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Inclusion body myositis (IBM). Morphological study.

Among the chronic idiopathic inflammatory myopathies inclusion body myositis (IBM) has emerged as a clinicopathologic variant. Slowly progressive weakness of the distal and the proximal muscle groups, the presence of rimmed vacuoles with basophilic granules as well as 15-18-nm filamentous inclusions in affected muscle confirm the clinical and histopathological distinction between inclusion body myositis and chronic polymyositis.

Diagnosis, Differential↗

[Two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis].

We present two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis. The first patient was a 38-year-old woman and the second, a 59-year-old woman. Both patients were admitted to our hospital complaining of dry cough and dyspnea on effort. The diagnosis of interstitial pneumonia was made from chest radiography, computed tomography and surgical lung biopsy. Anti-Jo-1 antibodies, which were highly specific for polymyositis and dermatomyositis (PM/DM), were detected in both patients. However, the serum creatine kinase concentrations and electromyographic findings in both patients were normal, and no clinical signs (including muscle weakness, rash and arthralgia) were found. In the first patient, oral prednisolone (PSL) treatment (20 mg day) improved the interstitial pneumonia, but PSL has now been tapered to 17.5 mg day. In the second patient, oral PSL treatment (40 mg day) improved interstitial pneumonia, and the dose was tapered to 5 mg day. The second patient was followed for more than 10 years after treatment, but she has never shown any signs of clinical myositis. Further investigation will be required, because no pathophysiological relation between anti-Jo-1 antibodies and interstitial pneumonia with PM DM has yet been established.

Adult↗

Myositis ossificans traumatica.

Myositis ossificans is a benign condition of heterotopic bone formation. It is a generalized term for four separate clinical entities. Although not a rare disease process, diagnosis often remains difficult. This article reviews the various forms of myositis ossificans, as well as the pathology, diagnostic tests, and treatment options.

Adolescent↗

[Polymyositis, dermatomyositis and inclusion body myositis, nosological aspects].

THREE GROUPS OF PRIMARY INFLAMMATORY MUSCLE DISEASES: The primary inflammatory muscle diseases comprise three main subsets: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). PM and DM are characterized by a proximal weakness that develops along weeks to months and by elevated creatine phosphokinase levels. Cutaneous involvement including both erythema and edema and infantile or adult onset are DM specific. PM and IBM only concern adults. Several PM/DM manifestations must be searched for because of their severity: swallowing disorders, various mechanisms of respiratory dysfunction (swallowing pneumopathies, interstitial lung disease, respiratory muscle deficiency) and cardiac involvement. DIAGNOSTIC ELEMENTS FOR PM AND DM: Two investigations, beside biopsy, are particularly useful: muscle MRI imaging showing inflammatory pattern and specific detection of antisynthetase autoantibodies (PM/DM with interstitial lung disease) and anti-Mi-1 and 2 in DM. PHYSIOPATHOLOGICAL DATA: PM and DM differ in their histological and physiopathological characteristics: perivascular B and CD4 lymphocyte infiltrates and complement deposits at the origin of humoral induced vascular disease in DM and perimysial CD8 lymphocytes inducing a cellular mediated cytotoxic injury in PM. Class I HLA antigen expression on the muscle fibers and production of cytokines play a crucial role in the pathogenesis of these two diseases. PM and DM may be associated with cancers, connective-tissue disease (overlap syndrome). Some PM are secondary to HIV, HTLV1 virus and toxoplasmosis infection. CHARACTERISTICS OF INCLUSION BODY MYOSITIS: IBM, the most frequent acquired myopathy after 50 years of age, is characterized by particular features: not only clinical (late onset, selective weakness, early distal involvement, slow course, unresponsiveness to corticosteroid and immunosuppressant agents); but also histological (rimmed vacuoles, filamentous inclusions) and pathogenic (cytotoxic and degenerative inflammatory process, similar to Alzheimer's disease, with beta-amyloid protein accumulation).

Adult↗

[Focal myositis].

Focal myositis is a rare benign pseudotumor of skeletal muscle, of unknown cause. Clinically it presents as an enlarging mass within a muscle, usually in en extremity. Biopsy shows characteristic histological changes of inflammation, focal degeneration and regeneration. The natural course of this disorder is spontaneous regression. This report describes the case of a 17-year-old boy with focal myositis of the thigh and a review of the relevant literature.

Adolescent↗

Paucity of anti-hepatitis C virus antibodies in the serum of Indian patients with Sjogren's syndrome and inflammatory myositis.

Several extrahepatic manifestations have been associated with infection with Hepatitis C virus (HCV) infection. It has been associated with Sjogren's syndrome (SS) and inflammatory myositis (IM). The objective was to look at the prevalence of anti-HCV antibodies in the serum of SS and IM patients of Indian origin. Individuals satisfying the European Economic Community criteria for the diagnosis of SS and those satisfying the criteria of Bohan and Peter for the diagnosis of IM were recruited in the study. Routine evaluation for liver functions was made. Anti-HCV antibodies were tested by a third generation ELISA, using microplate HCV3.0 ELISA. Of the 23 patients with SS studied, 14 had extraglandular features. The commonest were anaemia and arthritis in six each, followed by in lymphopenia in two. One patient each had interstitial lung disease, hypothyroidism and chronic active hepatitis. Twenty-two patients with IM were studied alongside. None of the patients had abnormal liver functions. One patient with primary SS tested positive for anti-HCV antibodies. None of the patients with inflammatory myositis tested positive for anti-HCV antibodies. The presence of anti-HCV antibodies in our cohort of patients with SS and IM is low and more in keeping with the generally low prevalence of the infection in the Indian population.

Adolescent↗

[A case with severe respiratory muscle weakness due to chronic myositis associated with PBC].

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.

Adult↗

[Inclusion body myositis after interferon-alpha treatment in a patient with HCV and HTLV-1 infection].

We report the first case of inclusion body myositis (IBM) which occurred after interferon-alpha treatment for chronic hepatitis C. A 63-year-old man contracted hepatitis C virus (HCV) and human T cell leukemia virus type 1 (HTLV-1) from a blood transfusion at age of 18. At age 57, he was treated with interferon-alpha (IFN alpha) for chronic hepatitis C. A month later, he developed muscle weakness in the proximal part of his lower extremities. IBM was diagnosed after a muscle biopsy at age 62. Steroid therapy improved his muscle power. One year later, worsening of his hepatic condition required re-administration of IFN alpha after gradual decrease and discontinuation of prednisolone. However, several days later, he rapidly became weaker and required a cane to walk. Elevated serum creatine kinase (2,199IU/L) and abnormal intensity in his MRI of thigh were demonstrated. The second muscle biopsy, performed after obtaining the informed consent from our patient, confirmed relapse of IBM. His symptoms improved again after discontinuation of IFN alpha and re-induction of prednisolone. Although a few cases each of IBM associated with HCV or HTLV-1 have been reported, the pathogenesis of virus-associated inflammatory myositis has not been clearly understood. Moreover, there has been no description on IBM associated with IFN alpha treatment, though several cases of polymyositis have been reported. Our case suggests that infection of HCV and HTLV-1 may be immunologically involved in the development of IBM and that IFN alpha can be directly related to onset and relapse of IBM.

HTLV-I Infections↗

Circumscribed myositis ossificans of the iliopsoas muscle. Description of a case followed-up after 1 and 14 years.

The term myositis ossificans (MO) means a pathology characterized by the formation of heterotopic bone tissue within the connective tissue of the muscle. Two clearly differentiated forms of the disease are described: one is generalized and one is localized or circumscribed (CMO). It is the purpose of this study to describe a case of circumscribed myositis ossificans located in the iliopsoas muscle, clinically by CT scan and followed-up after 1 and 14 years.

Adult↗

[Eosinophilia-myalgia syndrome with fasciitis and interstitial myositis after L-tryptophan administration].

We describe a 53-year-old women with eosinophilia-myalgia syndrome who suddenly developed severe persistent myalgias of her arms, legs, back, and shoulder after a 5-month period of daily L-tryptophan ingestion, associated with fever, progressive stenocardia and left-sided congestive heart failure. Laboratory tests showed a leukocytosis of 11.2/nl with 3.14/nl eosinophils and an elevated erythrocyte sedimentation rate. There was a marked, predominantly proximal sclerosis of her arms, legs and trunk with a brownish discoloration. The skin of her arms and legs appeared dimpled (peau d'orange). Findings of the electrophysiological examinations were consistent with sensory neuropathy and myositis. Remarkable fasciitis and interstitial myositis were present in a biopsy specimen (from skin to muscle) taken from her thigh. However, eosinophilic infiltrates were rare. Angiography revealed an apical obstructive cardiomyopathy. In this paper, we describe the clinical findings, the course over 2 years, as well as the therapeutic management. Furthermore, the most important differential diagnoses are discussed and the literature is reviewed with special attention given to more recent pathogenic insights into this newly recognized multisystem disease.

Biopsy↗

Expression of Major Histocompatibility Complex class I and II in a case of Neospora caninum myositis in a dog.

Neosporosis was diagnosed in a 2-month-old dog by indirect immunofluorescence antibody test (IFAT) and confirmed by means of histopathology and immunohistochemistry. The associated myositis was characterized by Major Histocompatibility Complex expression on some muscle fibres. This finding indicates an immunological activation, of the muscle cells that, acquiring Major Histocompatibility Complex expression, may, in some way, contribute to antigen presentation. A possible role of these glycoproteins in the pathogenesis of Neospora-associated myositis is discussed.

Animals↗

Acute pure motor quadriplegia: is it dengue myositis?

BACKGROUND AND AIM: In view of paucity of comprehensive evaluation about dengue infection producing quadriplegia, we report the clinical, laboratory and neurophysiological studies in these patients. SUBJECTS AND METHODS: Seven out of 16 patients with dengue infection presented with quadriplegia and they were subjected to a detailed clinical history and examination. Diagnosis of dengue was based on characteristic clinical and positive serum IgM ELISA. Blood counts, serum chemistry, CSF analysis and nerve conduction and electromyographic (EMG) studies were performed in all. Outcome was defined at the end of 1 month into complete, partial and poor on the basis of activities of daily living RESULTS: The age of the patients ranged between 9 and 42 years and 2 were females. Fever was present in all and myalgia in 5 patients. Weakness developed within 3-5 days of illness, which was severe in 4 and moderate in 3 patients. Hypotonia and hyporeflexia were present in 5 patients. Nerve conduction and EMG studies were normal in all except one whose EMG was myopathic. Serum CPK and SGPT were raised in all and serum bilirubin in 3 patients. All the patients had coagulopathy and 6 had thrombocytopenia. Muscle biopsy in 1 patient was suggestive of myositis. Six patients improved completely and one had poor recovery who needed ventilatory support. CONCLUSION: Dengue virus infection may result in acute pure motor quadriplegia due to myositis. In an endemic area it should be considered in the differential diagnosis of acute flaccid paralysis.

Adolescent↗

Group A beta-hemolytic streptococcal glossal necrotizing myositis--case report and review.

We report the first case of glossal necrotizing myositis by group A beta-hemolytic Streptococcus in an 8-year-old girl on chronic nonsteroidal anti-inflammatory drugs, immunomodulators, and steroids for juvenile rheumatoid arthritis. Treatment included partial glossectomy and parenteral antibiotics. After a critical course, full recovery ensued. The subject of necrotizing myositis is reviewed.

Anti-Bacterial Agents↗

Myositis ossificans: pedal manifestations.

Myositis ossificans traumatica is a benign muscular lesion that is frequently reported in the orthopedic literature, but is not commonly found in the foot. This case report discusses myositis ossificans occurring in the foot. It is most likely subsequent to a heel spur injection received by the patient.

Adult↗

[A patient with serious viral myositis following flu].

A 16-year-old girl presented at the emergency unit with myalgia following a flu-like episode. Laboratory tests indicated severe rhabdomyolysis and nephritis. Autoimmune-induced myositis was excluded on the basis of negative tests for antinuclear antibodies; prednisolone treatment was discontinued 1 week later. The patient recovered gradually and was discharged with physiotherapy 2 weeks later. High positive titres of complement-binding antibody against influenza B virus were found, i.e. 1:125 and 1:250 on days to and 25 of illness, respectively. Viral myositis is an uncommon disease entity that occurs following a viral infection, especially with influenza virus, that has been experienced for the first time. It usually runs a benign course: children often present with calf tenderness that resolves within a few days. There are cases, however, with a more serious course involving severe rhabdomyolysis and acute renal failure that can be sometimes fatal.

Adolescent↗

All-trans-retinoic acid-induced myositis in a child with acute promyelocytic leukemia.

Anthracyclin-based regimens and all-transretinoic acid (ATRA, tretinoin) as differentiating agent are commonly utilized for the treatment of acute promylelocytic leukemia (APL). There are many adverse effects that may be seen during the use of ATRA in patients with APL. Of these, ATRA-induced myositis is rarely described in adults and rare in the children with APL. Herein, we report an 11-year-old girl with APL who developed ATRA-induced myositis during induction treatment.

Adult↗

[Myositis as a 'radiation-recall phenomenon' following palliative chemotherapy with carboplatin-gemcitabin for non-small-cell pulmonary carcinoma].

A 58-year-old woman suffered pain due to a stage-IV adenocarcinoma of the right upper-lung lobe with signs of growth into the right axilla. Palliative local radiotherapy directed at the primary tumour followed by combination chemotherapy with carboplatin-gemcitabin led to partial remission. During the fourth and last cycle of her three-months course of chemotherapy, the patient again complained of pain in the right half of the thorax; later, a local swelling of the pectoralis muscles was found. She was diagnosed with myositis as a delayed reaction to irradiation (radiation-recall phenomenon) based on the clinical symptoms (redness of the skin, pain and distinct swelling) but especially on the basis of a CT-scan. This revealed a clear swelling of the pectoralis muscles, even though the tumour was in remission. The possibility of myositis as a radiation-recall phenomenon should be borne in mind whenever a patient presents with skin changes or pain within a previous irradiation field during chemotherapy with, for example, gemcitabin.

Antineoplastic Agents↗

Detection of parvovirus B19 in a case of erythema infectiosum with myositis.

A well documented case of erythema infectiosum is being reported here for the first time from India which was associated with myositis that has not been reported globally. A 9-year-old child presented with moderate to high grade fever, mild anemia, and erythematous rash involving face, trunks and limbs associated with arthralgia, myalgia and myositis. Parvovirus B19 infection was confirmed by detection of IgM antibodies (inhouse ELISA) and DNA (nested PCR) in patient's serum.

Child↗