to dermatomyositis began to improve. CONCLUSION: Dermatomyositis can be the only presenting symptom of ovarian cancer, so an evaluation for suspected underlying malignancy should be done.

Aged↗

PubMed1996

Vesicle formation in dermatomyositis associated with gynecologic malignancies.

Vesicle formation in dermatomyositis is rare. We describe two women with dermatomyositis and vesicle formation on their extremities. Both had an ovarian cancer and histologic examinations revealing subepidermal vesicles. In both patients, direct immunofluorescence did not reveal deposition of immunoglobulin in the basement membrane zone. Of our two patients and the 17 previously reported to have dermatomyositis with vesicle formation, 10 had an internal malignant disease. Of these 10 patients, eight women had gynecologic malignant disease and two men had gastric cancer and lung cancer, respectively. Vesicle formation in dermatomyositis is strongly related to the presence of an internal malignant process, especially gynecologic malignant disease in female patients.

Dermatomyositis↗

PubMed1996

Leishmaniasis presenting as a dermatomyositis-like eruption in AIDS.

Three patients are described with leishmaniasis and AIDS, with cutaneous lesions mimicking dermatomyositis. Leishmania organisms were observed in great numbers in the dermis of lesional skin biopsy specimens. They were also present inside keratinocytes in all layers of the epidermis in one patient. Skin cultures from all patients and bone marrow culture in patients 1 and 3 revealed Leishmania infantum. Leishmania organisms were also found in nonlesional skin. The absence of proximal symmetric muscle weakness, elevated muscle enzymes, myopathic electromyograms, or characteristic histopathologic and immunologic features of dermatomyositis, and the rapid and complete clearance or marked improvement of the cutaneous lesions after treatment for leishmaniasis, make us consider true dermatomyositis unlikely. We suggest that leishmaniasis be included in the list of diseases capable of inducing a dermatomyositis-like eruption.

AIDS-Related Opportunistic Infections↗

PubMed1997

Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis.

BACKGROUND: The cutaneous manifestations of dermatomyositis can be the most prominent finding and are often difficult to treat. OBJECTIVE: Our purpose was to determine whether low-dose methotrexate administered weekly in combination with other systemic therapies or as a sole systemic agent is effective in the treatment of the cutaneous disease in patients with dermatomyositis. METHODS: We reviewed the records of 13 patients who received oral methotrexate in doses ranging from 2.5 to 30 mg weekly. Their skin lesions had not been completely responsive to sunscreens, topical corticosteroids, oral prednisone, oral antimalarial therapy, and, in one patient each, chlorambucil and azathioprine. RESULTS: At the end of the study period, 4 of these 13 patients were free of all cutaneous manifestations of dermatomyositis, and another four had almost complete clearing. In the remaining five patients, methotrexate induced moderate clearing of their cutaneous lesions. In all patients, the addition of methotrexate allowed reduction or discontinuation of other therapies such as prednisone. All patients tolerated the methotrexate with minimal toxicity. CONCLUSIONS: Low-dose oral methotrexate administered weekly is effective in treatment of the cutaneous manifestations of dermatomyositis and frequently enables a reduction or discontinuation of corticosteroid therapy.

Administration, Cutaneous↗

PubMed1996

Spontaneous esophageal rupture in adult dermatomyositis.

Dermatomyositis is a chronic inflammatory myopathy with severe prognosis. A 57-year-old woman suffering from dermatomyositis is presented who, in the course of the disease, developed acute spontaneous esophageal rupture due to dermatomyositis involvement of the esophagus. She was successfully treated with total esophagectomy and stomach interposition. This is the first report of spontaneous rupture of esophagus in dermatomyositis.

Dermatomyositis↗

PubMed1999

Dermatomyositis in association with transitional cell carcinoma of the bladder.

We report a case of dermatomyositis occurring in association with transitional cell carcinoma of the bladder. The case illustrates the importance of a thorough search for neoplasms in elderly patients with dermatomyositis and is a reminder that bladder cancer may be a rare cause of dermatomyositis. The case also shows that successful treatment of an underlying tumour may lead to resolution of paraneoplastic dermatomyositis, and relapse of cutaneous and muscle symptoms and signs may indicate recurrence of tumour.

Aged↗