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Does Toxoplasma cause polymyositis? Report of a case of polymyositis associated with toxoplasmosis and a critical review of the literature.

We report here a case of polymyositis and toxoplasmosis, and review the previous examples of this association. We suggest that in most cases this relationship is due to reactivation of latent infection in an immunocompromised host. Gross immunological aberrations underline the pathogenesis of polymyositis and these predispose the patient to the development of toxoplasmosis. Anti-protozoal therapy is necessary and produces some clinical benefit, but it does not cure the polymyositis.

Adult

Fluorescent antibodies in polymyositis using cultured human skin fibroblasts: granular perinuclear cytoplasmic staining pattern by sera from patients with polymyositis and pulmonary fibrosis.

Immunofluorescent antibodies were studied in sera from 48 patients with polymyositis (PM) and 149 control subjects using cultured human skin fibroblasts as substrate. Forty-six (95.8%) of PM sera showed a positive reaction. Among them, a characteristic granular perinuclear cytoplasmic staining was seen in 8 sera, including 4 with anti-Jo-1. Four of these 8 sera showed positive staining on HEp-2 cells with the same pattern. All patients showing this pattern of staining had pulmonary fibrosis which was not seen in control patients. Consequently, this staining pattern might be a specific marker for a subset of patients with PM with pulmonary fibrosis.

Adult

[Vascular manifestations of dermatomyositis and polymyositis. Clinical, capillaroscopic and histological aspects].

Polymyositis is characterized by a T-cell-mediated and MHC-I-restricted cytotoxic process, whereas dermatomyositis is a primitively vascular disease with microangiopathy mediated by the complement C5b-9 membranolytic attack complex. We have tried to estimate the frequency of vascular abnormalities in polymyositis as defined by Bohan and Peter. We have retrospectively studied 17 patients with dermatomyositis and 15 patients with polymyositis. Vascular abnormalities have been defined by clinical, capillaroscopic and histologic (muscle biopsy and minor salivary glands biopsy) features. After clinical features, 5/17 dermatomyositis had a Raynaud's phenomenon, against 6/15 polymyositis. Digital necrosis has been observed for 2/17 dermatomyositis and 2/15 polymyositis. In capillaroscopy, 14/17 dermatomyositis had a microangiopathy with or no enlarged capillary loops, against 7/15 polymyositis. None of these polymyositis had enlarged capillary loops. The muscle biopsy showed a predominantly perivascular or perimysial inflammatory infiltrate (vascular process) for 10/16 dermatomyositis against 4/13 polymyositis; a perifascicular atrophy for 3/16 dermatomyositis against 2/13 polymyositis. The histological study of minor salivary glands, showed vascular lesions for 2/11 dermatomyositis and for 1/8 polymyositis. Finally, Bohan and Peter's classification is now inadequate to distinguish between dermatomyositis and polymyositis. Indeed, some dermatomyositis sine dermatitis, may exist and be recognized by their vascular features. To distinguish between dermatomyositis and polymyositis is important, to evaluate the risk of cancer which is more frequent in dermatomyositis.

Adult

EMG dynamics in polymyositis. A quantitative single motor unit potential study.

A multidimensional quantitative EMG analysis method with averaged motor unit potentials (MUPs) was applied to a study of different stages of polymyositis. The duration parameters had a general tendency to increase in polymyositis. The only exception was the total MUP duration, which decreased in the onset stage of acute polymyositis. The amplitude tended to decrease in acute polymyositis but increase in chronic, progressive polymyositis. The number of fast components both in the whole MUP complex and in the main MUP tended to increase in all stages of polymyositis. The mean fast component duration in the main MUP tended to decrease in polymyositis. Satellites (small signals time-locked to the main component but part of it) were numerous in active stages of polymyositis. There were significant quantitative differences in most of the parameters in different stages of polymyositis.

Adolescent

Ovarian malignancy in patients with dermatomyositis and polymyositis: a retrospective analysis of fourteen cases.

BACKGROUND: Dermatomyositis and polymyositis can be associated with an underlying malignancy. OBJECTIVE: Our purpose was to describe a group of patients with dermatomyositis and polymyositis in whom ovarian carcinoma was diagnosed and to evaluate further the characteristics of the association between these diseases. METHODS: A cross-sectional retrospective review identified 14 patients in a 45-year period (1950 to 1995) with dermatomyositis and polymyositis in whom an underlying ovarian malignancy was diagnosed. Clinical and laboratory data of these patients were reviewed, and immunofluorescent and hematoxylin-eosin-stained skin sections were examined. RESULTS: The mean age at diagnosis of dermatomyositis and polymyositis was 59 years. Of the 14 patients, 11 had dermatomyositis, two had polymyositis, and one had an overlap disease. The 12 patients with dermatomyositis had distinctive cutaneous lesions. In eight, cutaneous biopsy specimens demonstrated variable histologic changes. Thirteen patients had signs of proximal muscle weakness, and myopathy was further demonstrated by electromyographic testing in 10. Creatine kinase levels were increased in 6 of the 10 patients tested. Dysphagia was present in five patients, but esophageal dysmotility was not demonstrated in three. Dermatomyositis and polymyositis preceded the ovarian malignancy in nine patients, were concomitant with it in four, and succeeded the ovarian disease in one. CONCLUSION: Physical examination and imaging techniques failed to detect early ovarian cancer in our patients with dermatomyositis and polymyositis. When detected (usually by abdominopelvic examination, in two by computed tomographic examination), it was advanced, and survival was poor.

Adenocarcinoma

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

[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

[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

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

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

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

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

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