Assessment of intracellular cytokines and regulatory cells in patients with autoimmune diseases and primary immunodeficiencies — Novel tool for diagnostics and patient follow-up

Serum and intracytoplasmic cytokines are mandatory in host defense against microbes, but also play a pivotal role in the pathogenesis of autoimmune diseases by initiating and perpetuating various cellular and humoral autoimmune processes.     

Recurrent polymyositis triggered by bronchiectasis‐associated infections

Polymyositis is a cell‐mediated autoimmune disease and various systemic infections can trigger it. However, there have been only rare reports of recurrent polymyositis triggered by systemic infections. We report the case of a 69‐year‐old woman with recurrent polymyositis triggered by bronchiectasis‐associated infections. This case indicates that recurrent polymyositis triggered by systemic infections suggests a non‐specific autoimmune antigenic response of polymyositis.     

Polymyositis: Essential Information for Primary Care Providers

Polymyositis is one of a rare group of skeletal muscle diseases known as idiopathic inflammatory myopathies. The etiology is not fully understood, and its clinical presentation is often vague yet similar to more common neuromuscular diseases, making diagnosis difficult. A number of different tests are available to assist providers in making an accurate diagnosis. Once a diagnosis is made, there are a number of various treatment modalities available. Nurse practitioners must be familiar with treatment protocols and follow-up. The focus of this article is on polymyositis; its presentation, signs, and symptoms; the process of accurate diagnosis; and common treatment strategies.     

Renal Squamous Cell Carcinoma-related Polymyositis in a Patient with Autosomal Dominant Polycystic Kidney Disease

A 74-year-old Japanese woman diagnosed with autosomal dominant polycystic kidney disease (ADPKD) was admitted to our institute for the further examination of right-side groin pain developing in the past week. The patient was diagnosed with polymyositis (PM). Diagnostic imaging showed a mass lesion measuring 8 cm and a renal stone in the right kidney. Immediately following surgical resection of the right kidney, the patient''s serum CK decreased to the normal range. A histopathological analysis showed well-differentiated squamous cell carcinoma. In conclusion, this case showed a close relationship between the occurrence of squamous cell carcinoma and the development of PM in an ADPKD patient.     

HLA—DR2,DRB1＊0301,DQA1＊0501基因频率与肌炎及其临床表现的 …

目的：观察人类白细胞相关抗原ＨＬＡ－ＤＲ２，ＤＲＢ１＊０３０１，ＤＱＡ１＊０５０１基因频率为多发性肌炎／皮肌炎（ＰＭ／ＤＭ）发病及其临床表现的关系，方法：特异性引物聚合酶链式反应（ＰＣＲ－ＳＳＰ）方法分别测定了３１例ＰＭ／ＤＭ患者及５０例正常人的ＨＬＡ－ＤＲ２，ＤＲＢ１＊０３０１及ＨＬＡ－ＤＡＱ１＊０５０１的基因频率，结果：三种基因型在３１例肌炎患得中基频率分别为：６．４５％，９．６８％和７７．４     

髂腰肌针极肌电图在诊断多发性肌炎中的应用价值

 目的 探讨髂腰肌肌电图在诊断多发性肌炎中的应用价值。方法 回顾性收集2018-01至2019-02于首都医科大学附属北京天坛医院就诊并确诊为多发性肌炎的28例患者(多发性肌炎组)的临床资料,以及年龄、性别相匹配的健康人20名(对照组),就其髂腰肌、股四头肌、三角肌肌电图自发电位和小力收缩运动单位电位(motor unit action potential, MUAP)进行分析。结果 对照组髂腰肌未检测出自发电位,多发性肌炎组髂腰肌自发电位阳性率为57.14%(16/28),显著高于对照组(P<0.001);另外其MUAP波幅显著低于对照组[(424.20±82.41)μV vs. (593.93±65.49 )μV,P<0.001],MUAP时限也显著短于对照组[(9.73±2.05)vs. (11.26±0.42) ms,P<0.01]。在肌肉病患者中,股四头肌自发电位阳性率为35.71%(10/28),三角肌自发电位阳性率为25.00%(7/28),三角肌自发电位显著低于髂腰肌(P<0.05)。结论 髂腰肌在多发性肌炎患者中有明显的肌源性损害的表现,可为临床提供更加敏感、客观的诊断依据。     

Transbronchial biopsy is clinically useful in classifying patients with interstitial pneumonia associated with polymyositis and dermatomyositis

Background and objective: The histological type of intraluminal fibrosis is an important prognostic factor for interstitial pneumonia. We therefore examined whether transbronchial lung biopsy (TBLB) specimens are useful for predicting the clinical course and prognosis of patients with interstitial pneumonia associated with polymyositis and dermatomyositis (PM/DM), with particular attention to the different types of intraluminal fibrosis. Methods: Twenty‐five cases of interstitial pneumonia associated with PM/DM were classified according to the pattern of intraluminal fibrosis as assessed by TBLB, and the clinical course and response to treatment were compared. Interstitial fibrosis was evaluated by sequential thin‐section CT scans. Results: In 19 of 25 (76%) cases, there was sufficient intraluminal fibrosis to perform an evaluation. Intraluminal fibrosis was classified as bud (polyp) type or mural incorporation type (either alone or mixed with bud type). The bud type was seen in five cases and these improved following treatment with corticosteroids only. The mural incorporation type was seen in 14 cases. In 11 of these 14 cases, progressive long‐term fibrosis developed and four cases were fatal, in spite of corticosteroid and immunosuppressive therapy. The response to drugs (P < 0.01) and survival (P < 0.05) were significantly greater in patients with bud‐type than mural incorporation‐type intraluminal fibrosis. Conclusions: Classification of the pattern of intraluminal fibrosis as assessed by TBLB is useful for predicting the response to treatment, clinical course and prognosis of interstitial pneumonia associated with PM/DM.     

Nonvolitional assessment of muscle endurance in idiopathic inflammatory myopathies: There is no relationship between patient‐reported fatigue and muscle fatigability

Introduction: We investigated whether muscle endurance differs between IIM patients and controls and if a relationship exists between perceived fatigue and poor muscle endurance. Methods: Quadriceps contractility, measured using femoral nerve stimulation (TwQ), and strength, measured using maximal voluntary contraction (MVCQ), were assessed in 20 IIM patients and matched controls. Quadriceps endurance was assessed using repetitive electrical stimulation (3 minutes). Time for force to fall to 70% initial force was determined (T70). Reported fatigue was measured using the FACIT‐F/Fatigue Severity Scales. Results: TwQ and MVCQ were lower and perceived fatigue greater for patients. There was no difference in T70 between groups. No relationships were observed between perceived fatigue and endurance (T70). Conclusions: IIM patients reported more fatigue and were weaker than controls, but there was no difference in muscle endurance. Endurance and strength were unrelated to reported fatigue measures. Mechanisms driving perceived IIM fatigue are likely to be multifactorial. Muscle Nerve 50 : 401–406, 2014     

“Give me strength” – inflammatory muscle disease in children

Juvenile dermatomyositis is the most common idiopathic inflammatory myopathy of childhood. Management focuses on early aggressive suppression of inflammation to induce sustained remission and prevent complications such as muscle contractures or calcinosis. Advances in diagnostic modalities and treatment have led to improved mortality and morbidity, but long-term risks remain significant. Early disease recognition with appropriate referral and management by a specialist multidisciplinary team is crucial. This review focuses on juvenile dermatomyositis including differential diagnosis from other conditions causing muscle weakness in children.     

Treatment of inflammatory myopathy: emerging therapies and therapeutic targets

Despite the lack of placebo-controlled trials, glucocorticoids are considered the mainstay of initial treatment for idiopathic inflammatory myopathy and myositis-associated interstitial lung disease. Glucocorticoid-sparing agents are often given concomitantly with other immunosuppressive agents, particularly in patients with moderate or severe disease. First-line conventional immunosuppressive drugs include either methotrexate or azathioprine, and when they fail, more aggressive therapy includes mycophenolate mofetil, tacrolimus or cyclosporine, intravenous immunoglobulin, rituximab, or cyclophosphamide, used alone or in various combinations. Further investigations are required to assess the role of more novel therapies in the treatment of myositis and myositis-associated interstitial lung disease.