Case Of Myositis Research Articles

Paraneoplastic Proliferative Myositis on 18F-FDG PET/CT.

We present a rare and never-before-reported case of paraneoplastic proliferative myositis that was incidentally detected in a 42-year-old man who had been receiving systemic cytotoxic chemotherapy for classic Hodgkin lymphoma. We highlight his 18F-FDG PET/CT findings and its utility in helping to diagnose this condition, avoiding unnecessary surgical resection and the added costs associated with it.

Fatal Case of Immune-Related Myocarditis and Myositis Due to Treatment with Immune Checkpoint and Tyrosine Kinase Inhibitors.

Immune checkpoint inhibitor and tyrosine kinase inhibitor combinations have become the new standard of care in the first-line treatment of metastatic clear cell renal cell carcinoma. However, there is a growing concern regarding severe immune-related adverse events. A 78-year-old man with metastatic clear cell renal cell carcinoma, treated with pembrolizumab and axitinib, was admitted to the emergency department 30 days after initiating treatment due to rapidly progressive myositis. Myocarditis with severe ventricular dysfunction was identified. Muscular biopsy findings were compatible with inflammatory myopathy associated with immune checkpoint inhibitors. Initial treatment with high-dose corticosteroids showed an insufficient response. Therapeutic escalation on the third day with methylprednisolone, immunoglobulin, and abatacept resulted in clinical improvement. On the eleventh day, a sudden malignant arrhythmia occurred, followed by cardiac arrest. This represents one of the first case reports describing myocarditis and myositis during treatment with pembrolizumab-axitinib. While immune checkpoint inhibitor may play a major role, it is also possible that the tyrosine kinase inhibitor, while attempting to promote immune modulation, also increases severe toxicities.

Increased myositis and possible myocarditis in melanoma patients treated with immune checkpoint inhibitors in the COVID-19 era.

Immune checkpoint inhibitor (ICI)-mediated myocarditis results in significant morbidity and mortality. At our institution, we noted an increased incidence of ICI-mediated myocarditis cases, leading to further investigation in our database of advanced melanoma patients treated with ICI therapy. A single-center, retrospective cohort analysis of patients with advanced melanoma identified cases of ICI-mediated myocarditis and myositis. 366 patients with advanced melanoma received a dose of ICI from September 2014 to October 2019. Of these patients, there were 0 cases of ICI-mediated myocarditis (0%, 95% CI 0%-1.0%) and 2 cases of ICI-mediated myositis (0.55%, 95% CI 0.07%-1.96%). From November 2019 to December 2021, an additional 246 patients with advanced melanoma were identified. Of these patients, 10 (4.1%, 95% CI 1.97%-7.35%) developed ICI-mediated myocarditis and 10 developed ICI-mediated myositis. Our study suggests an increase in prevalence of ICI-mediated muscle damage including myositis and myocarditis in the COVID-19 era. Differentiation of these patients and further risk stratification may allow for development of guidelines for nuanced management of this serious complication.

A Rare Case of Severe Myositis with Microsporidia Invasion of the Kidneys

A Rare Case of Severe Myositis with Microsporidia Invasion of the Kidneys

Paediatric Myositis Linked to Influenza B Infection: A Case Report

Objective: To report a rare case of myositis associated with Influenza B infection in a pediatric patient, emphasizing the clinical presentation, diagnostic challenges, and management strategies. Case: We present the case of a 9-year-old male who developed acute myositis following an Influenza B infection, characterized by severe muscle pain, difficulty walking, and an exceptionally high Creatine Phosphokinase (CPK) level of 33,256 U/L. Despite initial negative results for Influenza B, subsequent testing confirmed the infection, leading to a prompt and successful treatment with intravenous hydration and oseltamivir. Conclusion: Myositis associated with Influenza B, although rare, should be considered in pediatric patients presenting with sudden muscle pain and gait disturbances, particularly during influenza season. Early diagnosis and intervention are crucial in preventing complications such as rhabdomyolysis and acute renal failure.

A POLYMYOSITIS AFTER mRNA COVID-19 VACCINATION CASE REPORT

Polymyositis is a rare, acquired, and inflammatory muscular disease, which is characterized by the inflammation of muscles and has no known etiology. The autoimmunity created by the vaccine arose from molecular mimicking or directly by vaccine adjuvants. Polymyositis cases were reported after hepatitis B, influenza A (H1N1), tetanus toxoid, and Bacille Calmette-Guerin (BCG) vaccinations. However, no relationship with COVID-19 vaccination has been reported yet. Myositis and dermatomyositis cases have been seen after covid-19 vaccine. m-RNA COVID-19 vaccines might cause abnormal activation of the immune system due to a strong t-cell-mediated immune activation by vaccinesin the present case, without any additional triggering factor, a case diagnosed with polymyositis developing 10 days after the second dose of messenger RNA (mRNA) COVID-19 vaccine is presented. The findings suggest a potential association between mRNA COVID-19 vaccination and the development of polymyositis, prompting further investigation into this rare occurrence.

Treatment resistance in inclusion body myositis: the role of mast cells

Inclusion body myositis is the commonest acquired myopathy in those over 50 years of age. Although it is classified as an idiopathic inflammatory myopathy and the most frequent finding on muscle biopsy in inclusion body myositis is an endomysial inflammatory infiltrate, it is clinically distinct from other myositis, including a lack of response to immunosuppressive medication. Neurogenic changes are commonly reported in inclusion body myositis and inflammatory changes are observed in muscle following neurogenic injury. The objective of our study was to explore whether neurogenic inflammation plays a role in the pathogenesis of inclusion body myositis, possibly explaining its resistance to immunosuppression. The number of mast cells and presence of neuropeptides, substance P and calcitonin gene-related peptide, were assessed in 48 cases of inclusion body myositis, 11 cases of steroid responsive myositis, two cases of focal myositis associated with neurogenic injury, and ten normal controls. The number of mast cells in inclusion body myositis focal and myositis associated to neurogenic injury were significantly greater than that observed in steroid responsive myositis. Our findings suggest that neurogenic inflammation mediated through mast cells may play a role in the pathogenesis of inclusion body myositis, and focal myositis associated to neurogenic injury, and thus, explain in some part its lack of response to immunosuppressive treatments.

Immune checkpoint inhibitor related myositis: an observational, retrospective, pharmacovigilance study

ABSTRACT Background Immune checkpoint inhibitors (ICIs) show promise in cancer treatment, but recent cases highlight myositis as a serious complication. Research design and methods We did a retrospective study on drug safety using FAERS data up to Q3 2022, focusing on immune checkpoint inhibitors (ICIs) and myositis. We used IC and ROR to assess the association. Logistic regression in R 3.2.5 helped identify factors linked to fatal outcomes. Results We identified 558 cases of ICIs-associated myositis. Our study found a significant link between ICIs and myositis (ROR 15.54 [14.23–16.96], IC 3.79 [3.66–3.92], see Figure 1). Notably, myositis was more common in patients on ICI combination therapy compared to monotherapy (ROR 1.72 [1.39–2.11], IC 0.63 [0.30–0.93]). Age increased the risk of ICI-associated myositis and was also a factor in fatality (p = 0.011). Common accompanying adverse events included myocarditis (21.33%), severe myasthenia gravis (16.49%), and malignant neoplasm progression (8.06%). Fatal cases were more common when myositis was accompanied by myocarditis, severe myasthenia gravis, or malignant neoplasm progression. Conclusions Clinicians must note the risk of ICI-associated myositis, especially dangerous in older patients or when combined with other issues like myocarditis or severe myasthenia gravis.

A peculiar case of idiopathic orbital myositis in adolescence

Abstract
 Introduction : Idiopathic orbital myositis, an inflammation localized at the extraocular muscles, is rarely reported in pediatrics. Until now, the prevalence and incidence of orbital myositis remain unknown. This paper aims to report a case of idiopathic orbital myositis in a young girl with headache.
 Case Illustration : A-16-year-old presented with a droopy eyelid of the left eye (LE) and double vision since 1 week before admission. She also experienced swollen eyelid and cluster headache for 5 months with no neurological abnormalities. Ophthalmic examinations showed exotropia with limited eye movement of LE. Ptosis of LE was noted with margin reflex distance 1 (MRD-1) +2 mm, while MRD-1 +6 mm for the right eye. Other examinations were unremarkable with normal funduscopy and negative RAPD. Non-contrast orbital sinus CT-Scan revealed an enlargement of the superior rectus and lateral rectus muscle. The laboratory result showed slight leucocytosis and normal ESR. High-dose intravenous corticosteroid was administered and tapered with oral steroid. The orbital clinical findings showed improvements, better ocular movements, and diminished headache complaints.
 Discussion : As a rare case, the diagnosis of orbital myositis in adolescence can be challenging and requires detailed examination, and proper imaging to exclude other diagnosis such as ophthalmoplegic migraine, infections, malignancies, or congenital lesions (hemangioma). The first-line therapy is a systemic corticosteroid, which well responded in this patient.
 Conclusion : In this patient, intravenous corticosteroid represent an effective treatment. Close monitoring of the disease progression is required after switching to oral therapy to prevent relapse.

Statin Associated Muscular Adverse Effects.

Statins are widely used in the treatment of hyperlipidemia and in the prevention of cardiovascular diseases. However, they may induce muscular adverse effects that range from asymptomatic elevation of creatine kinase to life threatening rhabdomyolysis. The aim of the study was to describe epidemiological and clinical characteristics of patients with muscular adverse effects. We conducted a retrospective and descriptive study over a ten-year period from January 2010 to December 2019. We included all cases of statin associated muscular adverse effects notified to the Tunisian National Centre of Pharmacovigilance during this period. The study involved 22 muscular adverse effects related to statins (28% of all adverse events reported with statins during this period). Patient's mean age was 58.7 years and the sex ratio was 1.6. There were 12 cases of isolated creatine kinase elevation, 5 cases of myalgia, 3 cases of myopathy, one case of myositis and one case of rhabdomyolysis. Muscular adverse effects occurred 7 days to 15 years after starting this drug. In all cases, the statin was withdrawn after muscular adverse effects and resolution of symptoms was observed within 10 days to 18 months. In seven cases, creatine kinase persisted elevated for 18 months. Involved statins were atorvastatin, simvastatin, rosuvastatin and fluvastatin. Early recognition of muscle symptoms is required to prevent rhabdomyolysis. Further researchare needed to completely elucidate the pathophysiology of statin-induced muscular adverse effects.

Myositis : Rare Presentation of Dengue, not Uncommon in Children

Dengue myositis is a recently recognized category of idiopathic inflammatory myopathies manifested as symmetric proximal muscle weakness with severe limb pain and high serum creatine kinase (CK) levels, in dengue patient (NS1 positive or IgM positive). A total 4 cases of dengue myositis were admitted at Shaheed Suhrawardy Medical College during the period from August to October, 2023. All were male & they presented mainly with severe limp pain with inability to walk they were diagnosed as dengue fever serologically. Two cases presented during febrile phase & two following fever. Their S. CPK level were high with mildly low platelet count. None of them developed shock or hemorrhagic manifestation. They were treated supportively and all 4 cases recovered without any sequelae. Dengue myositis in children is usually benign and is differentiated from other causes of flaccid paralysis by tenderness of calf and thigh muscles and raised CPK levels with other normal neurological findings. But sometimes it may result in serious consequences, so all children with dengue infection should be followed up carefully with S.CPK level J Bangladesh Coll Phys Surg 2023; 41: 101-104

P54 T-cell response to SRP-9001 dystrophin transgene in a patient treated with Delandistrogene Moxeparvovec: a case of immune-mediated myositis

P54 T-cell response to SRP-9001 dystrophin transgene in a patient treated with Delandistrogene Moxeparvovec: a case of immune-mediated myositis

Terbinafine-induced Myositis: A Case Report

We report a case of terbinafine-induced myositis in a 37-year-old Asian male patient treated for Tinea cruris. The patient complained of severe generalized body aches, weakness, fever, fatigue, and passing of concentrated urine after consuming one dose of terbinafine 250 mg tablet, which worsened after the second dose. At the presentation, the patient was afebrile, fatigued, and had difficulty moving. All the laboratory parameters were normal, except N-acetyl-cysteine-(NAC)-activated creatine kinase, which was elevated to 276 U/L. There was a slight elevation in alanine transaminase (ALT) levels (44 U/L) and albumin-to-globulin ratio (1.8). The oral terbinafine was stopped. Patient was prescribed with tablet acetaminophen 650 mg thrice daily for two days and as needed thereafter, to manage myalgia. The patient reported being symptomatically better after six days of terbinafine withdrawal. The Naranjo’s causality assessment scale score was eight, indicating a probable relation between drug exposure (terbinafine use) and adverse drug reaction (myositis). The severity of terbinafine-induced myositis in this patient was moderate (level 3) as categorized by the Modified Hartwig and Siegel scale.

Paraspinal myositis in a patient with COVID-19 infection: a case report

Myalgia is one of the most common symptoms of COVID-19 infection. With the progression of the COVID-19 pandemic, emerging cases of COVID-related myositis have been reported. However, COVID-19-related myositis of the paraspinal muscle is uncommon. We present a 66-year-old female who was admitted to the hospital for hemorrhagic shock secondary to gastrointestinal bleeding. She developed COVID-19 respiratory tract infection symptoms on the second day of admission with reported severe lower back pain 3 days later. Serum creatinine kinase level was slightly elevated, and magnetic resonance imaging (MRI) revealed paraspinal myositis.

E38 Pediatric dermatomyositis, what specificities

Abstract Introduction Dermatomyositis is a rare disease. In children the incidence is 2–3 per million population. It is a potentially serious disease whose mortality is < 5%, but whose complications and long-term prognosis remain poor for some children. Objectives We report the epidemiological, clinical, therapeutic and evolutionary characteristics of a retrospective case series of 14 children with dermatomyositis seen at the pediatric center of Setif (Algeria). Materials and methods The average age of onset is 8 years (0–15 ½ years), The sex ratio (M/F) was 0.21; The mean duration of symptoms prior to diagnosis was 4.4 months. At the time of diagnosis, 100% of children had skin rash, 84% eyelid telangiectasia, 40% Gottron's papules, 90% muscle weakness, 40% arthritis, 13% fever, and 25% dysphagia. The muscle enzymes were elevated in all, FAN positive in 6 cases, anti-DNA was positive in a single patient with overlapping myositis. The electromyogram cames myogenic for all our 14 patients, 10 patients had muscle biopsy with report of non-specific interstitial and parenchymal myositis Therapeutic regimens were variable, they depended on the severity of the initial picture, Nevertheless all of the children were treated with first-line corticosteroids (100%), methotrexate was prescribed in 12 cases (85%); Mycophenolate mofetil and Rituximab in one patient, and bisphosphonate in a single child with extensive calcinosis. Polyvalent immunoglobulins were used for 4 cases with swallowing disorders and calcinosis (28%), 4 patients had calcinosis as complication (28%), only one child died from aspiration pneumonitis. Discussion The diagnosis is made according to the criteria of Bohan and Peter, given the non-specificity of the muscle biopsies. The clinical and evolutionary characteristics of our serie are consistent with the overall data in the literature with a predominance of skin and muscle involvement. However, some specificities were individualized such as the case of myositis overlapping with lupus. The treatment is essentially based on corticosteroid therapy and methotrexate. Conclusion Juvenile dermatomyositis is a rare serious and chronic condition requiring multidisciplinary management. Ethics Associate Professor of pediatrics, department of medicine University Setif-Algeria (pediatric center –Setif-Algeria)

INFECTIVE MYOSITIS IN A PATIENT HAVING NO RISK FACTORS: A CASE REPORT

Abstract Infective myositis, an uncommon type of inflammatory myopathy caused by a wide range of infective agents like viruses, bacteria, fungi, and parasites, is a potentially treatable condition if diagnosed in time. It can cause devastating illnesses in previously well patients. We present a case of infective myositis which illustrates that it is a rare case in a patient with no risk factors and highlights the challenges encountered in the diagnosis. Key Words: Infective myositis, MRI findings in infective myositis

Mogamulizumab-Associated Myositis With and Without Myasthenia Gravis and/or Myocarditis in Patients With T-Cell Lymphoma.

Mogamulizumab is being increasingly prescribed for the treatment of T-cell lymphomas (MF/SS/ATLL). We conducted a retrospective cohort study to identify muscular immune-related adverse events (irAEs) associated with mogamulizumab in patients with T-cell lymphoma followed at Dana-Farber Cancer Institute from January 2015 to June 2022. We identified 5 cases of mogamulizumab-associated myositis and/or myocarditis (MAM/Mc), 2 additionally affected by myasthenia gravis, among 42 patients with T-cell lymphoma. Three cases experienced -mogamulizumab-associated rash (MAR) prior to developing MAM/Mc. The incidence (n = 5/42, 11.9%) of muscular mogamulizumab-associated irAEs may be higher than has been previously reported in clinical trials and may be of late onset (a median of 5 cycles and as late as 100 days from the last infusion). We highlight the utility of IVIG, together with systemic corticosteroids, for the treatment of these potentially fatal side effects associated with mogamulizumab therapy.

Triple M Syndrome with Triple Seronegative Myasthenia Gravis presenting as a Non-ST Elevation Myocardial Infarction (P1-8.002)

<h3>Objective:</h3> N/A <h3>Background:</h3> Immune checkpoint inhibitors hinder different receptors in the immune system that maintain immunologic self-tolerance. Despite their efficacy, multiple reports of immune related adverse events exist. 1–3% of these cases are neurological. There is no clear consensus on how to treat this patient population. <h3>Design/Methods:</h3> We present a case of myocarditis, myositis and triple seronegative myasthenia gravis overlap syndrome secondary to PD-1 inhibitor. <h3>Results:</h3> 75-year-old male with a history of lung cancer and severe coronary artery disease who presented for chest pain and dyspnea. He had recently been started on gabapentin for chemotherapy related neuropathy. Due to recurrent lung cancer he was started on a new PD-1 inhibitor. On admission, he had elevated troponins. Emergency cardiac catheterization was negative for new coronary artery disease. Echocardiogram showed septal and LV wall hypokinesis, patient was admitted as NSTEMI. On physical exam, he had neck muscle weakness with drooping head sign, right eye ptosis, and proximal limb muscle weakness with fatigability of all extremities. Ice pack test done at the bedside showed improvement in right eye ptosis and neck drop. Anti-Ach Binding, Block and Modulating, Anti-Musk and Anti-LRP4 antibodies were negative. Due to suspicion of triple M syndrome he was treated IV Methylprednisolone and Pyridostigmine which stabilized his weakness. His dyspnea continued to worsen despite steroid therapy and he was started on IVIG. After 5 doses he significantly improved and was discharged to an acute rehabilitation facility. <h3>Conclusions:</h3> ICIs associated myocarditis can occur in 1% of patients. Up to 10% of these patients can have overlapping myasthenia gravis and myositis. 10% of patients with this syndrome can have seronegative myasthenia gravis. There are no guidelines adressing the correct treatment approach for this syndrome. In our case, gabapentin possibly precipitated the myasthenic symptoms and treatment with Corticosteroids and IVIG resolved the patient’s symptomatology. <b>Disclosure:</b> Dr. Carranza-Renteria has nothing to disclose. Dr. Mattner has nothing to disclose. Ms. Sial has nothing to disclose. Dr. Babici has nothing to disclose. Dr. Dragomir has nothing to disclose. Dr. Rodriguez-Hernandez has nothing to disclose. Dr. Hammond has nothing to disclose.

Why so low? An unusual case of myositis in a child

BackgroundSarcoidosis is characterized by non-caseating epithelioid granulomas in various tissues throughout the body, most commonly the lung. Non-caseating granulomas may be seen in skeletal muscle, though typically asymptomatic and under-recognized. While rare in children, there is a need to better characterize the disease and its management. Here we present a 12-year-old female with bilateral calf pain who was ultimately found to have sarcoid myositis.Case PresentationA 12-year-old female presented to rheumatology with significantly elevated inflammatory markers and isolated lower leg pain. MRI of the distal lower extremities demonstrated extensive bilateral myositis with active inflammation, atrophy, and to a lesser extent fasciitis. This distribution of myositis in a child garnered a broad differential requiring a systematic evaluation. Ultimately, muscle biopsy revealed non-caseating granulomatous myositis with perivascular inflammation, extensive muscle fibrosis, and fatty replacement of the muscle with a CD4+ T cell predominant, lymphohistiocytic infiltrate consistent with sarcoidosis. Review of histopathology from age 6 of an extraconal mass resected from her right superior rectus muscle further confirmed the diagnosis. She had no other clinical symptoms or findings of sarcoidosis. The patient improved significantly with methotrexate and prednisone, though flared again after self-discontinuation of medications and was subsequently lost to follow-up.ConclusionThis is the second reported case of granulomatous myositis associated with sarcoidosis in a pediatric patient, and the first to present with a chief complaint of leg pain. Increased knowledge of pediatric sarcoid myositis within the medical community will enhance recognition of the disease, improve the evaluation of lower leg myositis, and advance outcomes for this vulnerable population.

A Case of Inclusion Body Myositis with Clinical, Pathological and Serological Consideration

Inclusion body myositis is a rare condition of idiopathic inflammatory myopathy. Prior criteria for the diagnosis of inclusion body myositis essentially required pathological features of rimmed vacuoles, tubulofilamentous inclusions, and amyloid deposits. However, recently developed new diagnostic criteria emphasize clinical characteristics including weakness of finger flexors and knee extensors. In addition, a serological evaluation of anti-cN1A antibody is helpful for the diagnosis. We report a case of inclusion body myositis with clinical, pathological, and serological consideration.