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 739 records · Page 41Linked to original sources
PubMed2006

MxA gene expression in juvenile dermatomyositis peripheral blood mononuclear cells: association with muscle involvement.

Juvenile dermatomyositis (JDM), a systemic vasculopathy, is characterized by inflammation of skin and muscle. Muscle biopsies from untreated JDM patients show upregulation of type I interferon (IFN)-inducible genes, including myxovirus resistance protein A (MxA). The present study examines whether MxA mRNA expression in peripheral blood mononuclear cells (PBMC) from JDM patients: (1) is elevated compared to healthy controls, (2) reflects disease activity, and (3) changes with the onset of clinically effective treatment. MxA mRNA expression in JDM PBMC obtained at the initial clinic visit was elevated compared to controls and was positively correlated with Disease Activity Score (DAS) for muscle, but not with DAS for skin, suggesting that damage to skin and muscle in JDM may each have a discrete pathophysiology. During the course of clinically effective treatment, decrease in muscle symptoms was associated with a decrease in PBMC MxA mRNA expression.

Adolescent↗
PubMed2006

Childhood dermatomyositis associated with intracranial tumor and liver cysts.

We present a girl with dermatomyositis, liver cysts and choroid plexus papilloma who was treated and followed for 7 years. Muscle histology revealed an inflammatory muscle disease and similar changes were detected in a brain tumor that was surgically removed at onset. Western blot analysis of the muscle revealed severely reduced calpain-3 protein. She was treated with pulse methylprednisolone treatment (800 mg i.v. for 4 days) followed by oral prednisone treatment (16 mg on alternate day) for 14 months, which improved muscle strength. Moreover, the cystic liver formations disappeared during steroid treatment. This is an unusual association of muscular disorder, steroid-responsive liver cysts, intracranial tumor and secondary calpain-3 deficiency. We speculate that this association is not coincidental, but mediated by an autoimmune attack against an antigen that is shared among the target tissues.

Antigens, CD↗
PubMed2005

The role of high-dose immune globulin intravenous in the treatment of dermatomyositis.

Dermatomyositis (DM) is a complement-mediated microangiopathy affecting skin and muscle resulting in skin abnormalities, including subcutenous calcifications, muscle weakness and disability. The disease generally responds to steroids or immunosuppressive drugs, but a number of patients are resistant or partially responsive to these therapies, prompting us to examine the efficacy of IVIg. A double-blind placebo-controlled study demonstrated that IVIg is very effective in improving both the muscle strength and the skin rash. The clinical benefit, which was impressive in patients with early disease, was associated with improvement in the muscle cytoarchitecture. Quantitative histological studies in repeated muscle biopsies showed a statistically significant increase in the size of muscle fibers and the number of capillaries with normalization of the capillary diameter. Resolution of the aberrant immunopathological parameters, including interception of complement activation products and downregulation of T cells, ICAM-I, VCAM, TGF-beta and MHC-I molecules, was also noted. Further, a number of immunoregulatory and structural genes were modified in the patients' muscle biopsies after therapy. The study concluded that IVIg is an effective second-line therapy for patients with DM incompletely responding to steroids.

Clinical Trials as Topic↗
PubMed2006

Dermatomyositis associated with malignant melanoma--a marker of poor prognosis?

BACKGROUND: Dermatomyositis (DM) is an inflammatory connective tissue disorder well recognized as a paraneoplastic syndrome in adults. OBJECTIVE: The objective of this study was to assess the prognosis of DM associated with malignant melanoma (MM). PATIENTS AND METHODS: We systematically searched databases (PubMed, MEDLINE, and WEB OF SCIENCE) for articles reporting the concurrence of DM and MM. For the literature study, time of onset of DM in relation to diagnosis of MM (before, concomitant with, or after), stage of MM after restaging (according to the American Joint Committee on Cancer [AJCC] guidelines, 2001), and survival time after diagnosis of DM were recorded. Survival time studies and univariate statistical analyses were performed. Furthermore, we present our own clinical case of a patient with DM concomitantly occurring with regional lymph node metastasis of MM. RESULTS: In 5 cases DM occurred before, in 6 cases concomitantly with, and in 6 cases after progression of MM. Univariate analysis identified the AJCC stage of MM as a significant prognostic factor. Gender, age, and the time interval between onset of DM and progression of melanoma were unrelated. The 1-year actuarial survival rate was 0% for patients with DM when occurring with MM at stage IV and 60% when occurring with MM at stage III (P < .05). The estimated mean survival time was 6.6 months for patients with MM stage IV and 57 months for stage III. LIMITATIONS: The conclusions from this study are limited by the relatively small number of articles that reported the association of MM and DM. CONCLUSION: DM occurring in patients with MM at stage IV is connected with an extremely poor prognosis, whereas the few reported patients with DM and MM at stage III, including our case, have a prognosis similar to stage III patients without DM.

Adult↗
PubMed2006

Multiple eruptive dermatofibromas in a patient with dermatomyositis taking prednisolone and methotrexate.

Dermatofibroma (DF) is a common, benign, dermal tumor, often occurring as a single lesion. Multiple eruptive DFs are rare and usually associated with autoimmune diseases, immunosuppressant therapy, or both. We present the case of a 28-year-old woman with dermatomyositis who developed multiple eruptive DFs after undergoing methotrexate and corticosteroid treatment. Immunosuppressants such as methotrexate and corticosteroids might cause multiple eruptive DFs.

Adult↗
PubMed2004

Immunolocalization of protectin (CD59) and macrophages in polymyositis and dermatomyositis.

Immunohistochemical stainings for protectin (CD59) and for the macrophage/monocyte antigen were performed in muscle specimens of polymyositis (PM) and dermatomyositis (DM) patients. In control muscles, a granular immunostaining of CD59 at the surface of 10-15% from among muscle fibers and an intense reaction in endothelia of blood vessels were noted. Strong immunostaining appeared inside vacuoles of vacuolar degeneration changes of muscle fibers, and in regenerating muscle fibers in PM and DM. No macrophages were found next to the CD59 positive muscle regions. The results seem to strongly support a role of the complement (C) system and of the factors protecting cells from the C-induced damage in the mechanism of muscle injury in PM and DM. The findings can have treatment implications.

Blood Vessels↗
PubMed2005

Fatal acute fibrinous and organizing pneumonia in a child with juvenile dermatomyositis.

Acute fibrinous and organizing pneumonia, a recently described form of diffuse acute lung injury, sometimes affects adults with inflammatory myopathy. We describe a child with juvenile dermatomyositis who had development of acute fibrinous and organizing pneumonia. There does not appear to be a successful method of treatment, particularly in severe cases with respiratory failure.

Acute Disease↗
PubMed2006

Duration of illness is an important variable for untreated children with juvenile dermatomyositis.

OBJECTIVE: To evaluate the impact of duration of untreated symptoms in children with juvenile dermatomyositis (JDM) on clinical and laboratory status at diagnosis. STUDY DESIGN: We examined physical and laboratory data from the first physician visit for 166 untreated children with JDM. Disease activity scores (DASs) assessed skin and muscle involvement. Height and weight were compared with the National Health and Nutrition Examination Survey III dataset. Duration of untreated illness was designated as the time from first sign of rash or weakness to diagnostic visit. RESULTS: Boys and girls with untreated JDM were shorter and lighter than national norms (P > .0005 for both), and nonwhite children were weaker than white children (P > .0005). Older children had more dysphagia (P = .017) and arthritis (P > .001). Duration of untreated JDM was negatively associated with DAS weakness (P > .0005), unrelated to DAS skin, and positively associated with pathological calcifications (P = .006). With untreated disease > or = 4.7 months, serum levels of 4 muscle enzymes (aldolase, lactic dehydrogenase, creatine kinase, serum glutamic-oxaloacetic transaminase/aspartate aminotransferase) tended toward normal (P > .01 for each). CONCLUSIONS: Duration of untreated symptoms is an important variable and should be included in decisions concerning both diagnostic criteria and intensity of therapy for children with JDM.

Abdominal Pain↗
PubMed2004

[Oculomotor muscles involvement revealing dermatomyositis in a patient with rheumatoid arthritis].

INTRODUCTION: Oculomotor muscles (OMM) involvement in dermatomyositis (DM) and in rheumatoid arthritis (RA) is unusual. The DM always leads to OMM inflammation, whereas the RA particularly leads to tenosynovitis of the superior oblique muscle referred to as the Brown syndrome. OBSERVATION: The patient is a 43-year-old woman who gives a 17-year-history of severe seropositive RA with bilateral coxite. She was hospitalized for acute painful proptosis. The clinical examination revealed an orbital erythema and a muscular rhizomelic weakness. The muscular enzymes were increased. The orbital CT revealed in the right side, an enlargement of the superior rectus muscle that was enhanced after intravenous injection, which is compatible with myositis involvement. The muscular biopsy practiced at the level of the calf showed the specific histological signs of the DM. This orbital involvement was resolved with a high dose of corticosteroids. CONCLUSION: Our observation has the specificity of associating RA with DM with an involvement of the superior rectus muscle, which is due to the DM rather than the RA.

Adult↗
PubMed2006

Oral lesions as an initial manifestation of dermatomyositis with occult malignancy.

Dermatomyositis (DM) is an inflammatory disease of skeletal muscle with characteristic cutaneous features. Oral manifestations of DM have been very rarely described. This case describes a patient in whom the oral lesions were among the initial manifestations of DM. The lesions were very painful and the most distressing component of the disease in this patient. High dose systemic steroids were required to control the lesions. Investigations for an occult malignancy in this patient resulted in the identification of a non-Hodgkin's lymphoma in her right kidney. Commencement of chemotherapy rapidly resulted in complete resolution of her oral and skin lesions.

Aged↗
PubMed2006

[Porokeratosis in a patient with dermatomyositis].

There are several reports of porokeratosis in the context of immmunosuppressive diseases. These mainly include organ transplant, HIV infection, lymphomas and some inflammatory and autoimmune diseases commonly treated with immunosuppresive drugs or chemotherapy. Disseminated superficial actinic porokeratosis is the clinical variant of porokeratosis that most frequently develops in immunosuppressive states. We report a case of porokeratosis in a woman with dermatomyositis.

Aged↗
PubMed1998

Dermatomyositis associated with fallopian tube carcinoma.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy associated with characteristic cutaneous and extracutaneous manifestations, including malignancy. Primary fallopian tube carcinoma (FTC) is the least common site of origin for a malignant neoplasm of the female genital tract. This report describes the first documented case of DM and concurrent FTC in the United States. A 62-year-old woman presented with DM and was subsequently found to have FTC. During her clinical course, she had improvement in strength and function with treatment of the underlying FTC, which also correlated with lower creatinine phosphokinase levels. An association between DM and FTC may exist because of significant functional strength improvements after tumor removal and chemotherapy.

Activities of Daily Living↗
PubMed2001

Cardiac troponin and beta-type myosin heavy chain concentrations in patients with polymyositis or dermatomyositis.

Cardiac troponin T (cTnT), cardiac troponin I (cTnI), myosin heavy chains (MHC), myoglobin, creatine kinase (CK), and creatine kinase isoenzyme MB (CKMB), were measured in blood samples from 39 polymyositis (PM) or dermatomyositis (DM) patients without clinical evidence for cardiac involvement to evaluate their clinical usefulness in this patient population. MHC, myoglobin, and CKMB were frequently elevated and correlated with each other and with disease severity. Undetectable cTnI in all but one patient indicated that MHC was released from skeletal muscle, thereby providing the first laboratory evidence of frequent slow-twitch muscle fibre-necrosis in patients with PM or DM. CKMB was elevated in 51%, cTnT in 41%, and cTnI in only 2.5% of patients. cTnI did not correlate with other markers or with disease severity scores. The close correlations found between cTnT and skeletal muscle damage markers and the relationship between cTnT with disease severity without clinical evidence for myocardial damage suggest a release of cTnT from skeletal muscle. The relationship of cTnT with disease severity indicates a possible role of the marker for risk stratification. However, the prognostic values of cardiac troponins and other muscle damage markers in PM/DM patients remain to be compared in prospective outcome trials.

Adolescent↗
PubMed1992

Recurrent juvenile dermatomyositis and cutaneous necrotizing arteritis with molecular mimicry between streptococcal type 5 M protein and human skeletal myosin.

An adult patient had a syndrome associating the features of juvenile dermatomyositis and cutaneous polyarteritis nodosa that followed a cyclic course from childhood; recurrences were always associated with a rise of serum antistreptococcal antibodies. Regions of homology between streptococcal type 5 M protein and skeletal myosin were found. These findings suggest that streptococcal infection, possibly through a molecular mimicry mechanism, played a role in the pathogenesis of the disease in our patient.

Acute Disease↗
PubMed1984

Course of treated juvenile dermatomyositis.

Sixty-six patients with possible juvenile dermatomyositis (JDMS) were observed at the Children's Hospital of Los Angeles from 1960 to 1982. In patients initially given high doses of corticosteroids followed by low-dose therapy, three different clinical courses had previously been observed: monocyclic, polycyclic, and chronic continuous. We reviewed the records of 32 patients who met study criteria. The course of JDMS was monocyclic in eight children, chronic polycyclic in 10, and chronic continuous in 14. Of these children, 25 are well and not receiving medication; one has mild JDMS, without corticosteroid therapy; four have active JDMS despite corticosteroid therapy (one is severely handicapped); and two have died. Our results support the improved prognosis of JDMS after corticosteroid therapy, but also the great clinical variability of the disease. Understanding of this variability, as reflected in the three disease courses, facilitates physician choice of the optimal treatment with the least drug toxicity for the individual patient, continuing efforts to clarify the disease pathogenesis, and research efforts to improve current treatment programs for the patient with severe JDMS.

Acute Disease↗
PubMed1996

Failure of pulse intravenous methylprednisolone treatment in juvenile dermatomyositis.

OBJECTIVE: To review the experience with high-dose intravenous pulse methylprednisolone (IVMP) therapy in patients with juvenile dermatomyositis (JDM) in our institution. STUDY DESIGN: We reviewed the charts of seven consecutive patients (four female subjects; three male subjects; age 3 to 18 years (mean age 8 years) treated for JDM between 1989 and 1992. RESULTS: All patients met the criteria of Bohan and Peter for JDM and were treated within 3 months of onset of weakness. All received initial treatment with IVMP, 30 mg/kg per day for 3 days, administered weekly for between 1 and 4 weeks. Treatment with orally administered prednisone was required because of deteriorating muscle strength and persistent elevation of creatine kinase activity despite IVMP in five patients, increased skin vasculitis in one patient, and elevated creatine kinase activity with no improvement in strength in one patient. CONCLUSION: We conclude that muscle strength in patients with JDM may deteriorate during pulse IVMP therapy when this is used alone as initial treatment, even early in the course of the illness. We recommend either conventional high doses of orally administered corticosteroids or IVMP with oral corticosteroid therapy as initial treatment of JDM.

Administration, Oral↗
PubMed1980

Ureteral diverticulosis in a case of dermatomyositis.

We present the third case of ureteral diverticulosis discovered by excretory urography alone and the twenty-sixth case reported in the world literature. Unlike previously reported cases our patient had dermatomyositis and is urologically asymptomatic.

Dermatomyositis↗
PubMed1978

Probable endometrial carcinoma of the prostate, crossed renal ectopia and dermatomyositis in a 32-year-old man.

An unusual type of prostatic carcinoma associated with crossed renal ectopia and dermatomyositis is reported in a young man. Infiltration by an epithelial tumor filled the enlarged prostate with soft papillary masses. Cystoprostatectomy and removal of a single large pelvic lymph node metastasis were done. Histologically, the tumor resembled endometrial carcinoma and may have arisen from paramesonephric tissue in the prostate. Large amounts of immunoglobulins were found in the tumor tissue. The patient has survived for more than 5 years, although a slow growing urethral recurrence has been noted.

Adenocarcinoma↗