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PubMed1996

Dermatomyositis sine myositis: association with malignancy.

OBJECTIVE: To describe dermatomyositis (DM) sine myositis as a distinct diagnostic subclass of DM and the association with malignancy. METHODS: This case series includes all patients presenting to our primary and tertiary care outpatient dermatology department with DM and DM sine myositis between 1986 and 1993. Seven patients were diagnosed with classic DM and 12 with DM sine myositis. We analyzed average age of onset, duration of followup, clinical course, and incidence of associated malignancy in the patients with classic DM and DM sine myositis. RESULTS: No statistically significant differences were found in these 2 groups on examining age at onset and frequency of associated malignancy. Malignancy was diagnosed in 4 of 12 (25%) patients with DM sine myositis followed for a total of 50.8 patient-years (average followup 51 months) and in 2 of 7 (28%) patients with classic DM followed for a total of 20.6 patient-years (average followup 35 months). CONCLUSION: Although these patients with DM are select and few, they demonstrate that DM sine myositis may be quite similar to classic DM. It may be said that like DM, DM sine myositis may represent a paraneoplastic syndrome. DM sine myositis should be added to the currently accepted polymyositis/dermatomyositis classification system, with appropriate emphasis on the potential association with malignancy.

Adult↗
PubMed1996

Clinical significance of specific autoantibodies in juvenile dermatomyositis.

OBJECTIVE: To determine the prevalence and clinical association of myositis specific antibodies in an unselected group of patients with juvenile dermatomyositis (DM). METHODS: The sera of 42 subjects, representing an unselected group of patients from a single center, with juvenile DM and 7 others with idiopathic inflammatory myopathy (IIM) were examined for the presence of myositis specific antibodies by immunodiffusion against calf thymus extract and immunoprecipitation with HeLa extract. RESULTS: Of the subjects with juvenile DM, only 2 had evidence of antibodies specific to myositis (anti-Mi2). Three other patients with juvenile DM had defined autoantibodies not usually considered to be specific to myositis. Two of the 3 subjects had anti-PM-Scl; both developed features of scleroderma after the juvenile DM remitted. The 5 subjects with defined autoantibodies did not differ clinically from the remainder of the subjects with the exception of the late development of scleroderma features in 2. Fourteen other subjects with juvenile DM had unidentified bands on immunoprecipitation, which may represent as yet undiscovered myositis specific antibodies. No myositis specific antibodies were detected in any of the 7 subjects with other IIM syndromes. CONCLUSION: Based on our findings, we do not recommend routine clinical testing for these antibodies in children with typical juvenile DM. Further study of the unidentified bands seen in our subjects may lead to better understanding of the clinical groupings and etiopathogenesis of childhood myositis.

Adolescent↗
PubMedDate not supplied

Abnormal numbers of cytokine producing cells in patients with polymyositis and dermatomyositis.

OBJECTIVE: To examine the constitutive pattern of cytokine production in patients with polymyositis (PM) and dermatomyositis (DM). METHODS: PBMC secreting the type 1 cytokines IL-2 and IFN gamma and the type 2 cytokines IL-6 and IL-10 were monitored by ELIspot assay. RESULTS: The frequency of IFN gamma-secreting PBMC was reduced 2-20 fold in patients with PM and DM when compared to normal controls (p < 0.01). This change correlated inversely with disease activity (p < 0.01). PM and DM were also characterized by increased numbers of cells secreting IL-10. Patients with DM had fewer IL-2 (p < 0.03) and more IL-6 (p < 0.006) secreting cells than normal controls or patients with other myopathies. CONCLUSIONS: Abnormal patterns of cytokine production characterize patients with PM and DM.

Adult↗
PubMed1996

Interstitial lung disease related to dermatomyositis. Comparative study with patients without lung involvement.

OBJECTIVE: To compare clinical and histopathological data and outcome of patients with dermatomyositis (DM) with and without interstitial lung disease (ILD). METHODS: Patients diagnosed with definite DM were prospectively evaluated. Clinical and analytical data were recorded and muscle biopsies were performed. In patients with respiratory symptoms an extensive pulmonary evaluation was done. RESULTS: 104 patients were diagnosed with idiopathic inflammatory myopathy, 63 with DM. Eight of the patients with DM (13%) also had associated ILD. Arthralgia and fever were more frequently seen in the ILD group and there was no associated malignant condition. Either atypical or nonspecific cutaneous lesions were more frequently seen in ILD patients. Anti Jo-1 antibodies were positive in 75% of the patients with ILD and in 3% of patients with DM without ILD (p < 0.001). Bronchoalveolar lavage measured for cell count showed an increased lymphocyte count in 3/5 cases in which this data was recorded. Lung biopsies showed interstitial pneumonitis in 2 cases and desquamative interstitial pneumonitis in one. All patients achieved complete remission of muscular involvement. With respect to lung disease, 4/8 obtained complete remission, and the other 4 showed partial improvement. No significant statistical differences in terms of survival were found between the 2 groups. CONCLUSION: ILD associated with DM represents a subgroup of DM with clinical and biological differences. However, such patients do not have poorer prognosis than patients without ILD if they are managed aggressively with immunosuppressive drugs.

Aged↗
PubMed1996

[Outcome in patients with interstitial lung disease and polymyositis-dermatomyositis--a subgroup with poor prognosis].

To determine the role of interstitial lung disease (ILD) in the outcome of polymyositis-dermatomyositis (PM/DM), we studied 41 patients who had radiographic evidence of ILD among 51 patients with PM/DM. The patients were divided into 3 groups according to therapy: progressive, no change, or improved. Those classified as progressive had significantly higher rates of skin and lung symptoms, milder muscle symptoms, and lower levels of muscle enzymes than the other 2 groups, and none was positive for the anti-Jo-1 autoantibody. Nine patients with DM died of respiratory failure; they had low serum creatine kinase levels, no anti-Jo-1 antibodies, and a rapidly progressive course before death. Autopsy findings were obtained in 7 patients, and all had diffuse alveolar damage as the main pulmonary histological finding. A subgroup of patients with PM/DM-ILD have a poor prognosis, and ILD in PM/DM is an important prognostic factor.

Dermatomyositis↗
PubMed1996

Regression of calcinosis during diltiazem treatment in juvenile dermatomyositis.

An 8-year-old girl with juvenile dermatomyositis (DM) developed dystrophic calcifications 26 months after diagnosis. She also had severe steroid induced bone loss (osteoporosis). The calcifications turned into generalized heterotopic calcinosis with an exoskeleton-like pattern, despite successful treatment of her myopathy with methylprednisolone and immunosuppressive drugs. She was subsequently treated with oral diltiazem (5 mg/kg/day) to control calcinosis and oral pamidronate (4 mg/kg/day) in addition to calcium and vitamin D supplementation, which she had been taking for 3 years. After 21 months of treatment, clinical and radiological examination revealed dramatic regression of the calcinosis. Bone mass reached normal levels, as determined by bone absorptiometry. Diltiazem alone or in combination with other drugs could be a useful therapy in patients with juvenile DM and pronounced calcifications.

Calcinosis↗
PubMedDate not supplied

Intravenous immunoglobulin therapy in juvenile dermatomyositis.

To investigate the effects of intravenous immunoglobulin (IVIG) on patients of juvenile dermatomyositis (JDM), seven children with JDM were given monthly IVIG in conjunction with other treatments. The indications included disease exacerbation, inability to reduce dose of steroid, replacing cytotoxic drug because of complication and remission-induction for those with severe initial manifestations. Two patients were reported to improve slowly and maintained improved status while on a markedly reduced dose of steroid. One patient responded favorably to the first three courses only. Three patients had evident and quick responses, which could be seen as early as two or three days after the infusion. One patient failed to experience any beneficial effect by IVIG. Four initial responders had an aggravation some time after discontinuing the monthly regimen. The untoward reactions included only two occasions of fever, and another two occasions of fever associated with transient proteinuria. In conclusion, IVIG may be of value in terms of its quick, pulsatile effect and the adjuvant potential to reduce the side effect resulting from exposure to steroid or other immunosuppressive agents as well. However, its long-term efficacy is doubtful, based on this observation.

Activities of Daily Living↗
PubMed1997

[Dermatomyositis and small cell tumor of the kidney].

Dermatomyositis is an inflammation of the striated muscle with an important leukocyte infiltrate which is accompanied by a characteristic cutaneous exanthema. In the international classification we find DM associated with neoplasm in 10% of cases. It is accepted that neoplasms are related to DM if it does not exceed it in two years (prior o after the onset of the disease. The most commonly associated are lung, ovary and breast cancers. In the pas ten years only two cases of DM associated to renal cancer (both in renal cell cancers) have been published. In this article we present a case history of a woman with a DM associated to a renal oat-cell carcinoma. Also, we will review the literature on this theme and will evaluate the predictive parameters of the presence of malignancies in this pathology.

Aged↗
PubMedDate not supplied

Levels of cytokine inhibitors: a possible marker of disease activity in childhood dermatomyositis and polymyositis.

The levels of cytokines and of their inhibitors were assessed by ELISA in serum samples from 3 children with dermatomyositis (DM) and polymyositis (PM) who were followed for several years. We observed normal levels of IL-6 and TNF alpha, but increased concentrations of IL-1Ra and TNF-sR75 at disease onset, which was followed by a decrease in the levels of IL-1Ra and TNF-sR75 in the patients with a favorable disease outcome. In contrast, in the one patient who relapsed no correlation with the levels of cytokines or of their inhibitors could be established. These results suggest that cytokine inhibitors such as IL-1Ra and TNF-sR may be useful additional parameters for monitoring the evolution of DM and PM.

Adrenal Cortex Hormones↗
PubMed1997

A retrospective study of 13 Oriental children with juvenile dermatomyositis.

This is a retrospective study of 13 Oriental children with juvenile dermatomyositis (JDM). We reviewed data found in the hospital records of children diagnosed to have definite (n = 4), probable (n = 7) and possible (n = 2) JDM who presented over a 10-year period at 4 centres in Singapore and compared our results with the experience of others. We found an overall female preponderance (female to male ratio of 3.3:1) but an equal sex ratio in children below 5 years of age. The majority (92%) had insidious onset and good outcome. Diagnosis was often delayed because of the insidious onset, and because weakness occurred late, was mild or absent. Only one child had an acute presentation and refractory course. She died despite aggressive therapy. Clinical features, complications and mainstay medication used were similar to Western studies.

Adolescent↗
PubMed1997

Fulminant Pneumocystis carinii pneumonia in 4 patients with dermatomyositis.

Between 1989 and 1996, 4 cases of Pneumocystis carinii pneumonia (PCP) were observed in patients seronegative for the human immunodeficiency virus who were receiving corticosteroid therapy for dermatomyositis in our institution. These cases were considered unusual in light of the short delay of their onset after initiation of immunosuppressive therapy and their fulminant course: 3 of these patients died of PCP occurring during the first month of treatment with prednisone. In all 4 patients lymphopenia was observed before the initiation of corticosteroid treatment and low CD4 and CD8 cell counts were evident at the time of PCP. These observations support the view of an increase in both the severity and incidence of PCP in patients without human immunodeficiency virus infection and question the need for a primary prophylaxis in patients with connective tissue diseases receiving high-dose corticosteroid therapy.

Adult↗
PubMed1997

Juvenile dermatomyositis in north India.

OBJECTIVE: To determine the clinical profile and therapeutic response of patients with juvenile dermatomyositis (JDM). DESIGN: Hospital based descriptive follow-up study. SAMPLE: 12 patients attending the Pediatric Rheumatology and Immunology Clinic over last five years. RESULTS: The patients were aged between 3 1/2 years to 12 years with a male to female ratio of 2:1. All patients had proximal muscle weakness at presentation. Distal muscle weakness and masseter atrophy was seen in 2 patients and neck flexor weakness and pharyngeal weakness was seen in 1 case. Muscle pain, tenderness or swallowing difficulties were not observed. Classical skin manifestations of JDM were present in all except one patient. Vasculitic lesions were not noted. One patient had diffuse lipoatrophy. Two patients developed calcinosis cutis while on treatment. All patients were put on oral corticosteroids (prednisolone 2 mg/kg/day) initially which were gradually tapered while monitoring clinical response. Early initiation of steroid therapy resulted in an excellent response. Two patients who did not show significant improvement even with prolonged steroid therapy were given oral weekly methotrexate (10 mg/m2/week). CONCLUSIONS: Most of the children with JDM showed good response to steroid therapy which needs to be continued for a prolonged period. Children who do not respond to this therapy may be given oral weekly methotrexate.

Adrenal Cortex Hormones↗
PubMed1997

Correlations between change in disease activity and changes in peripheral blood lymphocyte subsets in patients with juvenile dermatomyositis.

OBJECTIVE: To investigate whether changes in peripheral blood lymphocytes correlate with changes in disease activity in juvenile dermatomyositis (JDM). METHODS: Clinical changes in disease expression measured by a disease activity score were correlated with changes in percentages of peripheral blood lymphocyte subsets. RESULTS: Changes in the percentage of peripheral blood CD19 positive lymphocytes (B cells) correlated with changes in disease activity (Spearman rank coefficients = 0.47, p = 0.02). There were no significant correlations in disease activity with changes of T cell subsets or the T cell activation markers CD25 or DR. CONCLUSION: Change in the percentage of peripheral blood B cells correlates with change in disease activity in patients with JDM. This variable may be of use as an indicator of immunologic activity and response to therapy.

Adolescent↗
PubMed1997

The value of direct immunofluorescence as a diagnostic aid in dermatomyositis--a study of 35 cases.

Dermatomyositis (DM) in a inflammatory disorder of skeletal muscle and skin closely related to other connective tissue diseases; however, to date, no conclusive immunofluorescence (IMF) data are available for the disorder. The aim of this study was therefore to analyse retrospectively the clinical, histological and direct IMF findings for a group of 35 patients who had presented during an 8-year period. Clinically, 29 of the patients had the typical cutaneous features of DM, while 16 had evidence of myositis based on electromyographic or muscle biopsy findings; six developed malignant tumours during the study period, three of which proved fatal. Cutaneous histopathology findings were compatible with the diagnosis of DM in 18 cases, with evidence of a lupus band in six. Direct IMF showed a lupus band at the basement membrane zone in 19 (with IgM, IgG or C3), with colloid bodies in seven. Serologically, only nine were antinuclear antibody positive, one extractable nuclear antibody positive and all 35 anti-Jo-1 negative. Eight had other circulating autoantibodies, namely thyroid (three), gastric parietal cell (two), smooth muscle (two) and rheumatoid factor (one). Our findings suggest that direct IMF can be a useful adjunct in the investigation of patients with DM, and may prove helpful in diagnosis when both the histopathology and serology are inconclusive.

Adolescent↗
PubMed1997

In vivo proton magnetic resonance spectroscopy in polymyositis and dermatomyositis: a preliminary study.

The aim of the study is to determine whether biochemical abnormalities, such as lipid, creatine and choline metabolites, are observed in the more distal muscles of polymyositis (PM) and dermatomyositis (DM) patients. 12 patients suffering from chronic active PM/DM and 9 controls underwent a combined proton magnetic resonance (MR) imaging and proton MR spectroscopy study of the calf muscle. From a combination of T1-weighted MR images and corresponding fat suppressed images, fat infiltration into the muscle of both PM and DM patients was consistently observed. Muscular atrophy with a variable distribution was also noted in all patients. The biochemical profile of the localised proton MR spectra of soleus muscle showed reduction of both 'choline' to lipid and 'creatine' to lipid ratios in chronic PM and DM patients when compared with controls.

Adipose Tissue↗
PubMed1976

[Alterations of the arrector pili muscle in a case of dermatomyositis (author's transl)].

We have observed great alterations of the arrector pili muscle in a case of dermatomyositis. These alterations were as follows: diminution and then loss of the staining properties of the smooth muscle fibres, perinuclear vacuolization, great heterogeneity in the degeneration of fibres, elective involvement of the cyto-fibrillary system with integrity of the other constituents of the arrector muscle and the surrounding dermis. This smooth myositis is nonspecfic and similar to the one observed in other internal diseases (scleroderma, chronic alcoholism).

Cell Nucleus↗
PubMed1997

[Successful treatment of interstitial pneumonia with lipo-PGE1 and pentoxifylline in a patient with dermatomyositis].

Interstitial pneumonia complicated with dermatomyositis sometimes shows a resistance to high dose steroid therapy and a fatal course particularly in patients without showing the elevation of creatine kinase. We experienced a 48 year old woman who developed heliotrope rash, Gottron's sign, multiple cutaneous ulcers, and dyspnea on exertion. These symptoms were resistant to low dose steroid therapy. Serum levels of creatine kinase were normal. Anti-nuclear antibodies and anti-Jo-1 antibody were negative. High resolution CT scan of the chest showed areas of multiple air space consolidation and subpleural linear shadows. Lung biopsy performed under video-assist thoracosurgery revealed diffuse alveolitis with scattered lymphoid folicules and mild accumulation of macrophages in the alveolar spaces. There were no honey-combing. These features were compatible with "non-specific interstitial penumonia" proposed by Katzenstein, 1995. The patient was treated with 10 micrograms lipo-PGE1, PGE1 incorporated in lipid microspheres, and 300 mg pentoxifylline, which resulted in a dramatic improvement of both interstitial pneumonia and cutaneous ulcers. The present case suggested a novel strategy for the treatment of interstitial pneumonia.

Alprostadil↗
PubMed1997

Preliminary evidence for cyclosporin A as an alternative in the treatment of recalcitrant juvenile rheumatoid arthritis and juvenile dermatomyositis.

OBJECTIVE: To evaluate the safety and efficacy of cyclosporin A (CyA) with and without methotrexate (MTX) in refractory juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDMS). METHODS: Twenty-two patients (17 with JRA, 5 with JDMS) with refractory disease were studied retrospectively. All received CyA at a mean dose of 3.2 mg/kg/day over a mean period of 16 mo (range 6-42). All other medications except nonsteroidal antiinflammatory drugs, prednisone, and hydroxychloroquine were discontinued. In addition, 16/22 patients received concomitant MTX. RESULTS: Improvements in laboratory variables, joint counts, joint swelling, and morning stiffness were observed in most of the children with JRA. Muscle strength increased and muscle enzyme levels decreased in the patients with JDMS. CyA treatment permitted prednisone to be discontinued in 5/20 and reduced by greater than 50% in 10/20 patients. There was no evidence of hepatic or bone marrow toxicity or lymphoproliferative disease. Serum creatinine increased in 13/22 patients, but the actual values all remained within normal limits. CONCLUSION: CyA may be an effective agent in the treatment of refractory JRA and JDMS and concomitant MTX seems to be well tolerated. These preliminary data also suggest that combined CyA/MTX therapy may be associated with further improvement in clinical outcome.

Adolescent↗