Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Corticosteroids and immunosuppressive agents are standard treatment for polymyositis (PM) and dermatomyositis (DM) respectively. Recent reports have emphasized a potentially successful regimen with intravenous immune gammaglobulins (IVIG). The short term success of this treatment in a personally observed case is described. IVIG treatment resulted in normalization of the serum concentrations of the muscle enzymes after continued inflammatory activity under treatment with azathioprine, cyclophosphamide and methotrexate in combination with corticosteroids. The improvement of PM by IVIG was further documented by an increase in muscle strength of up to 367% of the initial value and a regression of the myositic changes in the muscles of the thighs as evidenced by magnetic resonance imaging (MRI). The therapeutic response was paralleled by reversal of peripheral lymphopenia. Experience with IVIG treatment in PM/DM is reviewed and the potential role of this regimen in the management of PM/DM is discussed.
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PMID:[Polymyositis: disease course and therapy with intravenously administered immunoglobulins]. 141 5

Juvenile dermatomyositis (JDMS) is a systemic vasculopathy characterized primarily by inflammation of skin and muscle. JDMS is identified in more than three per million persons per year, using established diagnostic criteria. Although originally thought to be a relatively homogeneous disease, new data confirm that heterogeneity in JDMS may be found at several levels and that each variant may be associated with a different disease course. Unlike adults with dermatomyositis, of whom more than 50% have a specific myositis-associated antibody (MSA), a much smaller number of children appear to test positive for a known MSA (about 10%), despite the evidence that more than 60% of children with JDMS test positive for antinuclear antibodies. In children, the most common MSA is directed against Mi-2, not toward one of the tRNA synthetases, such as tRNA histidine, as is found in 20% to 30% of adults with myositis. About 50% of children with JDMS have circulating evidence of endothelial cell damage (increased vWF:Ag), whereas others have different indicators of disease activity, such as elevated neopterin (> 60%) or increased circulating B cells with peripheral lymphopenia (> 80%). Newer modes of assessment of functional ability may help evaluate response to therapy. Finally, physicians with newly diagnosed (< 6 months) JDMS patients are urged to call the new National Institutes of Health Rare Disease Registry for New Onset Dermatomyositis (312-880-3333) to enroll their patients and for more information on the onset of this disease.
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PMID:An update on juvenile dermatomyositis. 851 18

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.
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PMID:T-lymphocyte immunophenotyping in polymyositis and dermatomyositis. 881 Jun 66

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.
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PMID:Fulminant Pneumocystis carinii pneumonia in 4 patients with dermatomyositis. 922 30

A 22-year-old male with juvenile dermatomyositis presented with fever up to 40 degrees C and acute pain in his right thigh accompanied by muscle weakness, a skin rash and a tender swelling. Serum aspartate aminotransferase (AST) and aldolase were mildly elevated. C-reactive protein (CRP) and fibrinogen were markedly increased. The differential white blood cell count revealed relative lymphopenia. Radiography showed diffuse calcifications particularly around the thighs and knees of both legs. Magnetic resonance imaging (MRI) demonstrated inflammatory infiltrates in the right thigh. The lesions were identified as phlegmone by immunoszintigraphy with 99mTc-labelled antigranulocyte antibodies. On the 10th day of treatment Staphylococcus aureus was cultured from blood. Patients with juvenile dermatomyositis and calcinosis may develop bacterial infections of soft tissue which sometimes mimic a disease flare. For differential diagnosis plain radiographs, CT scans and MRI are of limited value. Immunoszintigraphy is able to differentiate between infiltrates caused by granulocytes and lymphocytes.
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PMID:[Juvenile dermatomyositis--acute recidivism or sepsis?]. 1041

Lymphocytopenia has been reported in patients with connective tissue diseases, including dermatomyositis (DM). However, the risk of infectious complications and the changes of lymphocytic subsets during treatment have been poorly investigated in these patients. We investigated the alterations of peripheral blood lymphocyte counts in patients with DM. A retrospective analysis was conducted in patients with an ascertained diagnosis of DM admitted from 1994 to 2000 in both departments of Dermatology of the Saint-Louis Hospital in Paris. All patients had a peripheral blood absolute lymphocyte count available before therapy. From an initial set of 63 patients, 47 were included in the study. The median absolute lymphocyte count was 888/mm(3) (range, 400-4,070). Low peripheral blood CD4+ and CD8+ T-cell and B-cell counts were consistent findings (median CD4+: 382/mm(3); CD8+: 211/mm(3); CD19+: 122/mm(3)). There was a significant increase in lymphocyte count after 1 month (p < 0.0001), 3-6 months (p = 0.001), and 6-12 months (p = 0.0005) of corticosteroid treatment. Infectious events, mainly pneumonia (PCP), occurred in 12 patients. Their initial lymphocyte count was lower than that of patients who did not develop infections (p = 0.0001). These results support the high prevalence of lymphocytopenia in patients with DM and emphasize the risk for opportunistic infections, mainly PCP, in these patients. Further studies are warranted to evaluate the risk/benefit balance of PCP prophylaxis in patients with DM and severe lymphocytopenia.
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PMID:Peripheral blood lymphocyte subset counts in patients with dermatomyositis: clinical correlations and changes following therapy. 1264 Jan 84

The association of systemic lupus erythematosus (SLE) with idiopathic polymyositis or dermatomyositis is reported to occur in the range of 4-16%. Myositis can occur before or after SLE, or sporadically both diseases can be present simultaneously. This case report concerns a 36-year-old female patient suffering from Raynaud's phenomenon, polyarthralgia in the small joints of the hands, and skin changes compatible with Gotron's indications. Symmetric proximal muscle weakness of the extremities, fever of up to 40 degrees C, heliotrope rashes with erythematous changes in the face, upper arms, and posterior shoulders occurred subsequently. Laboratory analyses revealed increased acute phase reactants, hypochromic anaemia, lymphopenia, and increased levels of all muscle enzymes. Immunoserology demonstrated positive ANA, anti-Sm, and anticardiolipin antibodies (aCL), while anti dsDNA, anti Ro, anti La, and anti Jo-1 antibodies proved negative. Hypocomplementaemia and elevated levels of immune complexes were also detected. Pathologic sediment and proteinuria were revealed via urine analyses, while a kidney biopsy confirmed lupus nephritis (type IVa according to the World Health Organisation classification). Biopsy of erythematous changes of the posterior shoulder demonstrated leukocytoclastic vasculitis. Electromyography of the lower extremities established myopathic changes. Inflammation of the muscles was confirmed via magnetic resonance imaging. The patient was categorised as having two separate coexistent diseases--SLE and dermatomyositis. Both the classification criteria of the American College of Rheumatology for SLE and the diagnostic criteria for dermatomyositis, proposed by Bohon and Peter, were fulfilled simultaneously. Treatment commenced with pulses of methylprednisolone and continued with oral therapy, including Resochin. Pulses of intravenous cyclophosphamide were also administered. After six weeks of therapy, biohumoral remission of both diseases was achieved, while complete recovery from muscle weakness was accomplished after four months.
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PMID:[Systemic lupus erythematosus and dermatomyositis--case report]. 1653 99

Infections result in increased mortality rates in patients with polymyositis/dermatomyositis, leading to death in 9 to 30% of cases. The following parameters can be considered of predictive value for infection onset in polymyositis/dermatomyositis: age, lymphopenia, esophageal dysfunction, ventilatory insufficiency, interstitial lung disease, calcinosis cutis, as well as higher mean daily doses of steroids. A great variety of microorganisms may be responsible for pyogenic and opportunistic infections in polymyositis/dermatomyositis. Opportunistic infections are more often due to mycobacteria and fungi (Pneumocystis jiroveci, Candidasp.). Because a great variety of microorganisms may be responsible for opportunistic infections, it seems difficult to initiate primary prophylaxis in patients with polymyositis/dermatomyositis who exhibit risk factors for opportunistic infections. Primary prophylaxis of Pneumocystis jirovecipneumonia should be given in the group of patients exhibiting CD4-cell count lower than 250/mm(3). Vaccination should be performed in patients with polymyositis/dermatomyositis, prior to immunosuppressive therapy institution.
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PMID:[Infections in polymyositis and in dermatomyositis]. 1908 69

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.
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PMID:[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]. 1999 56

Dermatopolymyositis (DPM) is a term describing a group of disorders comprising multiple distinct entities depending on interactions between genetic and environmental factor. There is a paucity of studies on DPM in black Africa. The purpose of this report is to describe epidemiological, clinical, laboratory and therapeutic aspects of dermatomyositis (DM) and polymyositis (PM) observed at the Principal Hospital in Dakar, Senegal. A retrospective review as conducted of patients hospitalized for DM and PM in Medical Departments of Principal Hospital. Diagnosis of DRM was based on the criteria of Bohan and Peter's in all cases. A series of 21 black African patients was compiled including 15 with DM and 6 with PM. Mean age was 52 years and the M/F sex ratio was 0.6. The mean delay for diagnosis was 6 weeks (range, 3 to 12 weeks). Initial signs were dermatological in 12 patients, pulmonary in one and muscular in the remaining cases. The most common dermatological sign was erythema characterized by a zebra-like aspect on the extended limbs. Erythema was frequently pruriginous with a flagellate aspect on the back. Muscular signs were observed in 18 patients and included pharyngeal manifestations in 10 patients. Amyopathic DM was not observed. Cardiac abnormalities included tachycardia (4 cases), AVB (1), ischemic lesion (1), relaxation disturbances (4), pericardial effusion (3), myocarditis (2) and pulmonary hypertension (1). The most common pulmonary manifestation was interstitial lung disease observed in 6 patients. Gastrointestinal signs were noted in 9 patients including endoscopic evidence of superficial erosion in 4 cases. Electromyography (EMG) tracings revealed myogenic disease in 14 cases including 2 associated with reduced peripheral nervous conduction speed. Severe lymphopenia was observed in 3 patients but HIV serology was negative in all cases. Paraneoplasic DM was observed in 3 cases. Death occurred in 5 cases due to the cancer-related, pulmonary and infectious complications. Based on the findings of this study, the three main features of DM and PM in Senegal are flagellated and often pruriginous erythema, cardiac and interstitial lung disease, and peripheral neural involvement.
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PMID:[Dermatomyositis and polymyositis: 21 cases in Senegal]. 2048 54


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