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PubMed2002

Immunohistological analysis of CD59 and membrane attack complex of complement in muscle in juvenile dermatomyositis.

OBJECTIVE: To assess the presence of CD59 and the deposition of membrane attack complex (MAC) of complement system in skeletal muscle from patients with juvenile dermatomyositis (JDM), in comparison to patients with muscular dystrophies (MD) and children with normal muscle biopsies. METHODS: Muscle specimens obtained for diagnostic purposes from 10 patients with JDM, 6 with MD, and 7 children whose biopsies showed normal histology were analyzed. Immunohistological staining was performed using Mab against CD59 (YTH 53.1) and MAC (WU 7.2). RESULTS: Immunohistochemical staining for CD59 was weak and irregularly distributed on muscle fibers of all patients with JDM. Two of the 9 biopsies that allowed analysis of vessels showed negative CD59 staining in all vessels; in the remaining 7 patients, there was weak staining in a proportion of the vessels. In contrast, uniform and strong or moderate immunoreactivity was detected on the sarcolemma and in intramuscular endothelium in all normal and MD samples. Immunostaining for MAC was strong in JDM muscle vessels, and weak in normal or MD muscle. An inverse relation was found between MAC deposition and presence of CD59 in vessels in 6/9 JDM biopsies and in all normal and MD samples. CONCLUSION: Decreased CD59 expression in JDM muscle fibers and vessels may be associated with muscle lesions mediated by deposition of MAC of complement in JDM.

Biomarkers↗
PubMed2002

Dermatomyositis: diagnosis, evaluation and management.

Dermatomyositis (DM) is a condition primarily of the skin and muscles, but other systemic features may occur. DM in adults is associated with malignancy and thus a careful evaluation of each patient should be part of their initial and follow-up assessments. Patients should also be evaluated for the presence of esophageal, pulmonary and cardiac disease. Corticosteroids, immunosuppressives, biologic agents and/or immune globulin are effective therapies for the myopathy of DM, whereas the skin disease is best managed with sunprotection, topical corticosteroids, antimalarials, methotrexate and/or immune globulin. The prognosis is good except for patients with malignancy, those with severe weakness and those with cardiopulmonary dysfunction. However, many patients are left with residual weakness even after control of the disease.

Dermatomyositis↗
PubMed2002

Usefulness of cyclosporine A on rapidly progressive interstitial pneumonia in dermatomyositis.

BACKGROUND: Interstitial pneumonia in polymyositis and dermatomyositis (PM/DM) is recognized to be a major complication of PM/DM. Rapidly progressive interstitial pneumonia (RPIP) in DM is frequently refractory to steroids and is the prognosis-determining factor in DM. Recently cyclosporine A (CyA) has emerged as an available treatment for interstitial pneumonia in PM/DM, but its efficacy on RPIP is unclear. AIM OF THE WORK: To evaluate the usefulness of CyA in the treatment of RPIP in DM. METHODS: We reviewed the medical charts of 10 cases with RPIP in DM to whom CyA was administered. RESULTS AND CONCLUSIONS: Combined administration of oral CyA and steroids with/without immuno-suppressants was likely to be beneficial in 4 of 10 cases that were refractory to the conventional preceding therapy. The values of PaO2/FIO2 when CyA were initiated were suggested to be a prognosis-determining factor for the outcome of the disease, showing that initiation of CyA should be considered in the early stage of RPIP in DM. The dosage of steroids was tapered in 3 out of 4 CyA-responsive cases without re-exacerbation. Therefore administration of CyA might be useful in lowering the dosage of steroids.

Adult↗
PubMed2002

[Dermatomyositis and polymyositis: the attempt to differentiate and literature review].

Dermatomyositis (DM) and polymyositis (PM) are diseases displaying the symptoms of the idiopathic muscle inflammation (IZM). The skin lesions are visible most often 3 months to 3 years prior to appearance of the muscle inflammatory process. A frequent coexistence of DM with internal organ neoplasms obliges doctors to diagnose their patients quickly. The knowledge of skin lesions allows to begin this diagnosis earlier and to initiate proper therapy. Based on the latest medical literature, the authors present: the actual classification of the idiopathic muscle inflammation, the attempt to differentiate between DM and PM regarding prognostic factors and therapy possibilities based on conducted world trials.

Anti-Inflammatory Agents↗
PubMed2002

[A case of cutaneous tuberculosis under steroid & immunosuppressant therapy for dermatomyositis].

A 44-year-old man had been admitted for high fever and leg edema on November 1998. By the laboratory data, electromyography, and muscle biopsy, he was diagnosed as having polymyositis. Steroid (prednisolone 60 mg/day) and immunosuppressant (methotrexate 7.5 mg/week) therapy was administered and the symptoms were improved, so he had been followed up in out-patient clinic. After half a year, high fever and leg edema relapsed and erythema on the bilateral forearms appeared, so he was admitted again on January 2000. The symptoms, skin involvement and laboratory data suggested the disease of dermatomyositis. Steroid pulse therapy was administered again. But the symptoms were not improved. Skin biopsy was performed but it showed only inflammatory changes. Several antibiotics and cyclospolyn A were undertaken but they were not effective. On February 12th he passed away because of respiratory failure. The autopsy was undertaken and it revealed tuberculosis in the skin, subcutaneous tissues and muscles, however, pulmonary tuberculosis was not found. The patient with collagen disease is considered to be "compromised host", especially during corticosteroid therapy. We must keep in mind potential incidence of tuberculosis and do careful clinical observation for early diagnosis and be prepared for antituberculous chemotherapy. Chemoprophylaxis for tuberculosis seems to be desirable for higher risk patients.

Adult↗
PubMed2002

[Analysis of human cytomegalovirus infection of an autopsy case of dermatomyositis with rapidly progressive interstitial pneumonia: the possible correlation between the viral genome and its related protein on the pathogenesis].

We describe a case of a 61-year-old woman with amyopathic dermatomyositis (ADM), who developed rapidly progressive interstitial pneumonia and died of respiratory failure. An autopsy revealed interstitial pneumonia with diffuse alveolar damage, associated with infiltration of T cells, mostly positive for CD 8. The alveolar lining epithelial cells manifested the remarkable expression of immediate early/early antigen of human cytomegalovirus (HCMV). Moreover, the extract of the lung was transmittable of HCMV infection to cultured human embryo-fibroblasts in vitro. On the other hand, in the semi-quantitative analysis of HCMV genome, using laser-assisted microdissection, followed by PCR method, the genomic DNA in the alveolar lining epithelial cells was little detected in this case, although it was remarkable in the case of immunodeficiency with cytomegalovirus pneumonia. This case may be important to know the role of the immune response of host to HCMV infection on the development of rapidly progressive interstitial pneumonia.

Cytomegalovirus Infections↗
PubMed2002

[A case of nonspecific interstitial pneumonia associated with amyopathic dermatomyositis efficiently treated with a combination of cyclosporin A and prednisolone].

A 36-year-old man was admitted to our hospital complaining of cough, dyspnea on exertion, skin eruptions, and joint pain. Characteristic skin lesions such as erythema around the nails, telangiectasis, and edema of the eyelids were observed in this patient. He had never complained of muscle symptoms, and his laboratory examinations showed no elevation of either CPK or aldolase. From several lines of evidence including the skin biopsy findings, amyopathic dermatomyositis was diagnosed. Chest X-ray films showed subpleural funicular opacities and consolidation in both lower lung fields. Lung biopsy specimens taken under video-assisted thoracoscopic surgery revealed nonspecific interstitial pneumonia, group II. Oral prednisolone treatment was initiated at 60 mg daily together with oral cyclosporin A (100-150 mg daily). The minimum serum concentration of cyclosporin A was maintained between 100 and 200 ng/ml. Respiratory symptoms gradually improved, and the oral prednisolone dose was tapered off. Pulmonary function and chest CT findings showed marked improvement.

Adult↗
PubMed2002

[A case of amyopathic dermatomyositis with systemic sclerosis sine scleroderma presenting abnormal vermiculation].

A 24-year-old Japanese woman was admitted to our hospital in January 2000 with daily diarrhea, abdominal distention, and abdominal pain. Raynaud's phenomenon with erythroderma desquamativum eruptions had been found in 1992. In 1999, small intestinal transillumination showed dilation of the second and third portion of the duodenum. She was diagnosed as amyopathic dermatomyositis because of Gottron's sign despite not having symptoms of myositis, normal serum CPK levels and no histological abnormality by muscle biopsy. In addition, the patient was diagnosed as systemic sclerosis sine scleroderma because Raynaud's phenomenon, leukoma and gastrointestinal dilation were present. Also, antinuclear antibody and anti centromere antibody were positive and anti Scl-70 antibody was dull-positive, despite the absence of scleroderma, extreme edema, and bone resorption. Careful, long term observation will be required because of the splanchnopathy in youth and the complications of systemic sclerosis.

Adult↗
PubMed2002

[Dermatomyositis and polymyositis].

Dermatomyositis (DM) and polymyositis (PM) are the two main forms of idiopathic inflammatory myopathies. They have in common a proximal muscle weakness, but skin manifestations, juvenile forms and increased incidence of malignancies are clinical characteristics of DM. The follow up of creatine-kinases is the best biological test in spite of their possible normality. The significance of antibodies titers is uncertain, except the association Jo-1 interstitial and lung disease indicating a poor prognosis. The association with HLA haplotypes expresses a genetic predisposition of a dysimmunity to develop DM or PM. Pathological changes are well known with a humoral immune effector mechanism in DM, and a muscle fibre aggression by CD8 + T cells in PM. Non inflammatory forms of DM and PM and rhabdomyolytic forms of PM are not very rare, they are recognized by the HLA class 1 immunoreactivity. Pathophysiological processes involve muscle fibers, inflammatory cells and endothelial cells of capillaries, with a complex intervention of cytokines, adhesion molecules, MHC classe 1, membrane attack complex, anti endothelial cells antibodies, perforin secretion and sometimes apoptotic Fas-mediated mechanisms. Despite these recent advances, causal antigens and activator processes of endothelial cell lysis and autoinvasive cytotoxicity of muscle fibers remain to be identified.

Biomarkers↗
PubMed2002

[Overlap syndrome of systemic lupus erythematosus and dermatomyositis presented a large demyelinating subcortical lesion mimicking brain tumor and high level of CSF antineuronal and serum anti-ribosomal P antibodies].

We reported a 50-year-old man with an overlap syndrome of dermatomyositis and SLE, whose magnetic resonance image of the brain showed a rapidly increasing large tumor-like focal lesion unequally enhanced by Gd-DTPA in the left frontal lobe. Its pathological finding by the brain biopsy was fibrinoid necrosis, inflammatory cell aggregation around blood vessels and many myelin-laden macrophages with central necrosis. Although many cases of blood vessel injury are reported in CNS lupus, in this case the brain lesion partly took reversible course and neural symptoms such as paresis were slight and the lesion well responded to steroid. Moreover we considered that the measurement of serum anti-ribosomal P and CSF antineuronal antibodies are useful to diagnose cases as CNS lupus.

Autoantibodies↗
PubMed2001

[Juvenile dermatomyositis].

Basic aim of this paper is presentation of probable epidemiological characteristics of juvenile dermatomyositis (JDM) in children of Croatia, due to the fact that severity of condition in majority of patients require treatment in our Department of Pediatrics, KBC Zagreb. Our intention is to present guidelines of current diagnostic approach from recent literature, with special accent on therapy. JDM was previously considered as infaust disease, while current therapy has favourable prognosis for complete resolution, as the only one mesenchymopathy with such prognosis. In period between 1988 and 1999 we treated 18 patients with JDM in our Department of Pediatrics. Thirteen cases were male and 5 female (2.6:1). Thirteen patients had clinical course of isolated JDM (72%), while five had JDM inside overlap syndrome (with other mesenchymopathies) (28%). Average age at time of diagnosis for whole group was 10 years (6-14), for girls 10.6 years (6-14) and boys 8.4 years (7-10). At the present time 5 patients (28%) are considered as cured, 7 patients are in remission with low dosis of steroids (39%) and one girl had relaps of JDM after therapy stopage. Three children die (16%), one girl due to respiratory failure and two girls with pulmonary embolia. Two girls have some signs of JDM with dominant clinical signs of sistemic sclerodermia and one girl has signs of generalized morphea. One boy has dominant muscular calcinosis with contractures of large joints, despite treatment and normal laboratory findings. JDM was not discussed in rheumatic literature in Croatia for more then 15 years.

Adolescent↗
PubMed2001

[Dermatomyositis and malignant disease--case report].

The case of patients with dermatomyositis and malignant disease has been described. The diagnosis of malignant disease was made after death of the patient, when the malignant undifferentiated cells were found in the ascites. We think that dermatomyosistis was caused by malignant disease. The similar cases of disease were described and published in the literature.

Aged↗
PubMed2002

[Clinical and pathological investigation of polymyositis and dermatomyositis: report of 83 cases].

OBJECTIVE: To study the clinical and pathological characteristics and treatment of polymyositis (PM) and dermatomyositis (DM). METHODS: Eighty-three DM/PM cases were reviewed and analyzed. RESULTS: The main clinical presentations of PM/DM included amyosthenia, muscular tenderness, elevation of serum enzymes, accompanied by abnormal electromyography and muscular pathology. A total efficacy rate of 86.7% was achieved after treatment with corticosteroid and immuno- depressants. CONCLUSION: The different clinical and pathological presentations of various types of PM/DM suggest different pathogenesis between DM and PM. Nerve damage is a part of the systemic damages due to the disease, the prognosis of which is related to its classification and then timing of the treatment. Early effective treatment can improve the prognosis.

Adolescent↗
PubMed2002

[Polymyositis/scleroderma autoantibody-positive scleroderma with dermatomyositis (scleromyositis)].

Polymiositis/scleroderma autoantibody positive scleroderma with dermatomyositis connected to interstitial lung fibrosis is reported. The recently observed overlap syndrome is characterised by the presence of special autoantibodies, HLA-type association and benign course. A new skin symptom (mechanic's hands) is described which may indicate the syndrome and one of its important internal manifestations, the interstitial lung disease.

Autoantibodies↗
PubMed2001

[Expression of apoptosis of the skin lesion and muscle from patients with dermatomyositis].

OBJECTIVE: This study inquired into the relationship between the influence of corticosteroid and the expression of apoptosis, Fas and Bcl-2 in muscle from patients with dermatomyositis (DM). METHODS: Corticosteroid was given to group A (10 DM cases) but not given to group B (12 DM cases). RESULTS: Apoptotic cells were detected in situ by terminal deoxynucleotidyl transferase-mediated-dUTP nick end labeling (TUNEL). Fas and Bcl-2 expressions was determined with the use of immunohistochemical staining in DM. The results showed that more expression of apoptosis in keratinocytes, muscle cells and lymphocytes were observed in group A. The apoptotic index (AI) of muscle cells and that of lymphocytes were more marked in group A than in group B (P < 0.05). Less apoptosis cells were observed in two weeks of treatment, and no correlation was found between apoptosis and the lesion of skin and muscle. CONCLUSION: The authors suggest that corticosteroid may play a role in inducing the apoptosis of keratinocytes, muscle cells and lymphocytes, which may be one of the therapeutic mechanisms of corticosteroid. The length of administering corticosteroid to the patients seems related to apoptosis.

Adult↗
PubMed2002

[Simultaneous development of factor V inhibitor and autoimmune thrombocytopenia in a patient with dermatomyositis].

It has been previously demonstrated that aging, a history of malignancy or surgery, and exposure to bovine thrombin may be related to the presence of factor V inhibitor. However, dermatomyositis (DM) and autoimmune thrombocytopenic purpura (ATP) have rarely been associated with factor V inhibitor. Here we report a patient with factor V inhibitor accompanied by DM and ATP. In January 2000, a 77-year-old woman with DM was hospitalized because of susceptibility to bleeding. At the onset of DM, she had suffered from gastric leiomyosarcoma, and had undergone gastrectomy and splenectomy without the use of bovine thrombin. Thereafter, she had been treated with prednisolone until October 1999. On admission, prolongation of both APTT and PT was seen. Her factor V activity had fallen to 6%, and factor V inhibitor was positive at 8.9 Bethesda units. Moreover her platelet count had dropped to 1.0 x 10(9)/l. A bone marrow aspirate showed a cellular marrow with megakaryocytic hyperplasia, and the patient's PA-IgG level was elevated at 389 ng/10(7) cells. These findings suggested that ATP was also present. Additionally, the patient showed a positive result for Coombs test and consumption of complement. After a further course of steroid therapy, the patient's condition was markedly improved. This is a very rare case that showed factor V inhibitor and ATP simultaneously. Furthermore, the patient's clinical course suggests the relationship between the presence of factor V inhibitor and the reactivation of her collagen disease activity.

Aged↗
PubMed2002

[Intravenous cyclophosphamide pulse therapy for refractory juvenile dermatomyositis].

We described three children with juvenile dermatomyositis (JDM) refractory to the conventional therapy. They were successfully treated with intravenous cyclophosphamide (IVCY) pulses, and two of them were administered plasma exchange (PE) before IVCY. Case 1. A 17-year-old girl with JDM was previously treated for 2 years with the combination of prednisolone, intravenous gamma-globulin, methotrexate, and azathioprine. However, muscle weakness gradually progressed. She failed to hold her sitting position and to rise her arms, but both serum CK and aldolase were stable. After the episode of aspiration pneumonia the follow-up muscle biopsy was performed, which revealed muscle degeneration and massive mononuclear cell infiltration in perivascular area. The erythrocyte sedimentation rate (ESR) and fibrin degradation product E (FDP-E) levels were gradually increased. Because the active inflammation of muscle and muscle vasculature was suspected, the PE and IVCY combination therapy was administered. During the 6 courses of the therapy, muscle weakness was markedly improved so that she could hold herself at the sitting position and could have meals by herself. Case 2. A 5-year-old boy with JDM was treated for 8 months with prednisolone p.o., but his muscle strength became worse. The muscle enzyme levels, such as serum CK and aldolase, were not reflecting his status of the disease, but FDP-E levels were increased. Muscle MRI and biopsy revealed the inflammatory changes of perivascular area of muscle. The PE and IVCY combination therapy was effective, and he became able to walk and run by himself. Case 3. A 14-year-old boy was diagnosed as having JDM when he was 10 years of age, and treated with prednisolone p.o., and subsequently with intravenous methylprednisolone pulses and azathioprine. Three years later the flares were observed accompanied with the elevations of serum CK and FDP-E. The administration of IVCY improved muscle strength as well as serum muscle enzyme and FDP-E levels. These findings indicated that the clinical manifestations of JDM should be closely monitored, that the serum levels of muscle enzymes including CK and aldolase were sometimes not indicative for the flares of JDM, and that muscle MRI and re-biopsy examination were needed for the children with progressive muscle weakness. In addition, determination of ESR and FDP-E was not specific but helpful to detect flares of the disease in some cases.

Adolescent↗
PubMed2003

Aerobic exercise capacity in patients with juvenile dermatomyositis.

OBJECTIVE: To examine the feasibility of maximum exercise testing in patients with juvenile dermatomyositis (JDM), characterize the maximum oxygen consumption (VO2peak) of these patients, and determine if exercise time could be used as an surrogate index for VO2peak. METHODS: Fifteen patients diagnosed with JDM (age 5-14) performed a graded, maximum exercise test using a motor driven treadmill and metabolic cart to volitional exhaustion conforming to the Bruce protocol. RESULTS: All patients were able to perform the exercise test. Ten of the 15 patients performed a maximal effort (heart rate > 180 beats/min or respiratory exchange ratio > 1.0). The patients who had a maximal exercise performance, the mean absolute VO2peak, relative VO2peak (related to body mass), and exercise time were respectively -1.82 (+/- 1.5), -2.83 (+/- 1.9), and -3.65 (+/- 1.9) standard deviations lower compared to age and sex matched reference values (p < 0.05). Z scores for exercise time were significantly lower compared to Z scores for absolute and relative VO2peak, an indication of reduced muscular economy. When exercise time was converted to VO2peak using the equation: VO2peak = 0.1583 (exercise time) + 0.0828, the VO2peak values were not significantly different from the measured VO2peak values. CONCLUSION: Aerobic exercise testing on a treadmill was possible in patients with JDM and revealed an impairment in their maximal aerobic exercise capacity. Exercise time can be used as an indicator of VO2peak, when converted to VO2peak using a regression equation. VO2peak, measured or estimated, has the potential to be a good indicator of muscle function in patients with JDM.

Adolescent↗