Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a 37-year-old woman with slowly developing muscular weakness for 2 years following insidious stiffness of calf muscle. Serum CK was elevated up to 4,207 IU/l. She presented sleepiness, weakness of proximal and truncal muscles and systemic muscular atrophy. While she had not experienced dyspnea, her arterial blood gas analysis revealed extreme hypoxia and hypercapnea due to weakness of respiratory muscles. Echocardiogram showed thinness and hypokinesis of left ventricular wall, and arrhythmia was pointed out by holter ECG. Needle elctromyogram of the proximal muscles exhibited polyphasic units with low amplitude. Muscle biopsy showed degeneration and necrosis of muscle fibers as well as regeneration. Mild infiltration of inflammatory cells was shown. Serological examination showed positive antimitochondrial M2 antibody, especially specific for primary biliary chirrhosis (PBC). She was diagnosed as chronic myositis associated with PBC. Four cases of idiopathic myositis with severe weakness of respiratory muscle, associated with PBC had been reported. These cases and our present case share the similar feature in respect of insidious or chronic course and resistance to therapy. In our present patient, respiration had been supported by BiPAP and she has been successfully improving slowly by oral steroid following 4 courses of methylprednisolone pulse therapy.
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PMID:[A case with severe respiratory muscle weakness due to chronic myositis associated with PBC]. 1528 10

Shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus (SLE) characterized by unexplained dyspnea, a restrictive pattern on pulmonary function tests, and an elevated hemidiaphragm. A total of 59 cases are reported in literature including the current case. The mean age of these patients is 36.85 years (range, 15-61 years), and the female-to-male ratio is 6:1. This disorder is seen primarily during the later stages of SLE. The most common presenting features include dyspnea and pleuritic chest pain. Myositis has been reported in only 8 of 59 patients (13%). Diagnosis is made with chest x-ray showing an elevated hemidiaphragm and a restrictive pattern on pulmonary function testing without any evidence of interstitial lung disease along with decreased transdiaphragmatic pressure (Pdi). Corticosteroids are the most common method of treatment. Immunosuppressive therapy, beta-agonists, and theophylline are used in those resistant to steroids. The prognosis is generally good. This article reports the case of a 22-year-old man presenting with a 7-month history of dry mouth and dry eyes accompanied by increasing difficulty in breathing, progressing to dyspnea at rest. The patient's history included bilateral parotid gland swelling and nephrotic syndrome diagnosed 4 years earlier. Pertinent physical and laboratory findings included positive Schirmer's test results; bilateral parotid gland enlargement; bibasilar lung crackles; synovitis of the second and third proximal interphalangeal joints; a positive antinuclear antibody (Ro/SSA), Sm, and anticardiolipin antibodies; and elevated right hemidiaphragm on chest x-ray. Pulmonary function tests demonstrated restrictive lung disease with normal high-resolution computerized axial tomography. A dramatic response to oral prednisone (60 mg daily) was observed in all of the patient's complaints in a matter of several days. A diagnosis of SLE with secondary Sjogren's syndrome (SS) and SLS was made. Although SLS has been reported in association with SLE, there has been only one previous report of SLS in SLE/SS overlap syndrome. Early recognition with appropriate treatment can decrease the morbidity associated with this rare syndrome.
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PMID:Shrinking lung syndrome in systemic lupus erythematosus and Sjogren's syndrome. 1704 5

Respiratory failure from causes exclusive of intrinsic lung disease is rare in systemic sclerosis. We report an unusual case of a young woman with diffuse systemic sclerosis who presented with proximal muscle weakness, dyspnea, weight loss, and nasal regurgitation. On physical examination, she had normal breath sounds but severe limitation of chest wall expansion. Pulmonary function tests (PFTs) were consistent with restrictive lung disease. Pulmonary pressures were normal on right heart catheterization and chest radiography and high-resolution computed tomography showed no evidence of intrinsic lung disease. Quadriceps muscle biopsy was consistent with type II atrophy, without any element of inflammatory myositis. After a meal, the patient aspirated, developed severe persistent hypercapnia, and required ventilatory support. Therapy with corticosteroids and intravenous gamma globulin failed to improve her condition, and the patient subsequently expired. Hypercapnic respiratory failure on the basis of chest wall involvement from systemic sclerosis may occur in the absence of intrinsic lung disease, and the prognosis, as in this case, may be grave.
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PMID:Hypercapnic respiratory failure in systemic sclerosis. 1704 22

The lungs are frequently involved in systemic sclerosis ('scleroderma'), a rare, disabling disease of unknown origin, characterised by skin thickening and Raynaud's phenomenon. The pathogenesis of scleroderma is complex, but signs and symptoms of excessive fibrosis, vasculopathy and inflammation are almost universally present. Dyspnoea in scleroderma patients can be due to chest wall tightening from skin thickening, pleural disease, cardiac involvement, myositis of intercostal muscles, or so-called scleroderma lung disease. Scleroderma lung disease encompasses vascular (pulmonary artery hypertension) or interstitial lung disease, or both. A comprehensive work-up is required to delineate the underlying cause of dyspnoea in a scleroderma patient, and to establish the contribution of each component to the symptoms. This should include a 6-minute walk test, pulmonary function testing, high-resolution thoracic CT scanning, ECG, echocardiography and, if pulmonary artery hypertension is suspected, right-heart catheterisation; bronchoalveolar lavage is optional. Lung disease in scleroderma contributes significantly to excess morbidity and early mortality, especially when diffusion capacity drops below 40% and/or forced vital capacity below 50%. However, recent clinical studies have unequivocally demonstrated that scleroderma lung disease is amenable to treatment with new vasodilatory drugs that target specific pathways involved in vasoconstriction, or with cyclophosphamide for interstitial lung disease. Uncontrolled studies have suggested that these therapies also have an impact on survival, but controlled studies with a long follow-up are needed to corroborate this point.
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PMID:Scleroderma lung: pathogenesis, evaluation and current therapy. 1748 44

A 74-year old female patient suffered from dyspnoea, subfebrile body temperature, myalgias, arthralgias and presented a transitory skin rash. Further findings were an eosinophilia, synovitis, a myositis and pulmonary fibrosis. There was no evidence for a malignancy or an infectious disease. We diagnosed an Anti-Jo-1-Syndrome with a secondary Sjoegren syndrome. Immunsuppressive therapy with steroids, later in addition methotrexate led to improvement of the patient's clinical condition. Above mentioned symptoms and its differential diagnosis are discussed in this article.
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PMID:[Myositis with pulmonary fibrosis and eosinophilia]. 1772 58

Respiratory muscle involvement is a rarely reported manifestation of inflammatory myopathies. We report the case of a 38-year-old woman with antisynthetase syndrome who presented with inflammatory myopathy and acute pulmonary interstitial involvement that initially improved with corticosteroids and immunoglobulins. A few months later dyspnoea resumed despite the absence of evidence of interstitial deterioration or other common diagnosis including vascular or infectious disorders. Isolated respiratory muscular involvement was evidenced by functional testing. Disease course was favourable with corticosteroids and immunoglobulins. Inflammatory myopathy of the antisynthetases syndrome could be limited or mainly expressed to respiratory muscles. Unexplained dyspnoea should call in mind a muscular respiratory myositis.
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PMID:[Inspiratory muscle myopathy and antisynthetase syndrome]. 1803 54

A 51-year old man with severe acute respiratory failure was referred to our hospital. He presented with cyanotic digits, high fever and dyspnea. The chest radiograph and chest CT scan showed diffuse ground-glass-opacities and reticular shadows in both lung fields. We diagnosed acute interstitial pneumonia. The pulmonary infiltration seen on chest radiograph diminished gradually after pulse methylprednisolone and cyclophosphamide therapy. Later, we screened his sera for antibodies to anti-aminoacyl tRNA synthetase to diagnose anti-aminoacyl tRNA synthetase syndrome because anti-PL-12 antibodies were positive. This case has been followed but no myositis or recurrence of interstitial pneumonia has been recognized.
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PMID:[A case of rapidly progressive interstitial pneumonia with anti-pL-12-antibodies successfully treated by pulse methylprednisolone and cyclophosphamide]. 1870 May 73

A 61-year-old Caucasian was admitted to Department of Chest Diseases and Tuberculosis, Medical University of Bialystok, Poland for progressive muscle weakness and weight loss. Eighteen months prior to admission, the patient had been diagnosed with pulmonary embolism. At that point he was started on Enoxaparin QD. Past medical history was unremarkable. In the interim, the patient developed fever, myalgia and progressive dyspnea. Physical examination on admission revealed a rash on his upper torso and back, and the extensor surfaces of all four extremities. Laboratory values included CPK 8229, MB fraction 219, LDH 981. Chest X-ray and CT scan revealed bilateral patchy consolidations and ground-glass opacities. EMG was consistent with myositis. The patient was started on solumedrol 40 mg i.v., b.i.d., and then switched to prednisone 40 mg b.i.d. His symptoms and muscle strength improved remarkably. The patient was discharged with prednisone with an outpatient follow up.
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PMID:Dermatomyositis masquerading as pulmonary embolism. 2015 49

Systemic sclerosis (SS) can involve the pericardium, myocardium, conduction system, and cardiac valves. The presence of overt clinical signs of cardiac disease is a poor prognostic sign. Clinical manifestations include dyspnea, palpitations, chest pain, syncope, and symptoms of right heart failure. Prevalence of clinically symptomatic pericardial disease is 5-16%. However, ecocardiographic prevalence is 5.4- 41% and at autopsy is 33-77.5%. Patchy fibrosis is the characteristic myocardial finding in SS. Contraction band necrosis is the typical pathological finding. Important complications of fibrosis include left ventricular hypertrophy, as well as systolic and diastolic dysfunction of both ventricles. Early detection of these abnormalities is very important, mainly of the diastolic dysfunction, since it occurs before the systolic dysfunction and can predict important cardiac damage. Association of skeletal myositis with myocardial disease has been described. Patients with skeletal myositis are more likely to develop congestive heart failure, sustained symptomatic arrythmias, and cardiac sudden death. Coronary arteries are normal in systemic sclerosis, but there is no endomyocardial vessel involvement. There is an increased prevalence of arrhytmias, mainly premature atrial and ventricular contractions, as well as conduction system disease. Cardiac valvular involvement is minor in systemic sclerosis; mitral valve is the most frequently affected. Other abnormalities described in this disease include peripheral large vessels stiffness and secondary cardiac involvement due to pulmonary and systemic arterial hypertension. Cardiac involvement confers a high morbi-mortality risk in systemic sclerosis.
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PMID:[Cardiac involvement in systemic sclerosis]. 2179 85

A 46-year-old patient was frequently seen with a medically treated Anti-Jo-1 syndrome. The patient had already been treated with azathioprine and oral corticosteroids on account of decreasing lung function, dyspnoea, fatigue, and beginning signs of myositis. Although high doses of steroids and azathioprine were administered, the muscleskeletal syndromes increased steadily. The patient used to be an active long-distance runner (20 km), but now was unable to perform that kind of physical exercise. It was decided to start a treatment with the GalileoTM training device for active muscle training of the lower extremities. Before and after three months of training the following assessment was performed: measurement of health-related quality of life (St. Georges respiratory questionnaire, SGRQ), ultrasound measurement of the cross-sectional area of the quadriceps muscle, 6 minute walk test (6 MWT), lung function testing, and assessment of serum markers of inflammation (TNF-alpha, interleukin-8, CRP, CK, myoglobin). After only two months, training with the GalileoTM five times a week has improved the patient's conditions dramatically. The training will be continued.
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PMID:[Special training therapy to reduce inflammation in Anti-Jo-1 syndrome]. 2186 91


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