The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.
Male ; Humans ; Middle Aged ; Autoantibodies ; Myositis/diagnosis* ; Autoimmune Diseases ; Muscle, Skeletal/pathology* ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use* ; Necrosis/pathology* ; Muscular Diseases/drug therapy*

OBJECTIVE: Immune checkpoint inhibitors (ICI) have significantly improved the treatment efficacy of a variety of malignant tumors. However, patients may experience a series of special side effects during treatments with ICI. Immune-related myositis after ICI treatment is characterized by autoimmune rheumatic and musculoskeletal damage, which is relatively rare. To analyze the clinical characteristics and outcomes of ICI-associated myositis in urological tumors, we summarized the clinical manifestations, electrophysiological and pathological characteristics, treatments and outcomes in 8 patients. METHODS: The clinical data of the 8 patients with immune-related myositis after ICI treatment for urological tumors treated in the Department of Urology, Peking University First Hospital from March 2018 to March 2022 were retrospectively analyzed for demographic characteristics, drug regimen, clinical symptoms, laboratory indices, electromyography examination, pathological manifestations and outcomes. RESULTS: The eight patients included 2 females and 6 males with a median age of 68 years, all treated with ICI for urological neoplasms, including 2 upper tract urothelial carcinoma (UTUC), 3 renal cell carcinoma (RCC), and 3 bladder cancer (BCa). The median time between the first ICI treatment and the detection of immune-related myositis was 39.5 days, and the median duration of treatment was 2 sessions. The main symptoms were muscle pain and weakness, 5 cases with ptosis, 3 cases with secondary rhabdomyolysis, 5 cases with myocarditis, 1 case with myasthenia gravis, and 1 case with enterocolitis. Among them, patients with immune-related myocarditis had a shorter interval from the first anti-programmed cell death protein-1 (PD-1) therapy to the onset of immune-related myositis (P=0.042) compared with patients without myocarditis. The 8 patients had significant elevation of transaminases and muscle enzyme profile indexes, and 5 patients showed positive auto-antibodies. 3 patients had perfected muscle biopsies and showed typical skeletal muscle inflammatory myopathy-like pathological changes with CD3⁺, CD4⁺, CD8⁺, CD20⁺ lymphocytes and CD68⁺ macrophage infiltration. After the diagnosis of immune-related myositis, all the 8 patients immediately discontinued ICI therapy and improved after intravenous administration of methylprednisolone alone or in combination with gamma-globulin. CONCLUSION Immune-related myositis after ICI treatment is an immune-related adverse reactions (irAEs) with unique clinical and pathological features, commonly combined with cardiovascular adverse reactions. Immediate discontinuation of ICI and initiation of glucocorticoid therapy may improve the patient's condition in a timely manner.
Aged ; Antineoplastic Agents, Immunological/adverse effects* ; Carcinoma, Transitional Cell ; Female ; Humans ; Immune Checkpoint Inhibitors/adverse effects* ; Kidney Neoplasms/drug therapy* ; Male ; Myocarditis/drug therapy* ; Myositis/pathology* ; Retrospective Studies ; Urinary Bladder Neoplasms

Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.
Child ; Female ; Glucocorticoids ; Humans ; Infant ; Magnetic Resonance Imaging ; Myositis/drug therapy* ; Rituximab

No abstract available.
Aged ; Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Drug Therapy, Combination ; Female ; Glucocorticoids/therapeutic use ; Humans ; Immunosuppressive Agents/therapeutic use ; Magnetic Resonance Imaging ; Myositis/*complications/diagnosis/drug therapy/immunology ; *Paraspinal Muscles/drug effects/immunology/pathology ; Polymyalgia Rheumatica/diagnosis/drug therapy/*etiology/immunology ; Risk Factors ; Sacroiliitis/*complications/diagnosis/drug therapy/immunology ; Treatment Outcome

A 24-year-old Chinese woman with Graves' disease presented with myositis two months after treatment with carbimazole. The patient's myositis resolved with hydration and cessation of carbimazole. No other causes of myositis were found, and a change in the medication to propylthiouracil was uneventful. Review of the literature suggests a possible genetic susceptibility, as the majority of reported cases are Asian in origin, similar to patients who present with thyroid periodic paralysis. Changing the antithyroid drugs (ATDs) administered, decreasing the dose of pre-existing ATDs in the treatment regimen or addition of levothyroxine has been shown to result in clinical improvement of this complication. These observations suggest various mechanisms of carbimazole-induced myositis in the treatment of Graves' disease, including the direct effect of ATDs on myocytes, immune-related responses secondary to ATDs and rapid decrements in thyroid hormone with ensuing myositis.
Antithyroid Agents ; adverse effects ; Carbimazole ; adverse effects ; Female ; Genetic Predisposition to Disease ; Graves Disease ; drug therapy ; Humans ; Myositis ; chemically induced ; genetics ; therapy ; Young Adult

Radiation recall phenomenon is an inflammatory reaction which occurs at a previously irradiated area after the administration of causative agents, especially anticancer drugs. Radiation recall mostly involves skin and rarely affects internal organs. We, hereby, report a rare case of radiation recall myositis. A 51-years-old man visited the hospital due to pain and weakness of the left thigh. He had been diagnosed with a skin metastasis of pancreatic adenocarcinoma, at the left thigh and treated with radiotherapy and subsequent combination chemotherapy of 5-FU and cisplatin. After the 5th cycle of chemotherapy, the patient developed pain and weakness localized at the previous radiation field. An MRI revealed myositis of left gluteus medius muscle. Muscle biopsy showed no malignant cells or signs of infection. He was diagnosed as having radiation recall myositis and treated with steroid and NSAID. Soon after, pain and weakness gradually improved. Although rare, a possibility of radiation recall myositis should be considered when a patient with history of radiotherapy has a myopathy.
Adenocarcinoma ; Biopsy ; Cisplatin ; Drug Therapy, Combination ; Fluorouracil ; Humans ; Muscles ; Muscular Diseases ; Myositis ; Neoplasm Metastasis ; Skin ; Thigh

Trigger point injection is a simple procedure that is widely performed for relieving pain. Even though there are several complications of trigger point injection, myositis ossificans has not been documented as one of its complications. We treated a patient who suffered from painful limitation of elbow motion and this was caused by myositis ossificans between the insertions of brachialis and supinator muscles after a trigger point injection containing lidocaine mixed with saline, and we also review the relevant medical literature.
Adult ; Elbow/surgery ; Humans ; Injections, Intralesional/*adverse effects ; Lidocaine/*adverse effects/therapeutic use ; Magnetic Resonance Imaging ; Male ; Myofascial Pain Syndromes/drug therapy ; Myositis Ossificans/diagnosis/*etiology/radiotherapy/surgery ; Range of Motion, Articular

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause the DRESS syndrome. We report a case in a 29-yr-old female patient who had been on celecoxib and anti-tuberculosis drugs for one month to treat knee joint pain and pulmonary tuberculosis. Our patient's clinical manifestations included fever, lymphadenopathy, rash, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis). During the corticosteroid administration for DRESS syndrome, swallowing difficulty with profound muscle weakness had developed. Our patient was diagnosed as DRESS syndrome with eosinophilic polymyositis by a histopathologic study. After complete resolution of all symptoms, patch tests were positive for both celecoxib and ethambutol. Although further investigations might be needed to confirm the causality, celecoxib and ethambutol can be added to the list of drugs as having the possibility of DRESS syndrome.
Adult ; Anti-Inflammatory Agents, Non-Steroidal/adverse effects ; Antitubercular Agents/adverse effects ; Arthritis/complications/*drug therapy ; Drug Eruptions/*etiology/pathology ; Eosinophilia/*chemically induced/pathology ; Ethambutol/*adverse effects ; Female ; Humans ; Myositis/chemically induced/pathology ; Pyrazoles/*adverse effects ; Sulfonamides/*adverse effects ; Syndrome ; Tuberculosis, Pulmonary/complications/*drug therapy

Child ; Humans ; Male ; Myositis ; diagnosis ; drug therapy ; pathology

Dermatomyositis is a rare and idiopathic inflammatory myopathy with characteristic cutaneous manifestations. A 61-year-old female presented with the chief complaints of proximal muscle weakness, facial edema and erythema on the face, chest and back. The patient was diagnosed with dermatomyositis by the clinical manifestation, laboratory findings and an electromyogram. After a year following appropriate treatment, a tonsil cancer was found. The patient was treated with three rounds of neoadjuvant chemotherapy and wide excision of the left tonsil. In Korea, a few cases of dermatomyositis have been reported associated with stomach cancer, breast cancer, acute lymphoblastic leukemia and lung cancer, but no associated cases with tonsil cancer have yet been reported. We report a case of tonsil cancer that developed in a patient with dermatomyosits during therapy.
Breast Neoplasms ; Dermatomyositis* ; Drug Therapy ; Edema ; Erythema ; Female ; Humans ; Korea ; Lung Neoplasms ; Middle Aged ; Muscle Weakness ; Myositis ; Palatine Tonsil* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Stomach Neoplasms ; Thorax ; Tonsillar Neoplasms*