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PubMedDate not supplied

Differential diagnosis of chronic idiopathic polymyositis and neuromyositis.

The term neuromyositis indicates a very rare type of polymyositis where, beside the usual muscular manifestations, there are signs of peripheral neuropathy. Although a lot of patients with neuromyositis has been published, it is a very controversial entity. There may be considerable difficulty in distinguishing chronic polymyositis from neuromyositis, even with the aid of electromyographic and muscle biopsy examinations. The aim of this investigation was to re-establish the clues for distinguishing chronic polymyositis from neuromyositis. Fifty-seven patients took part in this investigation. They were subdivided into two groups according to EMG and muscle biopsy data. First group consisted of 29 patients with chronic polymyositis and second group consisted of 28 patients with chronic neuromyositis. Our data confirm the existence of neuromyositis as an entity with different clinical, electromyographic and biopsy findings than polymyositis. The differentiation between chronic polymyositis and neuromyositis is a difficult task on purely clinical ground. Electromyographic recordings with widespread myopathic features, some large potentials and spontaneous activity could be found in both diseases. The most common and important electromyographic findings in patients with neuromyositis are prolonged sensory and motor nerve conduction velocities. In conclusion electromyography and muscle biopsy may be useful in the differential diagnosis of polymyositis and neuromyositis.

Adult↗
PubMed1989

[Anatomoclinical expressions of polymyositis in the child. 23 cases].

Childhood polymyositis is generally believed to exhibit specific characteristics including involvement of multiple systems, absence of malignancies, severe outcome, cortico-resistance, perforations of the bowel, and inflammatory vasculitis. On the basis of our experience with 23 cases and a review of the literature, we divide childhood polymyositis into five groups: 1) Severe acute dermatomyositis (11 cases) that fits the description above; 2) subacute or chronic polymyositis (4 cases) that resembles the chronic forms seen in adults; 3) infantile polymyositis (3 cases) whose inflammatory origin has not been proved and whose cause may be dystrophic; 4) inclusion polymyositis (2 cases) that are identical with adulthood inclusion myositis; 5) segmentary polymyositis (3 cases). Thus, in children as in adults, polymyositis remains an obscure disease and is a syndrome rather than an entity.

Adolescent↗
PubMed2004

[Polymyositis induced or associated with lipid-lowering drugs: five cases].

PURPOSE: Rhabdomyolysis and myositis are rare, dose-related complications of statins and fenofibrates. The outcome is favorable as a rule with rapid regression after stopping the responsible drug. Recently, various auto-immune disease with evidence of hypersensitivity to HMG-CoA reductase inhibitors or fibrates drugs have been reported. Less than ten cases of dermatomyositis and polymyositis due to cholesterol-lowering drugs (CLD) have been previously reported. Five more cases polymyositis associated with CLD are reported. METHODS: Symptoms were compatible with diagnosis of polymyositis according to Bohan and Peter and with previous reported criteria for drug-induced myopathy in all cases. None of these patients had previous other connective tissue disorders. RESULTS: Five patients (median age 68 [54-78], female N =4) with CLD treatment (statin N =4, fenofibrates N =1) have developed iatrogenic polymyositis. All of them presented both proximal muscular weakness and increased muscle enzyme levels. One patient had iatrogenic antisynthetase syndrome characterized by mechanic's hand, Raynaud's phenomenon and anti JO1 antibodies. One other had sclerodermic hand oedema. Antinuclear antibodies were positive in 4 cases and muscle biopsy revealed polymyositis infiltrate in 4 cases. CLD treatment was discontinued with partial clinical improvement in 3 cases. Clinical remission was obtained with corticosteroid (N =5) in association with immunosuppresive agents in 3 cases. CONCLUSION: Muscular symptoms in patient with CLD treatment could be the first symptom of a polymyositis revealed or increased by this treatment and must encourage physician with antinuclear antibodies screening especially in case of proximal muscular weakness and increased muscle enzyme levels.

Aged↗
PubMed2000

[Association between polymyositis and hepatitis C infection. Treatment-related difficulties].

INTRODUCTION: Chronic hepatitis C virus infection is often associated with various auto-immune disorders. We report four cases of an association between polymyositis and hepatitis C virus infection. The course and the difficulties of therapy are discussed. EXEGESIS: Among 510 consecutive patients infected by viral hepatitis C, we report four cases of polymyositis. Corticosteroids increased serum alanine aminotransferase levels in two cases, leading to severe liver injury in one patient. Worsening of polymyositis under interferon-alpha therapy was observed in one case. Clinical and biological stability were reported in another case. Aggravation of polymyositis with severe muscle weakness and dyspnea occurred in two patients after disruption of interferon-alpha treatment. Intravenous gamma globulins subsequently improved their condition, without biological worsening of viral hepatitis. CONCLUSION: These observations suggest an association between hepatitis C virus infection and polymyositis. Because corticosteroids can induce adverse effects in the liver, intravenous gamma globulins could be used for the treatment of this particular form of polymyositis.

Antiviral Agents↗
PubMed1998

[Cardiac manifestations of polymyositis. Apropos of 2 Senegalese cases].

INTRODUCTION: Polymyositis cardiac involvement varies between 37% and 70%. EXEGENESIS: The authors report two cases of polymyositis with myocardial involvement observed in Senegal; the first case is a cardiac failure revealing an acute polymyositis occurring in a 44-year-old woman; the second case is a 34-year-old woman who had polymyositis with tachycardia and dyspnea: in the two cases echocardiography showed a left ventricular concentric hypertrophy with preserved systolic function and altered diastolic function; clinical and echocardiographic resolution were obtained by corticosteroid medication. CONCLUSION: Myocardial localization is the most common polymyositis cardiac involvement; clinical symptomatology is rare (3.3% to 6%). sometimes revealing polymyositis; electrocardiographic abnormalities are found in 18% to 81% cases and echographic abnormality in 42% cases; cardiac involvement is of bad prognosis.

Acute Disease↗
PubMed1997

Toxoplasmic polymyositis revisited: case report and review of literature.

Toxoplasmosis can cause polymyositis either by reactivation or by recent infection. Inconsistent response to antiprotozoal therapy has been the strongest argument against toxoplasmic polymyositis as a separate entity. We report a biopsy-proven case of toxoplasmic polymyositis in a cardiac transplant patient presenting with a severe proximal weakness, myopathic, electromyographic changes and ten-fold increase of anti-Toxoplasma antibodies. An early antiprotozoal therapy and plasmapheresis led to recovery. A review of previously reported cases of toxoplasmic polymyositis suggests that an early antiprotozoal therapy is the most important variable affecting the outcome of this disease. We propose that toxoplasmic polymyositis has two phases: acute, responsive to antiprotozoal therapy, and chronic, manifested by altered immune response requiring steroids. We suggest that all patients presenting with polymyositis should have serological tests for toxoplasmosis as a part of their initial evaluation and an early trial of antiprotozoal therapy in case of positive findings.

Aged↗
PubMed2001

[A case of polymyositis complicated with pulmonary tuberculosis concerned with steroid resistance due to rifampin administration].

A 63-year-old woman in whom polymyositis had been diagnosed since 1994 was treated with ten mg of prednisolone. In 1998, she contracted pulmonary tuberculosis simultaneously with acute exacerbation of the polymyositis. The polymyositis was then treated with 40 mg of prednisolone, and the pulmonary tuberculosis, with isoniazid (INH), rifampin (RFP) and ethambutol (EB). However, the polymyositis was worsening, possibly because the administration of RFP had induced steroid resistance. Because of this, a large amount of steroid was considered necessary for the treatment of the polymyositis (PSL 80 mg/day). An additional administration of cyclosporine (CyA) made possible a decrease in the amount of steroid given. RFP has a potency to induce the enzyme p450, which has the effect of metabolizing steroids or CyA, thus shortening the half-life of these agents. When a case of polymyositis is superimposed with pulmonary tuberculosis, it should be remembered that RFP may shorten the half-life of any steroid or CyA that is administered as treatment.

Antibiotics, Antitubercular↗
PubMed2005

[The practice guideline 'Dermatomyositis, polymyositis and sporadic inclusion body myositis'].

This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a skin biopsy is advisable. A muscle biopsy is indicated when other examinations are inconclusive and the musculature is involved. The working group considers screening for cancer to be required in adults with dermatomyositis and presents recommendations for the way that this should be done. At least one-third of all patients with polymyositis has, or will develop, an associated inflammatory connective tissue disease. If a patient with a connective tissue disease develops symmetrical, proximal muscle weakness in the course of weeks or months, this may be assumed to be due to polymyositis. In the absence ofpre-existing connective tissue disease, demonstration of a mononuclear cell infiltrate in muscle tissue is a prerequisite for the diagnosis ofpolymyositis. The histopathology of muscle tissue is used as the gold standard for the diagnosis of sIBM. The practice guideline presents criteria for the concept 'activity' of myositis. Disease activity serves as a guideline for the treatment of polymyositis and dermatomyositis. The treatment of choice for dermatomyositis and polymyositis is high-dose prednisone. Physical activity does not have a negative effect on the course of these diseases. The long-term prognosis ofdermatomyositis and polymyositis is not well known. The clinical course of sIBM is slowly progressive.

Anti-Inflammatory Agents↗
PubMed1991

Immunocytochemical and virological characteristics of HIV-associated inflammatory myopathies: similarities with seronegative polymyositis.

We performed an immunoperoxidase study on muscle biopsy specimens from 19 patients with polymyositis who were seropositive for human immunodeficiency virus (HIV) (21 specimens) and 5 HIV-seronegative patients with polymyositis and compared the findings. A quantitative analysis of T cells and T-cell subsets, B cells, natural killer cells, interleukin-2 receptor-positive cells, and macrophages was performed on serial sections from all the specimens. Localization of major histocompatibility complex (MHC)-I and -II antigens, alpha and gamma interferon, and HIV antigens (p24, gp120, and gp41) was performed using specific antisera. In specimens from HIV-positive and seronegative patients, the predominant cell population was CD8+ cells and macrophages invading or surrounding healthy muscle fibers that expressed MHC-I antigen on their surface. The endomysial infiltrates in specimens from HIV-positive patients differed from those seen in specimens from the seronegative patients only by a significant reduction of the CD4+ cells (12.6 +/- 3.2% versus 21.1 +/- 4.2%). HIV antigens were seen in occasional interstitial mononuclear cells (but not in muscle fibers) in 6 of the 21 specimens from HIV-positive patients. Interferon was not localized. We conclude that the development of HIV-associated polymyositis does not appear to be related to direct infection of the muscle fibers by HIV but rather is due to a T-cell-mediated and MHC-I-restricted cytotoxic process, perhaps triggered by HIV. Because this immunopathological mechanism is common in both HIV-associated polymyositis and polymyositis alone, it is suggested that viruses may also be responsible in triggering polymyositis.

Adolescent↗
PubMed1991

Polymyositis mediated by T lymphocytes that express the gamma/delta receptor.

BACKGROUND: The invasion and destruction of nonnecrotic muscle fibers by CD8+ cytotoxic T cells is considered a hallmark of polymyositis. In the cases of polymyositis reported so far, the autoinvasive CD8+ T cells expressed the common form of T-cell receptor for the recognition of antigen, the so-called alpha/beta T-cell receptor. We describe a 69-year-old man with polymyositis mediated by CD4-, CD8- T cells expressing the recently discovered, uncommon gamma/delta T-cell receptor. METHODS: We used immunofluorescence or immunoperoxidase techniques to study frozen sections of muscle from our patient, who had mild weakness of cervical and proximal limb muscles, and from control patients with polymyositis, inclusion-body myositis, dermatomyositis, or granulomatous myopathy with monoclonal antibodies against T-cell-related antigens (CD2, CD3, CD4, CD8, and gamma/delta T-cell receptor), B cells (CD22), major histocompatibility complex (MHC) and MHC-related antigens (MHC Class I, CD1a, CD1b, and CD1c), and the 65-kd heat-shock protein. The membrane contacts between the autoinvasive cells and the sarcolemma were investigated by electron microscopy. RESULTS: In the patient described here, but not in 28 others with inflammatory myopathies, myriad gamma/delta T cells surrounded and invaded nonnecrotic muscle fibers. All muscle fibers were highly reactive for MHC Class I antigen and the 65-kd heat-shock protein. Treatment with prednisone improved the clinical and histologic findings. CONCLUSIONS: Polymyositis can be mediated by gamma/delta T cells. This new form of polymyositis appears to be highly responsive to steroids.

Aged↗
PubMed1992

Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study.

BACKGROUND: An association between polymyositis and cancer was first proposed in 1916, but the existence of the association has been disputed. An association between dermatomyositis and cancer is better accepted, but its magnitude is not known. METHODS: We undertook a study to provide accurate estimates of the risk of cancer in patients with dermatomyositis or polymyositis. We studied the incidence of cancer and the rate of mortality from cancer in a population-based cohort of 788 patients with dermatomyositis or polymyositis in Sweden from 1963 through 1983. The results were compared with those for the general population. RESULTS: Among the 396 patients with polymyositis, 42 cancers were diagnosed at the same time or after polymyositis was diagnosed in 37 patients (9 percent). The relative risk of cancer was 1.8 (95 percent confidence interval, 1.1 to 2.7) in the male patients and 1.7 (95 percent confidence interval, 1.0 to 2.5) in the female patients. Eighty-four males and 85 females died, and in 24 of these cases (14 percent) cancer was the principal cause of death. The mortality ratio (the rate of mortality from cancer in these patients as compared with that in the general population) was 0.90 (95 percent confidence interval, 0.6 to 1.4). Among the 392 patients with dermatomyositis, 61 cancers were diagnosed at the same time or after dermatomyositis was diagnosed in 59 patients (15 percent). The relative risk of cancer was 2.4 (95 percent confidence interval, 1.6 to 3.6) in the male patients and 3.4 (95 percent confidence interval, 2.4 to 4.7) in the female patients. Fifty-seven males and 110 females died, and in 67 of these cases (40 percent) cancer was the principal cause of death (mortality ratio, 3.8; 95 percent confidence interval, 2.9 to 4.8). CONCLUSIONS: The risk of cancer is increased in patients with polymyositis or dermatomyositis. In patients with dermatomyositis there is also a higher rate of mortality from cancer.

Confidence Intervals↗
PubMed1976

An electrophysiological investigation of skeletal muscles in polymyositis.

An electrophysiological study has been made of the extensor digitorum brevis, thenar and hypothenar muscles in 25 patients with chronic and acute polymyositis. It was found a reduction of the number of functioning motor units in some patients with chronic polymyositis and only in one of those affected by acute polymyositis and only in one of those affected by acute polymyositis. The sizes of the surviving units suggested that the results could be explained in terms of a primary muscle involvement mainly in acute polymyositis, while in chronic polymyositis a combination of primary and neurogenic involvement of muscle fibers might take place.

Acute Disease↗
PubMed2003

S-protein is expressed in necrotic fibers in Duchenne muscular dystrophy and polymyositis.

Complement regulatory proteins (CD55, CD59) and a fluid-phase complement regulator (S-protein) expression and distribution were studied in Duchenne muscular dystrophy (DMD) and polymyositis by Western blots and immunocytochemistry. In muscle samples from control subjects, no specific signal was detected for CD55 or S-protein, and CD59 was present on the sarcolemma of the muscle fibers. In DMD and polymyositis, Western blots demonstrated a 18-20 kDa band corresponding to CD59, as well as a signal corresponding to S-protein. Immunocytochemistry showed a colocalization between complement membrane attack complex (MAC), a molecule previously demonstrated in necrotic muscle fibers in DMD and polymyositis, and S-protein in necrotic fibers of DMD and polymyositis. Necrotic muscle fibers were more numerous in muscle biopsies of DMD patients with stronger signals for S-protein in Western blots. These results suggest that S-protein is not able to prevent the full assembly of MAC in necrotic fibers of patients with DMD and polymyositis, but might instead inactivate MAC deposits present inside necrotic fibers or participate in the clearance of MAC-attacked muscle fibers.

Antibodies, Monoclonal↗
PubMed1993

Immunohistochemical characteristics of polymyositis in patients with HTLV-I-associated myelopathy and HTLV-I carriers.

The immunohistochemical characteristics of polymyositis in patients with HTLV-I-associated myelopathy (HAM) and HTLV-I carriers were studied. Infiltrating cells were predominantly T cells and were not different from in the control group. All specimens contained positively staining muscle fibers for MHC class I antigens, but class II antigens were also expressed in some muscle fibers in 1 patient with HTLV-I-negative polymyositis, 3 patients with HTLV-I-positive polymyositis, and all 5 HAM patients with polymyositis. Expression of the neural cell adhesion molecule was lowest in the HAM patients. These findings may suggest a different immune environment in polymyositis with HAM.

Antigens, CD↗
PubMed1994

Polymyositis associated with chronic myelogenous leukemia.

A 53-year-old man with chronic myelogenous leukemia developed progressive proximal muscle weakness with electromyographic and histologic features consistent with polymyositis. Although the association of polymyositis with solid tumors is well recognized, an association with hematologic malignancies has not been firmly established. A survey of the world medical literature reveals one previously reported case of polymyositis and one of dermatomyositis associated with chronic myelogenous leukemia. We conclude that polymyositis does occur in association with chronic myelogenous leukemia. Recognition of this association is important, since treatment of polymyositis can be successful.

Electromyography↗
PubMed2006

Myopathy including polymyositis: a likely class adverse effect of proton pump inhibitors?

OBJECTIVE: Polymyositis occurring in patients treated with omeprazole has been signalled as a possible adverse drug reaction (ADR) by the New Zealand Intensive Medicines Monitoring Programme (IMMP) and the WHO Collaborating Centre for International Drug Monitoring: the Uppsala Monitoring Centre (UMC). Polymyositis and other myopathies have also been reported in post-marketing data and in the medical literature in association with proton pump inhibitor (PPI) use. We wished to follow-up these signals and investigate the evidence of causality for the association of polymyositis and other myopathy with PPI use. METHODS: Spontaneously reported ADRs from national monitoring centres are sent to the WHO ADR database (VigiBase). VigiBase was searched for case reports of the PPIs, omeprazole, pantoprazole, lansoprazole, esomeprazole and rabeprazole, with terms indicative of myopathy, and further information was elicited from the national centres to help establish causality. Literature sources were reviewed for the occurrence of the above terms in combination with PPIs. RESULTS: In total, there were 292 reports of various myopathies with PPIs, excluding 868 cases of 'myalgia'. In this analysis, 69 patients recovered when the drug was withdrawn and, in 15 patients, the reaction re-occurred when the drug was reinstated. In one-third of the 292 cases, the PPI was the single administered drug, and the PPI was the single suspected drug by the reporter in 57% of reports where concomitant medication was used. In this analysis, three index cases are documented. One involves the same patient taking three different PPIs (lansoprazole, esomeprazole and rabeprazole) at different time periods, with myalgia and muscle weakness occurring with all three drugs. In the two other index cases, myopathies with esomeprazole and omeprazole were reported with positive rechallenge, and causality was assessed as 'possible' and 'certain' by the reporting centres. In 27 cases myositis or polymyositis was reported. Other myopathies were reported, including 35 cases with rhabdomyolysis. In 9 of these cases, the PPI was withdrawn and the reaction abated. The PPI was reinstated in one patient, but the reaction did not re-occur. Time to onset was given in 17 of the rhabdomyolysis cases, rhabdomyolysis occurred with the first week in 9 cases, and in 3 cases the reaction occurred between 14 days to 3 months of treatment. In 12 of these patients, an HMG-CoA reductase inhibitor (statin) was taken concomitantly. CONCLUSION: Case reports from the WHO ADR database, including index cases involving four out of five PPIs, along with evidence of a possible mechanism, provide compelling evidence that there is a causal association between members of the PPI drug class and myopathy including polymyositis. Evidence was also obtained to support the view that PPI use may be associated with occurrence of other myopathies, including the serious reaction rhabdomyolysis.

2-Pyridinylmethylsulfinylbenzimidazoles↗
PubMed2002

Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study.

BACKGROUND: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear. PATIENTS AND METHOD: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer. RESULTS: The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease. CONCLUSION: Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis.

Adult↗
PubMed1996

Polymyositis as a manifestation of chronic graft-versus-host disease.

A syndrome indistinguishable from idiopathic polymyositis occurred in 11 patients as a manifestation of chronic GVHD. All patients had elevation of creatine phosphokinase (CPK). Immunohistology demonstrated the effector cells in the muscle infiltrates as cytotoxic T cells, a finding similar to idiopathic polymyositis. Polymyositis is a rarely reported complication of chronic graft-versus-host disease (GVHD) with only 8 cases described in the literature. We encountered this syndrome in a small but significant percentage of our patients with chronic GVHD. Polymyositis associated with chronic GVHD does not affect the overall prognosis for the patient. Moreover, polymyositis can be the only manifestation of chronic GVHD. Awareness of this complication is important because it can be confused with other causes of muscle weakness after bone marrow transplantation. Finally, prompt initiation of corticosteroid therapy results in a rapid improvement of the associated symptoms.

Adolescent↗