Acute Polymyositis Research Articles

Idiopathic polymyositis in renal transplant recipient

Introduction and aim Myositis is a rare complication following renal transplant and is most commonly the result of a drug-mediated myotoxicity, but the idiopathic cause is still the most common. After kidney transplant, the differential diagnosis of polymyositis includes autoimmune disease, drug-induced viral infections, and rhabdomyolysis associated with electrolyte imbalance. We aimed to report a case of idiopathic polymyositis in a renal transplant recipient and review the literature for similar cases. Case report A 31-year-old male patient developed polymyositis three years following live-related kidney transplantation. Electromyography confirmed myopathic changes. The clinical features and course, MRI findings, electromyography features, positive anti-MI-2 antibody, and the response to high-dose steroid therapy are matched with immune-mediated acute polymyositis, especially after excluding viral infections and drug-induced myopathy. Conclusion Acute polymyositis may occur after a kidney transplant. Possible mechanisms include viral antigen transmission or a localized graft versus host disease. Muscle biopsy is not mandatory before prompt initiation of high-dose steroid therapy, which leads to clinical and biochemical recovery.

Case Report: Hemophagocytic lymphohistiocytosis associated with acute polymyositis

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition marked by uncontrolled histiocyte proliferation and activation, and phagocytosis of normal hematopoietic cells. This disease is rare, and a concurrent presentation with acute polymyositis is rare. A 14-year-old male was admitted to Chattogram Medical College Hospital with a high-grade fever for 25 days, generalized severe body aches, and multiple large joint pain in the lower limb for the same duration. On examination, the patient was found to have splenomegaly, tenderness in both knee joints, symmetrical proximal weakness of both lower limbs, several sensory losses, and loss of bowel and bladder control. The laboratory data showed that he had anemia, thrombocytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, hypoalbuminemia, raised D-dimer, and serum creatine phosphokinase (S. CPK). Along with these, a bone marrow biopsy revealed hemophagocytic cells, and he was diagnosed with HLH with acute polymyositis (PM). The patient was treated with intravenous (IV) hydrocortisone and dexamethasone. Clinical stability was achieved with gradual improvement of initial symptoms and biochemical markers. The patient was discharged with oral steroids at a tapering dose and was advised to regularly follow-up.

Acute polymyositis presenting as chronic graft-versus-host disease: A systemic review.

e19025 Background: Chronic graft-versus-host disease (cGvHD) remains a significant complication of allogenic hematopoietic stem cell transplantation (allo-HSCT), with an estimated incidence of over 50%. Commonly targeted organs are skin, eyes, mouth, gastrointestinal tract, and liver. Muscular involvement and presentation as acute polymyositis (APM) remains a rare manifestation of cGvHD. We present a systemic review of APM associated with cGvHD to summarize current evidence regarding epidemiology, clinical presentation, diagnosis, treatment, and prognosis. Methods: A systemic review was conducting following Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. We screened 97 articles from 3 databases (PubMed, Embase, and Cochrane) using the MeSH terms and keywords for “Allogenic stem cell transplantation,” “Chronic graft-versus-host disease,” “Polymyositis” and “Myositis” from the date of inception to Jan 2021. After excluding duplicate, review and non-relevant articles, we included 36 studies (3 retrospective, others case series/reports) reporting APM associated with cGvHD after allo-HSCT. Results: We identified 72 patients, presented with APM associated with cGvHD after allo-HSCT. It involves cases of all ages, with male predominance. The onset of APM ranges as early as 100 days to 5 years, with a median range of 1.6 years post-allo-HSCT. Over half (59%) of patients had prior acute GvHD. Majority (85%) of cases presented with myalgia and progressive bilateral proximal muscle weakness with elevated Creatine phosphokinase (CPK) and/or Aldolase. Isolated presentation of APM without other manifestations of GvHD was rare, and concurrent skin involvement was present in 42% cases. Steroids remain the mainstay of treatment, achieving complete treatment response in up to 78% of cases. In some refractory cases, Rituximab has also been effective. Conclusions: APM can present as a sole manifestation of cGvHD with an estimated incidence of up to 3.4%. Diagnosis can be challenging as it can mimic idiopathic polymyositis. Radiologically targeted muscle biopsy showing characteristic myonecrosis remains the gold standard for diagnosis. Most (90%) cases respond to steroids and immunosuppression agents. Nevertheless, refractory cases remain challenging to treat and can cause significant morbidity and mortality. Characteristics of APM in 3 cohorts of allo-HSCT patients.[Table: see text]

The concurrent association of inflammatory polymyositis and Crohn’s ileo-colitis in a Sri Lankan man: a case report of a rare association and literature review

BackgroundCrohn’s disease is a relapsing, systemic inflammatory disease affecting the gastrointestinal tract with associated extraintestinal manifestations and immune disorders. Among the few cases reported, the association of Crohn’s disease with polymyositis varies in its complexity and severity. We report here the first known case of inflammatory polymyositis leading to rhabdomyolysis in a male patient diagnosed with Crohn’s ileocolitis.Case presentationA 42-year-old previously healthy man presented with acute polymyositis leading to rhabdomyolysis. The acute nature of the illness raised the suspicion of an infective, toxic, or metabolic insult, which was excluded during further investigations. Prolonged low-grade fever and raised inflammatory markers led to the suspicion of inflammatory polymyositis, which was confirmed by electromyography and muscle histology. In the absence of an infective cause, the concurrent association of prolonged diarrhea containing blood and mucous after recovery from an acute phase of myositis proved a diagnostic challenge. Ileocolonoscopy findings of extensive aphthous ulceration with skip lesions extending to the terminal ileum, and histology showing polymorph infiltration of the lamina propria, transmural involvement, and micro abscess formation was suggestive of Crohn’s disease. Sensory motor axonal peripheral neuropathy, which is another rare association of inflammatory bowel disease, was also present.ConclusionAn unrecognized genetic predisposition or altered gut permeability causing disruption of the gut immune barrier triggering an immune response against skeletal muscles may have contributed to this unique association. Both polymyositis and Crohn’s ileocolitis responded well to corticosteroids and azathioprine, which is supportive of their immune pathogenesis. Myositis can be considered to be a rare extraintestinal manifestation of Crohn’s disease and can be used in the differential diagnosis of corticosteroid or hypokalemia-induced myopathy in Crohn’s disease.

Acute onset polymyositis after prolactinoma extirpation.

Hyperprolactinemia has been related to autoimmune diseases. Herein, we describe a case of a female with a prolactin producer pituitary macroadenoma who developed severe polymyositis one month after its removal. The patient had very high levels of CPK and muscle biopsy showed remarkable inflammatory infiltration. Steroid therapy was followed with total recovery. To the best of our knowledge, this is the first case reported of acute polymyositis after pituitary macroadenoma exeresis.

Acute polymyositis associated with hemophagocytic lymphohistiocytosis syndrome

Background Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease in which an exaggerated but ineffective immune response leads to severe hyper inflammation. It commonly affects infants from birth to 18 months of age, but cases in older children and adults have also been reported. Key players in HLH are activated lymphocytes and histiocytes, they infiltrate all organs and secrete large amounts of cytokines. Cardinal symptoms are prolonged fever, hepatosplenomegaly, cytopenias and hemophagocytosis [1]. The aim of this case presentation is to focus on the unique presentations of HLH syndrome and to be aware of unusual presentations, so that we could diagnose early and treat it appropriately to ensure long term survival.

Polymyositis after donor lymphocyte infusion

Chronic graft-versus-host disease (GVHD) is a common long-term complication of allogeneic hematopoietic stem-cell transplantation (HSCT), and is responsible for morbidity, mortality and a decrease in quality of life of patients after SCT. Polymyositis, which usually co-occurs with other manifestations of GVHD, has previously been reported. However, polymyositis as the sole manifestation of chronic GVHD following donor lymphocyte infusion (DLI) is rare. We report a 30-year-old man with Hodgkin's lymphoma who developed acute polymyositis following treatment by DLI 4months post-allogeneic HSCT. The patient developed fever and generalized myalgia 22days after a single dose of DLI. Laboratory testing showed elevated muscle enzymes and myopathic abnormalities on electromyographic examination. Muscle biopsy showed features of acute polymyositis, with widespread foci of muscle fiber necrosis associated with infiltration of small mononuclear cells. Twenty-four hours after diagnosis, the patient developed a fatal ventricular arrhythmia. Cardiac involvement may occur in association with polymyositis, but usually occurs in elderly patients after several months of illness. The present case highlights the importance of systematic cardiac evaluation when a diagnosis of polymyositis is initially made to exclude this infrequent presentation of chronic GVHD characteristically associated with some HLA-DR haplotypes.

Hepatocellular carcinoma presenting as polymyositis: a paraneoplastic syndrome

Polymyositis as a paraneoplastic syndrome associated with hepatocellular carcinoma is quite rare; only a few cases have been reported. We report a case of a 50-year-old female who presented with subacute quadriparesis, neck muscle weakness, elevated creatinine phosphokinase, a myogenic pattern on the EMG and was diagnosed as having polymyositis, a paraneoplastic syndrome associated with hepatocellular carcinoma with negative hepatic viral markers and a positive ANA. Improvement in patient symptoms and areduction in creatinine phosphokinase, which occurred after lobectomy, supports this rare association.

Acute acquired toxoplasmosis presenting as polymyositis and chorioretinitis in immunocompetent patient

The parasite Toxoplasma gondii mainly encysts in brain, retina, myocardium, and skeletal muscle. It has been implicated in the genesis of inflammatory myopathies for years, but the parasite usually cannot be detected in the muscle. It is established, however, that toxoplasmosis can cause myositis either by recent infection or by reactivation. The case of a non-HIV patient who developed an acute polymyositis upon infection by T. gondii is reported. We suggest that all patients with polymyositis should have serological tests for toxoplasmosis as a part of their initial evaluation and early trial of antiprotozoal therapy in case of positive findings.

A Case of Interferon-Induced Anti-Jo1 Positive Polymyositis with Pulmonary Fibrosis

Purpose: Interferon therapy for hepatitis C is rarely complicated by the onset of autoimmune diseases. Thyroid disease, hemolytic anemia and TTP are better known autoimmune diseases complicating interferon therapy. We present a case of anti-Jo1 positive polymyositis with pulmonary fibrosis induced by interferon therapy. A 54-year old female with hepatitis C and biopsy consistent with mild activity and mild fibrosis was treated with pegylated interferon and ribavirin. After starting therapy, she complained of flu-like symptoms. Four weeks later, she complained of worsening flu-like symptoms, shortness of breath and weakness in her arms and legs prompting admission to the hospital. At admission, her blood pressure, pulse and temperature were normal. She was dyspneic with increased work of breathing. On exam, she had bilateral coarse breath sounds and weakness in the proximal muscle groups in all extremities. Labs at admission were ALT93, AST115, Alkp144, TB0.2, Alb2.8, CPK2637, Aldolase33.8, ANA < 1:40, dsDNA < 30, C4 < 8, C3 < 9, RF14, and ESR70. Anti Jo1 was positive. SSA, SSB, pANCA, anticardiolipin, ASMA, and AMA were negative. Chest x-ray showed bilateral interstitial infiltrates. The patient underwent bronchoscopy with biopsy demonstrating interstitial organizing pneumonitis and chronic bronchitis. Pulmonary function tests demonstrated moderate chest restriction with reduced DLCO to 30% of predicted. Clinical presentation suggested a diagnosis of anti-Jo1 positive acute polymyositis with interstitial pneumonitis. This constellation of clinical features comprises the antisynthetase syndrome. Interferon therapy was discontinued and the patient was treated with cyclophosphamide and prednisone. She had gradual improvement in her weakness and pulmonary function. The serum aldolase and creatine phosphokinase levels returned to normal. Interferon therapy is rarely complicated by the development of autoimmune diseases. There have been few reports of acute polymyositis with pulmonary fibrosis associated with interferon therapy. The presence of anti-Jo antibody in patients with acute polymyositis is associated with the development of pulmonary fibrosis. An association between Anti-Jo1 positive polymyositis and hepatitis C has been previously described in patients without exposure to interferon therapy. Because of the temporal relationship between interferon therapy and the development of symptoms of this rare disease, the patient presents with interferon-induced antisynthetase syndrome.

Arterial Occlusion from Anti-Jo1 Antibody-associated Autoimmune Myositis: Arteritis not Compartment Syndrome

We present a case of arterial occlusion secondary to polymyositis associated with anti-Jo1 antibodies. A 44 year old lady presented with thigh pain and myositis associated with anti-Jo1 antibody positivity. She developed critical lower limb ischaemia with normal compartment pressures but occlusive disease on angiography. Management was with steroids and methotrexate with amputation of three digits. This case illustrates adult arteritis in the presence of acute polymyositis and anti-Jo1 antibodies as a rare yet diagnostically challenging cause of arterial ischaemia.

Myopathy associated with anti–signal recognition peptide antibodies: Clinical heterogeneity contrasts with stereotyped histopathology

We report three patients with anti-signal recognition particle antibodies who had different presenting clinical pictures, mimicking acute polymyositis, limb-girdle muscular dystrophy, and acute rhabdomyolysis. Muscle biopsies typically showed necrotizing myopathy with little or no inflammation and deposits of membrane attack complex (C5b-9) in endomysial capillaries. The clinical course was severe in two patients and mild in one. The combination of corticosteroid with either an immunosuppressive agent or intravenous immunoglobulins was required to improve the condition of these patients.

Acute polymyositis during treatment of acute hepatitis C with pegylated interferon alpha-2b

Hepatitis C virus is not cleared after primary infection in 50–85% of subjects exposed to hepatitis C virus. Anti-viral treatment during the early phase of infection significantly enhances the likelihood of a sustained clearance of hepatitis C virus. Although, a variety of autoimmune-related side effects have been observed during interferon therapy for chronic hepatitis, immuno-mediated adverse reactions have not been reported during treatment of acute hepatitis C. We describe the case of a patient who developed acute hepatitis C virus infection and, while receiving pegylated interferon alpha-2b monotherapy, developed a severe polymyositis. This case illustrates the potential risk of autoimmunity by interferon, also for acute hepatitis, and underlines the importance of a prompt diagnosis and a rapid discontinuation of interferon treatment for an improvement of clinical outcomes.

The Electromyographic Features of Acute Rhabdomyolysis

Rhabdomyolysis and acute polymyositis share a similar clinical profile with elevated serum creatine kinase (CK). Unlike polymyositis, the electromyography (EMG) findings of acute rhabdomyolysis with myoglobinuria are not well defined. We retrospectively evaluated 15 patients during the acute phase of rhabdomyolysis. All patients (age range, 10-84 years) underwent electrodiagnostic studies, including needle EMG, during the first 2 weeks from the onset of symptoms. All patients presented with acute myalgia, muscle tenderness, and severe generalized weakness. The median peak CK was 48.0 k u/L (25th and 75th percentiles, 11.0 and 68.0 k u/L), whereas the median CK elevation at the time of EMG examination was 10.0 k u/L (25th and 75th percentiles, 4.5 and 48.5 k u/L). Nerve conduction studies were normal in all except 1 patient who had a preexisting mild polyneuropathy. Needle EMG was performed on 117 muscles. Eighty-seven of 117 (74%) muscles were normal, and 19 of 117 (16%) showed variable proximal myopathic motor unit action potentials. None had persistent fibrillation potentials. One third of patients (5 of 15) had myopathic EMG that was present in 1 to 3 sampled proximal muscles. We conclude that the EMG findings during rhabdomyolysis are often normal and when abnormal, the changes are subtle and are in contrast to the often-prominent EMG findings in polymyositis and dermatomyositis.

Acute polymyositis after donor lymphocyte infusion

Polymyositis usually occurred along with other manifestations of chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (HSCT) had been reported. However, polymyositis with a sole manifestation of acute GVHD following donor lymphocyte infusion (DLI) is rare. We reported a 45-yr-old man of acute lymphoid leukemia post-allogeneic HSCT 6 months developed acute polymyositis after DLI. He did not develop any symptoms, signs of acute or chronic GVHD following allogeneic HSCT, despite withdraw of immuosuppresive agents, cyclosporin A (CsA). As the DNA-STR of bone marrow analysis showed mixed chimerism, he received the DLI for remission on May 16, 2002. Acute polymyositis developed following DLI 22 d later. The clinical presentation of polymyositis is compatible with a manifestation of acute GVHD following DLI. It also responds to the treatment of steroid and CsA.

Acute Polymyositis Following Renal Transplantation

Myositis is a rare complication following renal transplantation and is most commonly the result of drug-mediated myotoxicity. Other causative disorders include viral infection, electrolyte imbalance and myositis of autoimmune origin. We describe a 60-year-old patient who developed acute polymyositis 4 weeks after a 000 human leukocyte antigen (HLA) mismatch cadaveric renal transplant. Following an uncomplicated transplant course with maintenance triple immunosuppression (prednisolone, mycophenolate mofetil and cyclosporine), the patient presented with severe symmetrical proximal muscle weakness associated with a rise in serum creatine kinase to 46 800 U/L. Electromyography confirmed myopathic changes and muscle biopsy demonstrated extensive muscle-fiber necrosis with an inflammatory infiltrate. There were no obviously culpable drugs and viral studies were negative. Prompt initiation of high-dose steroid therapy led to clinical and biochemical recovery. Acute polymyositis may occur following renal transplantation. Potential mechanisms include viral antigen transmission or a localized form of graft vs. host disease.

Manifestations cardiaques des polymyosites. À propos de deux cas sénégalais

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. 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.

Fatal myocarditis with acute polymyositis in a young adult

A 21 year old woman presented with acute polymyositis associated with fatal myocarditis. The significance of cardiac involvement in polymyositis is discussed in relation to this unusually fulminant case.

Acute polymyositis in an adult associated with Mycoplasma pneumoniae infection

Summary A previously healthy adult developed a hitherto unreported major complication of Mycoplasma pneumoniae infection, namely acute polymyositis.

Acute Polymyositis and Hyperthyroidism in a Patient on Long-Term Amiodarone Therapy

A 52-year-old male received continuous oral amiodarone therapy for 3 years for the management of ventricular tachycardia nonresponsive to other antiarrhythmics, after he sustained a Q wave anterior myocardial infarction. He developed pain and weakness in both thigh and shoulder muscles in addition to weight loss and profuse sweating. His investigations confirmed the diagnosis of acute polymyositis and hyperthyroidism. His symptoms disappeared and the abnormal biochemical tests returned to normal after the withdrawal of amiodarone. We have not been able to find any other such report and believe that these were adverse side-effects of this drug.