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PubMed1999

Dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum.

We describe two cases of dermatomyositis (DM), which subsequently developed into rapidly progressive fatal interstitial pneumonitis and pneumomediastinum during steroid therapy. Both cases showed the classical cutaneous manifestations of DM, but the muscular symptoms were absent or mild. Both rapidly progressive interstitial pneumonitis and pneumomediastinum can occur in DM showing less inflammatory changes in the muscles. Patients with this form should be treated with extreme caution.

Adult↗
PubMed2006

Polymyositis-dermatomyositis and infections.

In genetically predisposed individuals, viruses, bacteria, or parasitic infectious agents are suspected to induce autoimmunity and/or to exacerbate the disease once the self-tolerance is broken. Although direct evidence for this association is still lacking, numerous data from animal models as well as from humans support the hypothesis of a direct contribution of pathogens to the induction of several autoimmune diseases. This review focused on the possible role of infectious agents as triggers of autoimmunity in polymyositis (PM) and dermatomyositis (DM). Epidemiological studies, clinical and experimental findings that support the hypothesis of infection-induced PM and DM are summarized and discussed. In addition, immune response abnormalities and immunosuppressive medications may be responsible for the high percentage of infectious complications in PM and DM patients. In this review, the increased risk of developing infections in these patients is also underlined and published data are reported.

Animals↗
PubMed2006

Clinical assessment in juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a multisystem inflammatory disease of unknown etiology that affects primarily the skin and muscles. Although the prognosis of JDM has improved considerably in the last three decades, a number of patients may develop irreversible damage due to the disease activity or its treatment. This damage may cause permanent disability and affect the quality of life of patients and their families. In the clinical management of patients with JDM, there is, therefore, the need of monitoring the level of disease activity, the accrual of organ damage, and the impact of the illness on patients' daily living. A reliable assessment of these different aspects of disease requires the availability of well-designed and standardized clinical tools. In the recent years, there has been increasing collaborative effort to devise new assessment measures and these measures have been included into disease activity and damage core sets of outcome variables that have been developed through international consensus. In addition, preliminary definitions of clinical improvement for patients with JDM and other idiopathic inflammatory myopathies have been created. In this review, the latest advances in the development of standardized instruments for the clinical assessment of JDM patients are illustrated and the recent international efforts that have led to the development of core sets of outcome measures and to preliminary definitions of improvement for JDM clinical trials are summarized.

Dermatomyositis↗
PubMed2006

National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis.

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.

Adolescent↗
PubMedDate not supplied

Increased Fas and Bcl-2 expression on peripheral blood T and B lymphocytes from juvenile-onset systemic lupus erythematosus, but not from juvenile rheumatoid arthritis and juvenile dermatomyositis.

Defective regulation of apoptosis may play a role in the development of autoimmune diseases. Fas and Bcl-2 proteins are involved in the control of apoptosis. The aims of this study were to determine the expression of Fas antigen and Bcl-2 protein on peripheral blood T and B lymphocytes from patients with juvenile-onset systemic lupus erythematosus (JSLE), juvenile rheumatoid arthritis (JRA) and juvenile dermatomyositis (JDM). Thirty-eight patients with JSLE, 19 patients with JRA, 10 patients with JDM and 25 healthy controls entered the study. Freshly isolated peripheral blood mononuclear cells (PBMC) were stained for lymphocyte markers CD3, CD4, CD8, CD19 and for Fas and Bcl-2 molecules. Expressions were measured by three-color flow cytometry. Statistical analysis was performed using Kruskal-Wallis test. Percentages of freshly isolated T lymphocytes positively stained for Fas protein from JSLE patients were significantly increased compared to healthy controls, patients with JRA and patients with JDM. Percentages of B lymphocytes positive for Fas from JSLE patients were higher than healthy controls and JRA patients. In addition, Fas expression on T cells from patients with JRA was increased compared to JDM patients. Otherwise, Fas expression on T and B cells from JRA and JDM patients were similar to healthy controls. MFI of Bcl-2 positive T lymphocytes from JSLE patients were significantly increased compared to healthy controls and JRA patients. MFI of Bcl-2 protein on B lymphocytes from JSLE patients was similar to healthy controls and patients with JRA and JDM. Bcl-2 expression did not differ between JRA and JDM patients and healthy controls. In conclusion, increased expression of Fas and Bcl-2 proteins observed in circulating T and B lymphocytes from patients with JSLE, but not from patients with JRA and JDM, suggests that abnormalities of apoptosis may be related to the pathogenesis of JSLE and probably are not a result of chronic inflammation.

Adolescent↗
PubMed2002

Reduced oxidative phosphorylation and proton efflux suggest reduced capillary blood supply in skeletal muscle of patients with dermatomyositis and polymyositis: a quantitative 31P-magnetic resonance spectroscopy and MRI study.

Quantitative MRI and phosphorus magnetic resonance spectroscopy ((31)P-MRS) were used to investigate skeletal muscle metabolism in vivo in patients with dermatomyositis (DM) and polymyositis (PM) in order to evaluate the role of mitochondrial abnormalities in the pathogenesis and clinical expression of these conditions. Nine patients with DM (mean age +/- SD, 57 +/- 14 years) and five with PM (42 +/- 12 years) and with age at disease onset 53 +/- 16 and 38 +/- 12 years, respectively, were included in the study together with 18 age-matched controls. Post-exercise (31)P-MRS indices of muscle oxidative metabolism were all impaired in DM and PM. In both groups of patients, the phosphocreatine and adenosine diphosphate recovery half-times were almost twice as long as in controls (P < 0.05 for each variable) and the maximum rate of mitochondrial ATP production was half that found in normal subjects (P < 0.001). The rate of proton efflux from muscle fibres was significantly reduced in DM (P < 0.001) and PM (P = 0.02). The impairment of (31)P-MRS recovery indices in DM and PM patients was similar to that found in a group of 10 patients with a primary mitochondrial disorder that showed a normal proton efflux rate. There was no correlation between the MRS-detectable abnormalities and the degree of inflammation or fatty infiltration of the muscle, as measured by MRI. The in vivo findings in DM and PM patients indicate impaired muscle aerobic function, which, considering the reduced proton efflux, is likely to be secondary to an impaired blood supply. Our results suggest that the abnormal mitochondria seen in some muscle biopsies are unlikely to be the primary cause of the oxidative insufficiency in these patients.

Adenosine Diphosphate↗
PubMed2001

Cathepsins are upregulated by IFN-gamma/STAT1 in human muscle culture: a possible active factor in dermatomyositis.

The aim of this work was to study which genes upregulated by the IFN-gamma/STAT1 system in human muscle might be involved in the process of muscle fiber atrophy in dermatomyositis (DM). These proteins included proteases (cathepsins B and L, calpain), proteins implicated in apoptosis and cell cycle (Bcl-x(l), Fas, p21), structural proteins (beta-actin, utrophin, desmin), and other proteins whose expression is known to be modified by IFN-gamma (neural cell adhesion molecule (N-CAM), major histocompatibility complex-I (MHC-I)). We performed immunocytochemistry, Western blot, and semiquantitative reverse transcriptase-polymerase chain reaction using human muscle cultures. We found upregulation of cathepsins B and L, bcl-x(l) and p21 while N-CAM, calpain, utrophin, desmin, beta-actin and Fas remained at basal levels. Immunohistochemistry on frozen sections from biopsies of patients with different muscle diseases showed upregulation of cathepsin L and calpain in perifascicular muscle fibers in DM. In view of these results, the increased expression of cathepsins L and B after IFN-gamma stimulation in muscle cultures and its inhibition using fludarabine, a STAT1 blocker, further support our previous studies and suggest that the increased expression of cathepsins detected in perifascicular muscle fibers in DM is mediated by IFN-gamma/STAT1 and contributes to their atrophy.

Adult↗
PubMed1991

Antinuclear antibodies in first-degree relatives of patients with polymyositis-dermatomyositis: analysis of the relationship with HLA haplotypes.

Antinuclear antibodies (ANA), as detected by indirect immunofluorescence on HEp-2 cells, have been investigated in five spouses and 41 first-degree relatives of nine probands with polymyositis-dermatomyositis (PM-DM) and in 41 sex- and age-matched controls. ANA were detected in 12 out of the 41 first-degree relatives and in two controls (chi 2 = 6.97; P less than 0.01). HLA typing was done in four out of the nine families; in two of them only, ANA segregated with a haplotype. ANA positivity was not correlated either to sex or to age or to household contact. Our results show that ANA occur in a significant percentage of first-degree relatives of patients with PM-DM. The finding seems to be genetically conditioned.

Adolescent↗
PubMed1995

The incidence of juvenile dermatomyositis: results from a nation-wide study.

There have been no previous attempts to estimate the incidence of juvenile dermatomyositis (JDM) in the UK and Ireland. Consultant paediatricians from these countries were asked to report all cases of JDM which were diagnosed in 1992 and 1993 to the British Paediatric Surveillance Unit. One-hundred-and-twenty-one reports were received, of which 48 were confirmed cases of JDM and three were confirmed cases of polymyositis. The estimated incidence of JDM was 1.9 per million children aged under 16 yr (95% confidence interval 1.4-2.6). The median age at onset was 6.8 yr and the median delay in diagnosis was 4 months. Girls were affected much more often than boys (ratio 5.0:1). In the cases with an onset in 1992 there appeared to be a cluster of cases with an onset in April and May.

Adolescent↗
PubMed1996

T-lymphocyte immunophenotyping in polymyositis and dermatomyositis.

The objective was to elucidate the immunological abnormalities underlying polymyositis (PM) and dermatomyositis (DM). The phenotype of peripheral blood mononuclear cell (PBMNC) subsets and cell surface expression of activation (CD25 and HLA-DR) and adhesion (LFA-1) molecules was studied in 12 patients with PM and in 10 patients with DM. PBMNC subsets and expression of T-cell activation molecules were evaluated by cytofluorography. Double immunofluorescence and indirect immunoperoxidase techniques were applied to muscle biopsies to define T-cell phenotype and LFA-1/ICAM-1 expression. In PM, the absolute number of circulating cytotoxic (CD8+CD28+) T cells was selectively reduced. T cells showed increased expression of activation molecules, CD25 and HLA-DR, and increased adhesion capacity as the absolute numbers of CD3+CD25+, CD8+HLA-DR+, CD3+LFA-1+('bright') and CD8+ CD8+LFA-1+('bright') cells were higher than in healthy donors and DM patients. In PM muscle biopsies, T cells were mainly CD3+CD8+ and LFA-1+; additionally, endothelial cells and myofibres surrounded by T cells showed positive staining for ICAM-1. In DM, there was a general lymphopenia that led to a decreased absolute number of all T-lymphocyte subsets. It is proposed that in PM, in contrast to DM, LFA-1/ICAM-1 interactions enable activated CD8+ T cells to migrate selectively into the inflamed muscle and to adhere to myofibres, leading to tissue injury.

Adult↗
PubMed1997

Severe juvenile dermatomyositis complicated by pancreatitis.

We report two boys with juvenile dermatomyositis (JDM) complicated by pancreatitis. One also had hepatitis and probably mild bowel vasculitis, while the other had catastrophic bowel vasculitis with multiple perforations. Both were on corticosteroids, but had features of active vasculitis. The former improved with high-dose i.v. pulsed methylprednisolone, while the latter improved only after immunosuppression with i.v. methylprednisolone, cyclophosphamide and plasmapheresis. Although bowel vasculitis is a known complication of severe JDM, pancreatitis and hepatitis are extremely rare. We have found in a literature search only three other reports of pancreatitis complicating JDM. We wish to alert physicians that pancreatitis may develop in JDM. It should be considered as a differential diagnosis in the child with active disease who develops abdominal pain. Control of vasculitis with adequate immunosuppression, as well as general supportive measures, may be valuable in the treatment of pancreatitis in JDM.

Adolescent↗
PubMed1998

Familial dermatomyositis.

A case of familial dermatomyositis (DM) is reported in a 27-yr-old mother and her daughter aged 2 yr and 8 months. Familial DM is a rare occurrence and has never been reported in a child of < 4 yr old, although isolated cases of DM have been found in infants as young as 1 yr. The mother responded readily to corticosteroids; however, the child proved resistant to steroids and was eventually successfully managed on a combination of methotrexate and prednisolone. Another daughter presented with the dermatological stigmata of DM, but with no evidence of muscle involvement.

Adult↗
PubMed1998

Anti-Jo-1 antibodies in polymyositis or dermatomyositis: evaluation by ELISA using recombinant fusion protein Jo-1 as antigen.

We evaluated an enzyme-linked immunosorbent assay (ELISA) for detecting anti-Jo-1 antibodies in patients with polymyositis (PM) or dermatomyositis (DM) by use of the recombinant fusion protein Jo-1. Sera from 64 patients with PM or DM, from 80 patients with other connective tissue diseases, and from 64 healthy subjects matched for age, sex and race, were studied by the ELISA and by the double immunodiffusion (DID) method. Eight patients with myositis (six PM, one DM and one DM with malignancy) with positive anti-Jo-1 by DID also showed positive results by the ELISA method, whereas five patients with positive anti-Jo-1 by this ELISA showed negative results on DID. One of the five had non-specific results. The incidence of positive results for anti-Jo-1 with the ELISA (18.8%) was greater than that for DID (12.5%), but the difference was not statistically significant. All patients with positive results for anti-Jo-1 by DID were also positive by the ELISA. The ELISA system with the recombinant Jo-1 antigen was useful in the detection of anti-Jo-1 antibodies in patients with PM/DM.

Adolescent↗
PubMed1999

Safety of a home exercise programme in patients with polymyositis and dermatomyositis: a pilot study.

OBJECTIVES: To investigate whether a home exercise programme could safely be performed by patients with stable, inactive polymyositis (PM) and dermatomyositis (DM), regarding disease activity, muscle function, health status and pain. METHODS: Ten patients with reduced muscle function completed the study. A home exercise programme including exercises for strength in the upper and lower limbs, neck and trunk, for mobility in the upper limbs and moderate stretching was developed. The patients exercised for 15 min and took a 15 min walk 5 days a week during a 12 week period. Assessments included clinical evaluation of disease activity, serum creatinine phosphokinase (CPK) levels, magnetic resonance imaging (MRI) of the quadriceps, repeated muscle biopsy of the vastus lateralis, a muscle function index (FI), a walking test and a health status instrument (the SF 36) performed at the start of the study and after 12 weeks. RESULTS: After 12 weeks of exercise, there were no signs of increased disease activity as assessed clinically, by CPK values, MRI or muscle biopsy findings. On an individual basis, all patients improved regarding muscle function according to the FI, in six cases the improvement reached statistical significance (P < 0.05). A significant improvement regarding muscle function in the upper and lower limbs, walking distance and general health status was achieved. CONCLUSIONS: Our results indicate that this home exercise programme can be safely employed in patients with stable, inactive PM and DM, with beneficial effects on muscle function.

Adult↗
PubMed2001

Pulmonary involvement in juvenile dermatomyositis: a two-year longitudinal study.

OBJECTIVE: To investigate the prevalence and features of asymptomatic pulmonary involvement in juvenile dermatomyositis (JDM). METHODS: Twelve JDM patients underwent pulmonary function tests at baseline, 12 and 24 months. Disease activity, duration, serum lactate dehydrogenase (LDH) values and antinuclear antibody (ANA) titres were also evaluated. RESULTS: Five patients showed lung impairment at baseline and four at 12 and 24 months. Forced expiratory volume in 1s, forced vital capacity (FVC), carbon monoxide diffusing capacity (DLCO) and alveolar volume were the most frequently altered variables, indicating a restrictive pattern and impairment of diffusion. The prevalence and features of pulmonary alterations did not change during follow-up. FVC values were significantly lower in active JDM patients and were inversely related to LDH. DLCO values were significantly lower in ANA-positive patients. About half of the patients of this small case series of JDM had asymptomatic lung disease. CONCLUSIONS: We suggest that lung function should be evaluated at disease onset and regularly during follow-up, as pulmonary function tests can detect otherwise unpredictable pulmonary involvement.

Adolescent↗
PubMed2003

Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A.

OBJECTIVES: Interstitial lung disease (ILD) is a rare complication of juvenile dermatomyositis (JDM). The aim of this study was to clarify the clinical features of JDM-associated ILD and to evaluate the efficacy of cyclosporin A (CSA). METHODS: We reviewed clinical records of 10 cases of JDM that were admitted to Hokkaido University Hospital between April 1990 and March 2001. RESULTS: Five cases were complicated with ILD, three with interstitial pneumonia and two with bronchiolitis obliterans organizing pneumonia. ILD was associated with active JDM and progressed despite corticosteroid therapy. Testing for anti-Jo-1 antibody was negative in all cases. Respiratory symptoms were initially noticed in only one case. In the other cases, ILD was first detected by routine examination of chest X-ray. All the cases received CSA (3-5 mg/kg/day) in combination with prednisolone. One patient died of respiratory failure, but the others responded well to treatment with CSA. CONCLUSION: ILD should be evaluated carefully in all cases of JDM regardless of respiratory symptoms. CSA is a choice for steroid-resistant cases of JDM-associated ILD.

Adolescent↗
PubMed2003

Urinary levels of creatine and other metabolites in the assessment of polymyositis and dermatomyositis.

BACKGROUND: A simple and reliable method is needed to assess disease activity and monitor the efficacy of therapy in polymyositis (PM) and dermatomyositis (DM). This study used in vitro proton ((1)H) magnetic resonance spectroscopy (MRS) to explore whether excretion of urinary metabolites can be used as a reliable marker of disease in PM and DM patients. METHODS: Urine samples were obtained from PM/DM patients (n=34), healthy controls (50) and subjects with known muscle-wasting conditions including adult-onset muscular dystrophy (8), stroke patients (10), rheumatoid arthritis (RA) patients on steroids (13) and not on steroids (16) and patients with alcoholic myopathy (12). Levels of urinary metabolites were then correlated with creatine kinase (CK) activities and quadriceps muscle strength. RESULTS: Creatine was detected in the urine in 26 of 35 patients with PM/DM, four of 60 cases with other medical disorders (including one with adult-onset dystrophy, one with a stroke and two with RA who were not on steroids) and 10 of 50 healthy controls. The urinary creatine/creatinine ratio exceeded 0.4 in 20 patients with PM/DM but no patients with other medical disorders and no healthy controls. These differences were highly significant (P<0.001) by Kruskal-Wallis test (comparing all groups) and by Mann-Whitney U-tests (comparing individual groups with PM/DM cases). Citrate, glycine, choline-containing compounds and taurine levels were significantly increased in PM/DM when compared with controls. There were positive correlations between CK activities and choline-containing compounds (r=0.78, P=0.0006) and also between CK activities and betaine (r=0.57, P=0.026). CONCLUSIONS: This study shows significant differences in the urinary levels of creatine, choline-containing metabolites, betaine and citrate in PM/DM subjects compared with controls, although further work is required to elucidate the underlying metabolic processes.

Adult↗