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PubMed2001

Juvenile dermatomyositis: the association of the TNF alpha-308A allele and disease chronicity.

Little is known concerning factors associated with the outcome of juvenile dermatomyositis (JDM), which can be variable and lethal. Previous work has documented that the association of DQA1*0501 with JDM is higher than in control groups and that the first symptoms (rash and weakness) of JDM appear to follow evidence of an infectious process--most frequently upper respiratory in nature. Preliminary data show that a long period of symptoms being left untreated before starting therapy and the TNF alpha-308A allele are associated with prolonged JDM symptoms requiring > or = 36 months of immunosuppressive therapy. A short duration of untreated disease is associated with a relative increase in CD8(+) T cells and CD56(+) natural killer (NK) cells in the untreated JDM muscle biopsy compared with a longer duration of untreated disease. The TNF alpha-308A allele is overrepresented in white children with JDM. In addition, it is associated with pathologic calcifications, increased production of TNF alpha by peripheral blood mononuclear cells in vitro and JDM muscle fibers in vivo, and occlusion of capillaries, which may be mediated in part by elevated circulating levels of thrombospondin-1, a potent anti-angiogenic factor. We speculate that DQA1*0501 is associated with JDM susceptibility to an infectious process, eliciting and activating NK cells early in the disease course. We conclude that the TNF alpha-308A allele indicates directly (or is a surrogate marker of) children with JDM who produce higher concentrations of TNF alpha in response to this undefined inflammatory stimulus, as well as increased concentrations of TSP-1 with resultant small vessel occlusion, contributing to subsequent disease chronicity.

Adolescent↗
PubMed2006

Treatment of refractory polymyositis and dermatomyositis.

Polymyositis (PM) and dermatomyositis (DM) are autoimmune inflammatory diseases that primarily target muscle. Although similar, PM and DM have different pathophysiologic mechanisms. Current therapy for PM and DM does not take into account these pathophysiologic differences. Recent work has started to define outcome measures to apply to future therapeutic trials, which will allow better comparison of treatment outcomes. For patients who are unresponsive to standard therapy with high dose prednisone supplemented by methotrexate and/or azathioprine it is not clear what represents the next best choice for therapy. Although there are few controlled studies in the area, there is reason to be optimistic for use of intravenous methylprednisolone pulses, intravenous gammaglobulin, cyclosporine A, tacrolimus, mycophenolate mofetil, and rituximab for nonresponding patients. Better understanding of the underlying pathophysiology of PM and DM, as well as carefully performed multicenter clinical trials is going to be necessary to make better recommendations in the future.

Dermatologic Agents↗
PubMed2006

Juvenile dermatomyositis update.

Recent studies involving juvenile dermatomyositis indicate that the majority of affected children have symptoms suggestive of infection prior to disease onset, damage to skin and muscle each have a distinct pathophysiology, certain urinary muscle metabolites may be useful laboratory markers, and methotrexate used as first line therapy with corticosteroids is associated with greater height velocity and smaller increase in body mass index.

Biomarkers↗
PubMed2006

Clinical manifestations and pathogenesis of hydroxyapatite crystal deposition in juvenile dermatomyositis.

Pathologic deposition of mineral in the form of bone-like hydroxyapatite is a frequent occurrence in juvenile dermatomyositis (JDM) and other connective tissue diseases. Although the sizes of the mineral crystals in JDM are similar to those in bone, there is much more mineral in the deposits than there is in bone. Bone matrix proteins also accumulate associated with the deposits. The reasons for the formation of these deposits are not known. It is our hypothesis that persistent inflammation is a component of JDM and other hydroxyapatite deposition diseases. Other contributing factors are genetic, environmental, and physical chemical. This paper discusses the influence of inflammation on the deposition of hydroxyapatite, with emphasis on the clinical and environmental factors that may facilitate the formation of calcific deposits in JDM.

Adolescent↗
PubMed1975

Amyloidosis associated with dermatomyositis and features of multiple myeloma. The progression of amyloidosis associated with corticosteroid and cytotoxic drug therapy.

Described here is a 59 year old man with dermatomyositis and hypogammaglobulinemia. His muscle power improved after corticosteroid therapy, but extensive amyloidosis and repeated infections appeared. Bone marrow morphology suggested multiple myeloma, but treatment with cytotoxic drugs had no beneficial effect on the amyloidosis. Because of rapid progression of the amyloidosis and further infections, cytotoxic drug therapy was stopped, corticosteroid dosage was decreased, and supplementary immunoglobulin therapy was instituted. The infections occurred less frequently and the amyloidosis appeared to regress. This case suggests that immunosuppressive therapy may exacerbate amyloidosis. The literature is reviewed, and the possible role of humoral immunodeficiency in the pathogenesis of amyloidosis is discussed. It is suggested that supplementary immunoglobulin may be beneficial in amyloidosis.

Agammaglobulinemia↗
PubMed1979

Articular manifestations of polymyositis and dermatomyositis.

Nine patients with polymyositis or dermatomyositis associated with arthritis without antinuclear antibodies have been studied. In eight patients a mildly inflammatory nonerosive arthritis occurred coincident with early manifestations or before the appearance of symptomatic muscle disease. Hands, wrists and knees were prominently involved. This arthritis responded to corticosteroids given for the myositis. One patient had erosive arthritis and periarticular calcifications associated with acute inflammation that seemed to diminish with colchicine therapy. Synovial biopsy specimens showed surface fibrin deposition, focal loss of lining cells and mild inflammation--findings similar to those in scleroderma. By electron microscopy tubuloreticular structures were found in vascular endothelium in synovium, as in other tissues in polymyositis. All patients surprisingly had pulmonary manifestations suggesting the possibility that the condition in this group of patients may represent a distinctive subset of polymyositis.

Adult↗
PubMed1987

Dermatomyositis.

Dermatomyositis and polymyositis are related disorders of unknown cause in which immunologic changes may be important. They sometimes overlap with other collagen vascular diseases, and not infrequently are associated with malignancy. The clinical features of the adult and juvenile forms of the disorder and the visceral manifestations of DM-PM are outlined, along with enzymatic, electromyographic, and histologic aids to diagnosis. While the cutaneous lesions often are resistant to treatment, systemic involvement may respond to corticosteroids or to immunosuppressive drugs. Treatment definitely alters the prognosis in cases not associated with malignancy.

Adult↗
PubMed1978

Myasthenia in patients with dermatomyositis: clinical, electrophysiological and ultrastructural studies.

In 4 patients with clinical signs of dermatomyositis, confirmed by electromyography and muscle biopsy, a form of muscle fatigue was detected which was expressed clinically by predominantly proximal motor deficit, with phonation and deglutition disturbances, slightly influenced by prostigmine. In all patients, stimulation of the ulnar nerve at 3--10 Hz induced a decrement of muscle-evoked potentials in abductor digiti minimi and at 15--50 Hz an increment at the end of the trains (1.2 sec in duration) of repetitive stimulation (preceded in two cases by a decrement in the response to the fifth stimulus in the train). Stimulation at 30 Hz for 10 sec resulted in a transient facilitation, followed (at 3 Hz stimulation) by postactivation exhaustion which disappeared after 5--15 min. The post-tetanic facilitation, the incremental response and the myasthenic symptoms reverted to normal under treatment with corticosteroids, an immunosuppressor agent and guanidine hydrochloride. A mixed, pre- and postsynaptic mechanism is presumed to underlie the muscle fatigue in our patients. Electron microscopy of muscle biopsies disclosed zones of necrosis and, in incipient stages, large agglomerations of glycogen that had disorganized the structure of myofibrils. The end-plates in the biopsies were larger than normal and the cholinesterase reaction was hyperactive. Serum immunoelectrophoretic and electrophoretic data--increase of IgG and IgM, decrease of IgA and hypergammaglobulinaemia -- point to a possible autoimmune mechanism of the neuromuscular disorders in our patients.

Adolescent↗
PubMed1988

Perifascicular atrophic fibers in childhood dermatomyositis with particular reference to mitochondrial changes.

Ten of 17 muscle biopsy specimens from 15 patients with childhood dermatomyositis (DM) showed distinct perifascicular atrophy. The atrophic fibers showed the following characteristics: (1) decreased cytochrome c oxidase (CCO) activity, (2) rare positive reaction with acridine orange (AO) staining, (3) type 2C reaction in 7.9% (0.4-17.5%) of the fibers, (4) an increased number of activated satellite cells, (5) mitochondria which were increased in number but decreased in size, (6) a significantly decreased CCO activity in isolated mitochondria (51.6 +/- 30.3 nmol/min per mg mitochondrial protein) as compared with that in the controls (103.6 +/- 41.5). The major pathogenetic mechanism in muscles in childhood DM is thought to be ischemia due to involvement of the microvasculature. The presence of type 2C fibers and increased numbers of activated satellite cells reflect a focal repair process taking place concomitantly in the damaged myofibers. Mitochondrial enzyme defect, especially CCO deficiency is present not only in genetic disorders with mitochondrial involvement but in other neuromuscular disorders including inflammatory myopathies.

Age Factors↗
PubMed1990

Nature of the mononuclear infiltrate and the mechanism of muscle damage in juvenile dermatomyositis and Duchenne muscular dystrophy.

Normal skeletal muscle does not express class I MHC antigens. In contrast, these antigens are strongly expressed at the periphery of muscle fibres in patients with juvenile dermatomyositis (JDM) and Duchenne muscular dystrophy (DMD). Interferons can induce the expression of class I antigens, but in this study interferon-gamma could not be detected in JDM muscle biopsy specimens using four different immunocytochemical techniques. However, an infiltrate of mononuclear cells capable of synthesising interferons was present in biopsies from JDM and DMD patients. The predominant cell types detected in both diseases were macrophages and T lymphocytes, these two cell types comprising more than 80% of the infiltrating mononuclear cells. A striking predominance of CD4+ helper/inducer T cells was observed. The majority of these cells expressed class II MHC antigens and were, therefore, considered to be activated. Additional evidence for the functional activity of CD4+ T cells was derived from the finding that it was this population of cells from JDM biopsies which proliferated in response to interleukin-2 in vitro. T cell subsets in peripheral blood were also investigated in JDM and DMD patients. Only in the case of JDM were any significant differences from normal observed, where a significant reduction in the number of peripheral blood CD8+ T cells resulted in an elevation of CD4+/CD/8+ ratios. The role of CD4+ T cells and aberrant class I MHC antigen expression in mediating muscle damage in JDM and DMD is discussed.

CD4-Positive T-Lymphocytes↗
PubMed1994

Lymphoid neoplasms in patients with rheumatoid arthritis and dermatomyositis: frequency of Epstein-Barr virus and other features associated with immunosuppression.

We recently reported two cases of reversible Epstein-Barr virus (EBV)-associated lymphomas in patients undergoing methotrexate therapy for rheumatic disease. The current study was undertaken to investigate how frequently lymphoid neoplasms in patients with rheumatic disease show features of lymphoproliferations occurring in immunocompromised patients. Eighteen patients (including the two previously reported patients) with rheumatoid arthritis or dermatomyositis who developed lymphoproliferative lesions and on whom detailed clinical information was available were studied. As a group these patients developed a spectrum of lymphoproliferative lesions; however, a subset of patients developed neoplasms with features associated with immunosuppression. The neoplasms occurred in extranodal sites in 10 (56%) patients, showed a diffuse large-cell histology in nine (50%) patients, and contained EBV (EBER1) transcripts and EBV latent membrane protein in six (33%) patients. In three (17%) patients the neoplasms showed the entire constellation of features typical of immunosuppression-associated lymphoproliferations, including extranodal location, large-cell or polymorphous histology, geographic areas of necrosis, and the presence of EBV. These three patients were receiving both steroids and methotrexate at the time they developed their neoplasms. The findings of this study support the hypothesis that a subset of lymphoid neoplasms in rheumatic patients occurs in an immunocompromised setting and suggest that therapeutic immunosuppression may contribute, at least in part, to the development of these lymphoid neoplasms.

Adolescent↗
PubMed1977

Acute dermatomyositis-polymyositis and malignancy.

A partial review of selected published case reports of AD-P associated with malignancy has been enhanced by the presentation of pertinent data on 15 unreported examples of the association. It is noteworthy that the first case in current literature of AD-P associated with a malignancy was described in 1916. The brief clinical report of a patient with proximal muscle weakness and skin lesions, with the obvious association with a malignancy (adenocarcinoma of the stomach), describes an example that has been repeated many times with different types of tumors but with essentially no variations in the clinical findings. In 1959 Williams identified 590 cases of AD with an overall tumor rate of 15%, and recently Barnes identified 258 cases of AD associated with a malignancy. The original designation, dermatomyositis or AD, has now been expanded to include proximal muscle polymyositis with systemic involvement, which syndrome at the current state of the art is indistinguishable clinically and pathologically from AD except for the lack of skin lesions. It may be that at some future time one or more immunologic features may differnetiate the clinical entity polymyositis from AD and further subdivide each of these entitites from similar clinical syndromes associated with a malignancy. However, the problem in management in either AD or polymyositis is similar. A number of patients with a malignancy and muscle weakness or neuropathy have been reported. These associations have been mentioned briefly, but insufficient data are available to determine whether these should be considered as a variant of AD-P or only casually related conditions with certain clinical features in common. Most of the patients described in the literature of AD-P with an associated malignancy have had skin lesions; a minority only have lacked this feature. However, unless a patient is followed carefully, it is possible for a transient or evanescent erythema or insignificant skin lesions to be present and not recorded in the case record.

Acute Disease↗
PubMed1990

Adult-onset polymyositis-dermatomyositis: description of 25 patients with emphasis on treatment.

A retrospective study of 25 patients with polymyositis-dermatomyositis (PM-DM) is analyzed with special attention to the effects of therapy and follow-up. All patients (100%) complained of muscle weakness and 68% of these demonstrated typical skin changes of DM. All patients, except 2, received corticosteroids at the onset of the disease, 23 were treated with azathioprine, 7 received cyclophosphamide, 4 methotrexate, and 1 had total body irradiation. Among the patients adequately treated with azathioprine, 75% had a good response, but 5 patients did not improve. Cyclophosphamide was used subsequently in 2, with a satisfactory response in 1. Another patient had a striking response to oral methotrexate, and total body irradiation helped to improve another patient. Although high dose corticosteroids were the preferred starting medication for the treatment of PM-DM, it is important to detect those patients who do not respond adequately and/or develop side effects. In these circumstances, the prompt use of immunosuppressive agents appears justified.

Adult↗
PubMed1993

Juvenile dermatomyositis associated with partial lipoatrophy.

A 26-year-old woman developed partial lipoatrophy 12 years after juvenile dermatomyositis was diagnosed. Renal function was normal, but she had other features typically associated with partial lipoatrophy, including hepatomegaly, acanthosis nigricans, hypertrichosis, and hyperinsulinemia.

Adult↗
PubMed1991

Molecular genetic studies of major histocompatibility complex genes in children with juvenile dermatomyositis: increased risk associated with HLA-DQA1 *0501.

Juvenile dermatomyositis (JDMS) is an inflammatory disease associated with HLA-DR3. We therefore undertook molecular genetic studies of HLA region genes to determine whether HLA-DR3 itself confers susceptibility to JDMS or whether susceptibility is conferred by alleles in linkage disequilibrium with HLA-DR3. Our results indicate that JDMS is associated with the HLA-DQA1 allele DQA1 *0501 on non-DR3 haplotypes in Caucasian JDMS. Furthermore, the reported of association between the C4A gene deletion and JDMS is likely due to linkage disequilibrium with HLA-DR3.

Adolescent↗
PubMed1989

Nasopharyngeal carcinoma with dermatomyositis.

From 1976 to 1986, ten out of 1154 consecutive nasopharyngeal carcinoma (NPC) patients were found to have dermatomyositis (DM). Their clinical features and treatment results were analyzed. The skin manifestation was typical of DM and myopathy occurred in seven patients. All the tumor on presentation were early stage and locoregional control after radiotherapy was satisfactory. All but one patient had DM prior to diagnosis of NPC. The prognosis of NPC was not affected by DM with survival being comparable to contemporary controls. The complication of radiotherapy were unusually severe. Chronic radiation skin ulceration occurred in 2 patients and subcutaneous indurated fibrosis affecting both sides of neck occurred in all patients. Acute radiation mucositis was also prominent.

Adult↗
PubMed2006

Rare association of chronic lymphocytic thyroiditis with dermatomyositis.

Autoimmune thyroid disease (AITD) has been associated with other autoimmune diseases such as chronic urticaria, insulin-dependent diabetes mellitus, Sjøgren's syndrome, inflammatory bowel disease, rheumatoid arthritis, multiple sclerosis, systemic lupus erythematosus, psoriasis, ankylosing spondylitis, and dermatomyositis (DM). AITD is a common disorder affecting primarily women, and both genetic and environmental factors are included in its pathogenesis. DM is considered an autoimmune disease of the muscles and skin. Although AITD is the most common cause of hypothyroidism, to the best of our knowledge, only three cases of DM and AITD in the same patient have been reported in the last 40 years. We consider that both are developed from the same autoimmune background. Herein, we present a case of a 30-year-old man with a 4-year history of AITD who was diagnosed as suffering as well from DM.

Adult↗