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PubMed2005

[Importance of aggressive treatment in juvenile dermatomyositis].

Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children. Its outcome depends on the precocity of the diagnosis and of the treatment, but predictive parameters for guiding the correct therapeutic and prognostic approaches to JDM are still lacking. We analysed the one-year-old outcomes of 20 JDM patients treated with methylprednisolone boluses, methotrexate, and cyclophosphamide, through a longitudinal retrospective study. The outcome variables included: the Childhood Myositis Assessment Score (CMAS); Manual Muscle Testing (MMT); the Childhood Health Assessment Questionnaire (CHAQ); the Child Health Questionnaire (CHQ: physical score CHQ PhS and psycho-social score CHQ PsS), patient and parent Visual Analogue Scale (VAS), as well as laboratory data: ESR, LDH, CK, and ALT. Within all JDM patient groups, we discovered significant improvement in all disease activity parameters CMAS (p<0.001) and MMT (p<0.001), followed by a significant decrease in CHAQ (p<0.001), as well as parent VAS (p<0.001) and physician VAS (p<0.001). With regard to laboratory parameters, only CK (p=0.001) and LDH (p=0.013) levels were found to be significantly decreased, while there were no significant changes in ESR and ALT. The results of our study support the findings that the aggressive treatment of JDM patients improves their short-term outlook.

Child↗
PubMed1981

Calcinosis in dermatomyositis treated with probenecid.

Childhood dermatomyositis is frequently complicated by deposition of calcium salts in the soft tissues. This calcinosis represents a major problem, as severe disability may result. Various drugs have been tried to dissolve the calcinosis, but the results of these trials are disappointing. In this article we present a case report of a 9-year-old girl treated with probenecid. This treatment led to a dramatic decrease in subcutaneous and intermuscular calcinosis. The authors call for controlled studies of probenecid treatment in calcinosis.

Calcinosis↗
PubMed1983

Penicillamine-induced dermatomyositis. A case history.

A 69-year-old woman with classical rheumatoid arthritis developed a severe dermato-myopathy during treatment with penicillamine. Remission occurred on withdrawal of the drug. Penicillamine (dimethylcysteine) is a pharmacological agent used for its chelating properties in the treatment of Wilson's disease and heavy metal poisoning, and in cysteinuria because of soluble disulphide formation. Within the last 17 years penicillamine has been increasingly applied in the treatment of rheumatoid arthritis, the mechanism of action still being unknown. A great number of side effects have been reported, including less common auto-immune disorders such as drug-induced systemic lupus erythematosus, myasthenia gravis and polymyositis. These and other possible side effects have been well reviewed by others (1, 2). To our knowledge only a few earlier cases of dermatomyositis as a complication to penicillamine treatment of rheumatoid arthritis have been reported (3, 4, 5). We describe here another case.

Aged↗
PubMed1999

A case of Sweet's syndrome in patient with dermatomyositis.

Sweet's syndrome (SS) has been reported as an association with malignant neoplasms and autoimmune diseases, e.g., Behçet's disease, Sjogren's syndrome, and rheumatoid arthritis. But dermatomyositis (DM), one of the rare autoimmune diseases, was not reported as an associated disease of SS. We describe an interesting case of SS associated with DM. Diagnosis was made by skin biopsy, and subsequent clinical resolution occurred after institution of prednisolone.

Dermatomyositis↗
PubMed2002

Gene expression profiling in DQA1*0501+ children with untreated dermatomyositis: a novel model of pathogenesis.

Juvenile dermatomyositis (JDM), the most common pediatric inflammatory myopathy, is a systemic vasculopathy affecting young children. Epidemiology studies documenting an antecedent illness in the 3 mo before the first definite symptom (rash and/or weakness) of JDM are supported by immunologic data that suggest that the disease pathophysiology is Ag driven. The purpose of this study was to compare the gene expression profiles in muscle biopsies of four untreated DQA1*0501(+) JDM children with profiles from children with a known necrotizing myopathy (Duchenne muscular dystrophy), as well as an in vitro antiviral model (NF90), and healthy pediatric controls. Nearly half (47%) of the dysregulated genes in JDM were associated with the immune response. In particular, increased expression of IFN-alphabeta-inducible genes 6-16, myxovirus resistance protein p78, latent cytosolic transcription factor, LMP2, and TAP1 was observed. This profile is consistent with an IFN-alphabeta transcription cascade seen in the in vitro viral resistance model. The IFN-alphabeta-inducible profile was superimposed on transcription profiles reflective of myofiber necrosis and regeneration shared with Duchenne muscular dystrophy. Expressed genes were confirmed by quantitative real-time PCR (6-16), immunofluorescence (thrombospondin 4), and immunolocalization (IFN-gamma, p21). We hypothesize that these data support a model of Ag (?viral) induction of an apparent autoimmune disease based on dynamic interaction between the muscle, vascular, and immune systems in the genetically susceptible (DQA1*0501(+)) child.

Adolescent↗
PubMed2004

Does HLA-dependent chimerism underlie the pathogenesis of juvenile dermatomyositis?

Juvenile dermatomyositis (JDM) is a multisystem autoimmune disease that at times resembles chronic graft-vs-host disease. This led us to suggest that nonself cells may play a role in the disease process. In this study we examined the relationship between HLA genotype and the presence of maternally derived chimeric cells in JDM patients and healthy controls, and assessed immunologic activity in the chimeric cells. We identified chimeric cells more often in children with JDM (60 of 72) than in their unaffected siblings (11 of 48) or in healthy controls (5 of 29). The presence of chimerism in the JDM patients, their healthy siblings, and unaffected control children was associated with a HLA-DQA1*0501 allele in the mother (p = 0.011). Further, we show that maternally transferred chimeric T cells are responsive to the host's (JDM childs') lymphocytes (33.75 +/- 8.4 IFN-gamma-producing cells from JDM cells vs 5.0 +/- 1.25 from maternal cells), and that this is a memory response. These combined data indicate that chimeric cells play a direct role in the JDM disease process and that the mother's HLA genotype facilitates the transfer and/or persistence of maternal cells in the fetal circulation.

Adolescent↗
PubMed1989

Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders.

Idiopathic inflammatory myopathy, a category encompassing polymyositis, dermatomyositis, and a number of other disorders, is very uncommon, but has been the focus of intense study in the Arthritis and Rheumatism Branch of the National Institute of Arthritis and Musculoskeletal and Skin Diseases for the past several years. We describe the clinical picture, stressing the need for biopsy to ensure correct diagnosis. It is especially important to recognize the treatment-resistant variant, inclusion body myositis. The extraskeletal manifestations, particularly the cardiopulmonary, oropharyngeal, gastrointestinal, and endocrine involvement, are described. The cardiopulmonary involvement, especially interstitial lung disease, arrhythmias, and cardiac failure, may dominate the clinical picture. The known causes are varied, and include drugs, toxins, and some infectious agents, however, in most cases a cause cannot yet be identified. Circumstantial evidence suggests that picornaviruses may initiate some cases in humans, and a very similar disease in mice caused by a picornavirus is actively under study. Studies of autoantibodies and cellular immune function support a central role for disordered immunity in the pathogenesis. The myositis-specific autoantibodies, especially those directed at certain enzymes important in protein synthesis (the aminoacyl-transfer RNA synthetases), are found in a clinically distinct subset of patients. Although most patients respond initially to corticosteroids, cytotoxic drugs are sometimes added when steroid toxicity or refractoriness develops. We describe several newer therapies under study for such cases and outline future directions in research.

Animals↗
PubMed1999

[Polymyositis and dermatomyositis].

This review summarizes the current progress in the clinical research on pathogenesis, diagnosis and treatment of polymyositis (PM) and dermatomyositis (DM). Recent studies on immunohistochemical and molecular findings of biopsied muscle tissues have shed light on pathogenetic mechanisms in myositis. Muscle imaging techniques such as ultrasonography and magnetic resonance imaging facilitate the assessment of the type (edema, inflammation, fat, and fibrosis), degree, and localization of these alterations. They are useful for the diagnostic evaluation and the assessment of treatment. Measurement of myositis-specific autoantibodies such as antisynthetases (anti-Jo-1 and others), anti-Mi-2, and anti-SRP is also useful for both diagnosis and classification of subgroup of patients with clinical, prognostic, and possible therapeutic implications. Novel treatment of refractory myositis includes methotrexate, cyclosporin, and intravenous high dose immunoglobulin. Anti-cytokine therapy will be a novel strategy for the treatment of myositis.

Autoantibodies↗
PubMedDate not supplied

Juvenile dermatomyositis: clinical profile and disease course in 25 patients.

A retrospective analysis of 25 Arab patients with juvenile dermatomyositis (JDMS) was conducted between 1988 and 1996. The mean age at disease onset was 8.25 years (range 1.5-15 yrs) with a male: female ratio of 1.5:1. The disease duration before diagnosis was 1-108 months. Two patients had a family history of JDMS. The clinical features included fever in 14 patients (56%), weight loss in 20 (80%), muscle weakness in all 25 (100%), and muscle pain in 14 (56%). Skin lesions included Gottron's papules in 15 patients (60%), heliotrope in 13 (52%), erythematous malar rash in 8 (32%), and pigmentary changes in 12 (48%). Seventeen of the 25 patients had arthralgia (68%) and 16 patients had arthritis (64%). Gastrointestinal symptoms were noted in 19 patients (76%). Myocarditis with cardiac failure was the initial presentation of 1 patient, while 2 had conduction defect. Twelve patients (48%) had respiratory symptoms. The course of the disease was complicated by calcinosis in 10 patients (40%). All of the patients were treated with prednisone; 15 were also treated with methotrexate. The duration of follow up ranged from 6-108 months (mean 54.5 months). Twenty-three patients improved, including those who had calcinosis at the time of presentation, with a current muscle power of 4/5 in 10 patients (40%) and 5/5 in 13 patients (52%). No deaths were reported in our series and no patients are currently bedridden.

Adolescent↗
PubMed1999

Decreased capillary density in juvenile dermatomyositis and in mixed connective tissue disease.

OBJECTIVE: To assess whether quantitative capillary microscopy is a useful tool to evaluate capillary abnormalities in children with connective tissue diseases. METHODS: Eight children with juvenile dermatomyositis (JDM), 6 with mixed connective tissue disease (MCTD) and 23 healthy children were investigated with computer based quantitative capillary microscopy. Median disease duration was 1 year among JDM (1-4) and 3 years (1-7) among MCTD. RESULTS: Capillary density was decreased in JDM [median 2.5 (1.4-4.3) loops/mm (p < 0.001)] and in MCTD [median 5.0 (4.1-7.0) loops/mm (p < 0.05)] compared to healthy controls [median 6.8 (5.3-8.0) loops/mm]. Median capillary loop area was increased in JDM [median 8.5 (3.0-15.8) 10(-3) mm2 (p < 0.001)] and in MCTD [median 4.5 (3.0-6.0) 10(-3) mm2 (p < 0.02)] compared to controls [median 2.5 (1.0-4.0) 10(-3) mm2]. CONCLUSION: Quantitative nailfold capillary microscopy is a sensitive indicator of JDM. In MCTD this technique is less discriminative.

Adolescent↗
PubMed1999

[Amyopathic dermatomyositis with interstitial pneumonia: effective treatment with cyclophosphamide pulse therapy].

A 42-year-old man was admitted to our hospital because of dyspnea on exertion, skin eruptions on the face and extremities, and interstitial shadows mainly in the lower fields of both lungs. Characteristic skin lesions and skin biopsy findings without muscle symptoms or elevated CPK resulted in a diagnosis of amyopathic dermatomyositis (ADM). Thoracoscopic lung biopsy specimens disclosed BOOP-type interstitial pneumonia. Oral prednisolone (PSL) was initiated at 60 mg/day and gradually tapered. However, because his respiratory symptoms and laboratory findings deteriorated, the patient underwent 5 courses of cyclophosphamide (CPA) pulse therapy. Following improvement of his subjective symptoms, arterial blood gas data, and respiratory functions, the patient was discharged and placed on oral PSL and CPA. CPA was discontinued 18 months later. The patient has continued to receive PSL (5 mg/day) and has been in good condition for 3 years. Though the prognosis for interstitial pneumonia associated with ADM is reported to be poor, our patient represented a rare case of BOOP-type interstitial pneumonia brought into remission by CPA pulse therapy.

Adult↗
PubMedDate not supplied

Long-term prognosis of patients with juvenile dermatomyositis initially treated with intravenous methylprednisolone pulse therapy.

OBJECTIVE: To assess the effects of initial treatment with intravenous methylprednisolone (IVMP) on the clinical course and laboratory measurements in patients with juvenile dermatomyositis (JDM), and to report the long-term prognosis of these patients. METHODS: This was a retrospective study of 24 children with JDM, treated with IVMP as the initial therapy since 1981 and followed for a mean of 5.3 yrs. (range 10 months to 12.5 yrs.). Clinical and laboratory data, and complications of the disease and of therapy were ascertained by a chart review. RESULTS: The disease in 13/24 patients initially treated with IVMP followed a monocyclic course. The mean time for patients with a monocyclic course to normalize skin and muscle strength parameters was shorter than for patients with a chronic course (p < 0.005). Patients with a chronic course received more IVMP (mean 12.3 doses) (p < 0.005), and took longer to achieve normal strength (14.9 mos.) (p < 0.005), and remission of skin rash (33.7 mos.) (p < 0.005). Patients with a chronic course suffered more disease- and treatment-related complications than patients with a monocyclic course. CONCLUSION: Most patients whose disease followed a monocyclic course achieved normal muscle enzymes within one month and had improved muscle strength within 2.5 months of the initiation of therapy. If patients fail to achieve normal muscle strength over 4 months, muscle enzymes over 3 months and normalizing VWF over 10 months, there may be a higher probability that their disease will follow a chronic course.

Adolescent↗
PubMed1999

Intravenous immunoglobulins as treatment of life threatening esophageal involvement in polymyositis and dermatomyositis.

Esophageal involvement is considered a major cause of morbidity and an indicator of poor prognosis in polymyositis (PM) and dermatomyositis (DM). We describe 3 patients with steroid resistant PM/DM with life threatening esophageal involvement, resulting in impossible oral feeding and enteral nutrition with a gastric tube. All patients had both dramatic and rapid improvement of all clinical manifestations after initiation of intravenous immunoglobulin (IVIG) therapy. Swallowing disorders completely disappeared after the second infusion of IVIG, which permitted normal oral feeding and ablation of the gastric tube. Our findings suggest IVIG should be considered the treatment of choice in such cases.

Adult↗
PubMed1999

[A case of amyopathic dermatomyositis presenting blister and oral ulcer].

An 84-year old man was admitted to Mitoyo General Hospital because of progressive malaise and edematous erythema on both eyelids (Heliotrope erythema). He also noted blister on his neck as well as erythema on the extensor surface of finger joints (Gottron's sign), elbows and knees. Neither weakness nor pain of his proximal muscle was elicited on physical examination on admission. His blood test disclosed positive inflammatory signs (i.e., mild elevation of ESR and positive CRP) without elevated value of muscle enzymes. Electromyogram showed normal pattern. Infiltration of inflammatory cells was not revealed by histological examination of biopsied muscle. A diagnosis of 'amyopathic dermatomyositis' was made based on these observations. Computed tomography of his chest disclosed interstitial pneumonia spreading over both lower lung fields. Colon fiberscopy revealed a polyp in his descending colon, which was classified into group I on cytological examination. He was treated with two sets of methylprednisolone (mPSL) pulse therapy (500 mg/day, 3 consecutive days, intravenously) followed by 30 mg/day of oral prednisolone (PSL). His skin lesions responded well to the above treatment and the dose of oral PSL was tapered. One month after the initiation of treatment, severe stomatitis as well as a large ulcer beneath his tongue developed accompanying an intractable pain. Mucosal biopsy revealed necrotizing vasculitis in medium-sizedartery at the bottom of ulcer. Another set of mPSL pulse therapy brought a prompt relief of his symptom and prohibited the recurrence of oral lesion. It should be noted that our patient did not fulfill the diagnostic criteria for DM because of the lack of muscular symptoms whereas he had characteristic skin lesions. Regarding the frustration possibly encountered at the time of diagnosing amyopathic DM, both sensitivity and specificity of the skin lesion for the diagnosis of DM were investigated. Moreover, the rarity of blister as a skin manifestation of DM was discussed as well.

Aged↗
PubMed2000

Increased expression of interleukin 1alpha and MHC class I in muscle tissue of patients with chronic, inactive polymyositis and dermatomyositis.

OBJECTIVE: To investigate if there is a molecular correlate in muscle tissue to the persisting decreased muscle function in patients with chronic, inactive polymyositis (PM) and dermatomyositis (DM). METHODS: Muscle function was assessed using a muscle function index of myositis. To assess disease activity both histopathological investigation of muscle biopsies and magnetic resonance imaging (MRI) scans of the thigh muscles were performed. Inactive chronic disease was defined as persisting muscle weakness and absence of inflammatory infiltrates in muscle biopsy and absence of signs of inflammation on MRI. Expression of interleukin 1alpha (IL-1alpha), IL-1beta,, adhesion molecules, and MHC class I molecules in muscle tissue was investigated with immunohistochemistry. RESULTS: Muscle weakness was confirmed by a reduction of muscle function score. No signs of inflammation typical for myositis were observed. The most striking finding in our study was the strong expression of IL-1alpha and MHC class I molecules in muscle tissue from patients with inactive chronic PM and DM. Increased IL-1alpha expression was evident in capillaries and increased MHC class I expression was detected in muscle fiber membranes. CONCLUSION: IL-1alpha and MHC class I molecules may have an importance in the pathogenesis of the chronic muscle weakness and fatigue in patients with PM and DM.

Adult↗
PubMed2000

Seropositive rheumatoid arthritis with dermatomyositis sine myositis, angioimmunoblastic lymphadenopathy with dysproteinemia-type T cell lymphoma, and B cell lymphoma of the oropharynx.

Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder that often progresses to high grade T cell lymphoma. We describe a 63-year-old woman with longstanding seropositive rheumatoid arthritis who developed fever, cutaneous findings of dermatomyositis, a diffuse pruritic maculopapular rash, enlarged lymph nodes, polyclonal elevated serum gammaglobulins, and an IgG lambda paraprotein. Lymph node biopsies yielded tissue with characteristic changes of AILD and T cell lymphoma. Interleukin 6 (IL-6) was present during the early, active phase of disease, and circulating IL-6 and IL-2 were detected one month before tumor recurrence. Two years after AILD and T cell lymphoma were diagnosed, she developed a B cell lymphoma that involved the oropharynx.

Arthritis, Rheumatoid↗
PubMedDate not supplied

Features of polymyositis and dermatomyositis in the elderly: a case-control study.

OBJECTIVE: Polymyositis (PM) and dermatomyositis (DM) are uncommon idiopathic inflammatory myopathies (IIM). Little is known about these diseases in the elderly. We attempted to define the characteristics of PM/DM in the elderly by a case-control study involving the retrospective review of medical files of PM/DM patients. METHODS: We drew from among 200 PM/DM patients being followed in our Internal Medicine Department 21 patients (14 F/7 M), aged > or = 65 years at the onset of myositis (17 PM/4 DM) (mean: 69.9 +/- 4.8 yrs.). They were compared with 21 (15 F/6 M) randomly selected younger patients with IIM: PM (14) and DM (7) (mean: 46.4 +/- 12.4 yrs). Clinical, biological, electrophysiological and pathologic features, treatment regimens and side-effects in the 2 groups were collected. RESULTS: Clinical features were similar for the 2 groups. Elderly patients tended to have a higher frequency of cancer (24% vs 9.5%, p = 0.06), particularly of rectal adenocarcinoma. The time from disease onset to diagnosis was significantly longer in older patients (26 +/- 37 months vs 9 +/- 15 months; p = 0.02), normal CK levels were more frequent (40% vs 5%; p = 0.02) and serum CK levels were lower than for the population as the whole (11.5 N vs 22 N, p < 0.03). Electromyography features were more frequently suggestive of a chronic form of the disease in elderly patients. Treatment regimens and short-term side-effects were similar for the 2 groups. CONCLUSION: PM and DM are often diagnosed late in the elderly. Biological data and electromyography features argue for a chronic form of the disease in this age group. Clinical and endoscopic rectal examinations should be carried out in elderly patients with PM/DM.

Adenocarcinoma↗