The association of colitis ulcerosa and coeliakia with idiopathic inflammatory myopathy

2014 ◽  
Vol 155 (26) ◽  
pp. 1033-1038 ◽  
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Katalin Dankó
Keyword(s):  
Ulcerative Colitis ◽  
Celiac Disease ◽  
General Population ◽  
Rare Case ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
International Literature ◽  
Inflammatory Myopathies ◽  
Immune Mediated

The authors discuss a rare case of a 25-year-old female patient having dermatomyositis associated with celiac disease and ulcerative colitis. The idiopathic inflammatory myopathies are systemic, chronic, immune-mediated diseases characterized by proximal, symmetrical muscle weakness. Many examples from the literature refer that celiac disease occurs more often in patients with myositis than in the general population, but its association with ulcerative colitis is a real rarity in the international literature. Orv. Hetil., 2014, 155(26), 1033–1038.

scholarly journals Series of clinical cases of patients with idiopathic inflammatory myopathy and interstitial lung disease from the registry of idiopathic inflammatory myopathies of the Argentine Society of Rheumatology

2020 ◽  
pp. 12-17
Author(s):  
Y.M. Ponce ◽  
M.M. Zalazar ◽  
A.D. García Coello ◽  
O.L. Rillo
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inflammatory Myopathy ◽  
Clinical Manifestations ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Disease Prognosis ◽  
Immune Mediated ◽  
Necrotizing Myositis

Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TI-F1γ respectively).

scholarly journals A rare case of inflammatory myopathy

2020 ◽  
Vol 7 (4) ◽  
pp. 701
Author(s):  
Praveen M. P. ◽  
Lokesh Shanmugam ◽  
Akshay Prashanth
Keyword(s):  
Rare Case ◽  
Clinical Presentation ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Treatment Procedure ◽  
Inflammatory Myositis ◽  
History Of ◽  
Systemic Rheumatic Diseases

Idiopathic inflammatory myopathies (IIMs) happened to be the group of heterogeneous, systemic rheumatic diseases including adult polymyositis (PM), adult dermatomyositis (DM), myositis accompanied with another connective disease or cancer. A 52 years old male patient with known history of type 2 diabetes mellitus presented with complaints of muscle pain and swelling over left arm. These cases was successfully treated by using Corticosteroids. This cases study described the clinical presentation and features of inflammatory myositis. Although this is a rare case, its clinical features and treatment procedure helps in management of similar cases.

Autoantibody testing in idiopathic inflammatory myopathies

2019 ◽  
Vol 19 (4) ◽  
pp. 284-294 ◽  
Author(s):  
Anke Rietveld ◽  
Johan Lim ◽  
Marianne de Visser ◽  
Baziel van Engelen ◽  
Ger Pruijn ◽  
...  
Keyword(s):  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Methodological Issues ◽  
Antibody Testing ◽  
Histological Features ◽  
Diagnosis And Classification ◽  
Insight Into

The diagnosis and classification of idiopathic inflammatory myopathies are based mainly on clinical and histological features. The discovery of myositis-specific and myositis-associated antibodies has simplified the (sub)classification of inflammatory myopathies. Patients suspected of having an idiopathic inflammatory myopathy should undergo routine antibody testing to gain more insight into distinct phenotypes, comorbidities, treatment response and prognosis. Furthermore, autoantibody testing can help in patients with atypical patterns of weakness or with an unresolved limb-girdle myopathic phenotype, or interstitial lung disease. However, some important technical and methodological issues can hamper the interpretation of antibody testing; for example, some antibodies are not included in the widely available line blots. We aim to provide a practical review of the use of autoantibody testing in idiopathic inflammatory myopathies in clinical practice.

Juvenile Dermatomyositis: Advances in Pathogenesis, Assessment, and Management

2021 ◽  
Vol 17 ◽  
Author(s):  
Alexander K.C. Leung ◽  
Joseph M Lam ◽  
Saud Alobaida ◽  
Kin Fon Leong ◽  
Alex H.C. Wong
Keyword(s):  
Juvenile Dermatomyositis ◽  
English Literature ◽  
Oral Prednisone ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Muscle Disease ◽  
High Dose ◽  
Idiopathic Inflammatory Myopathies ◽  
Aggressive Treatment ◽  
Inflammatory Myopathies

Background: Juvenile dermatomyositis is the most common inflammatory myopathy in the pediatric age group and a major cause of mortality and morbidity in individuals with childhood rheumatic diseases. Mounting evidence suggests that early diagnosis and timely aggressive treatment are associated with better outcomes. Objective: This purpose of this article is to provide readers with an update on the evaluation, diagnosis, and treatment of juvenile dermatomyositis. The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to the English literature. The information retrieved from the above search was used in the compilation of the present article. Methods: A PubMed search was performed in Clinical Queries using the key term “juvenile dermatomyositis” as search engine. Results: Juvenile dermatomyositis is a chronic autoimmune inflammatory condition characterized by systemic capillary vasculopathy that primarily affects the skin and muscles with possible involvement of other organs. In 2017, the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR) developed diagnostic criteria for juvenile idiopathic inflammatory myopathies and juvenile dermatomyositis. In the absence of muscle biopsies which are infrequently performed in children, scores (in brackets) are assigned to four variables related to muscle weakness, three variables related to skin manifestations, one variable related to other clinical manifestations, and two variables related to laboratory measurements to discriminate idiopathic inflammatory myopathies from non-idiopathic inflammatory myopathies as follows: objective symmetric weakness, usually progressive, of the proximal upper extremities (0.7); objective symmetric weakness, usually progressive, of the proximal lower extremities (0.8); neck flexors relatively weaker than neck extensors (1.9); leg proximal muscles relatively weaker than distal muscles (0.9); heliotrope rash (3.1); Gottron papules (2.1); Gottron sign (3.3); dysphagia or esophageal dysmotility (0.7); presence of anti-Jo-1 autoantibody (3.9); and elevated serum levels of muscle enzymes (1.3). In the absence of muscle biopsy, a definite diagnosis of idiopathic inflammatory myopathy can be made if the total score is ≥7.5. Patients whose age at onset of symptoms is less than 18 years and who meet the above criteria for idiopathic inflammatory myopathy and have a heliotrope rash, Gottron papules or Gottron sign are deemed to have juvenile dermatomyositis. The mainstay of therapy at the time of diagnosis is high-dose corticosteroid (oral or intravenous) in combination with methotrexate. Conclusion: For mild to moderate active muscle disease, early aggressive treatment with high-dose oral prednisone alone or in combination with methotrexate is the cornerstone of management. Pulse intravenous methylprednisolone is often preferred to oral prednisone in more severely affected patients, patients who respond poorly to oral prednisone, and those with gastrointestinal vasculopathy. Other steroid-sparing immunosuppressive agents such as cyclosporine and cyclophosphamide are reserved for patients with contraindications or intolerance to methotrexate and for refractory cases, as the use of these agents is associated with more adverse events. Various biological agents have been used in the treatment of juvenile dermatomyositis. Data on their efficacy are limited and their use in the treatment of juvenile dermatomyositis is considered investigational.

Mortality in idiopathic inflammatory myopathy: results from a Swedish nationwide population-based cohort study

2017 ◽  
Vol 77 (1) ◽  
pp. 40-47 ◽  
Author(s):  
Gerd Cecilie Dobloug ◽  
John Svensson ◽  
Ingrid E Lundberg ◽  
Marie Holmqvist
Keyword(s):  
General Population ◽  
Causes Of Death ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Population Based ◽  
First Year ◽  
Inflammatory Myopathies ◽  
Cumulative Mortality ◽  
Common Causes

Patients with idiopathic inflammatory myopathies (IIMs) suffer an increased burden of comorbidities, but data on mortality in recently diagnosed IIM are conflicting. Also, little is known when, if ever, in relation to IIM diagnosis, mortality is increased.MethodsA population-based IIM cohort of patients diagnosed between 2002 and 2011 and general population comparators were identified using healthcare registers. They were linked to the cause of death register for follow-up.Results224 (31%) of the 716 patients with IIM and 870 (12%) of the 7100 general population died during follow-up. This corresponded to a mortality rate of 60/1000 person-years in IIM and 20/1000 person-years in the general population. The cumulative mortality at 1 year after diagnosis was 9% in IIM and 1% in the general population, and increased in both IIM and the general population with time. The overall hazard ratio (HR) 95%CI of death comparing IIM with the general population was 3.7 (3.2 to 4.4). When we stratified on time since diagnosis, we noted an increase in mortality already within the first year of diagnosis compared with the general population, HR 9.6 (95% CI 6.9 to 13.5). This HR then plateaued around 2 after >10 years with the disease, although the estimates were not statistically significant. Malignancies, diseases of the circulatory and respiratory system were common causes of death.ConclusionMortality is increased in patients with contemporary IIM. The increased mortality was noted within a year of diagnosis, which calls for extra vigilance during the first year of IIM diagnosis.

Biological therapy in idiopathic inflammatory myopathies

2014 ◽  
Vol 155 (1) ◽  
pp. 3-10
Author(s):  
Levente Bodoki ◽  
Melinda Nagy-Vincze ◽  
Zoltán Griger ◽  
Andrea Péter ◽  
Csilla András ◽  
...  
Keyword(s):  
T Cells ◽  
Muscle Weakness ◽  
Biological Therapy ◽  
Therapeutic Options ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Progressive Muscle Weakness ◽  
Immune Mediated ◽  
Novel Therapeutic

Idiopathic inflammatory myopathies are systemic, immune-mediated diseases characterized by proximal, symmetrical, progressive muscle weakness. The aim of this work is to give an overview of the biological therapy used in the treatment of idiopathic inflammatory myopathies. The authors also focus on novel results in the therapy directed against the B- and T-cells. They emphasize the importance of new trials in these diseases which may lead to the introduction of novel therapeutic options in these disorders. Orv. Hetil., 2014, 155(1), 3–10.

scholarly journals Acute Coronary Syndrome in Idiopathic Inflammatory Myopathies: A Population-based Study

2019 ◽  
Vol 46 (11) ◽  
pp. 1509-1514 ◽  
Author(s):  
Valérie Leclair ◽  
John Svensson ◽  
Ingrid E. Lundberg ◽  
Marie Holmqvist
Keyword(s):  
Acute Coronary Syndrome ◽  
General Population ◽  
Incidence Rate ◽  
Competing Risk ◽  
First Year ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Risk Of Death ◽  
Coronary Syndrome ◽  
Increased Risk

Objective.Evidence suggests an increased risk of cardiovascular (CV) diseases, including acute coronary syndrome (ACS), in idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the risk of ACS in an incident IIM cohort compared to the general Swedish population.Methods.A cohort of 655 individuals with incident IIM and 6813 general population comparators were identified from national registries. IIM subjects were diagnosed from 2002 to 2011. Followup started at IIM diagnosis and corresponding date in the general population. ACS, CV comorbidities, and CV risk factors were defined using International Classification of Diseases codes. Incidence rates including 95% CI were calculated. Cox proportional hazards models were used to compare the risk of ACS in patients with IIM and the general population. The competing risk of death was accounted for using competing risk regression models.Results.The incidence rate of ACS in IIM was higher than in the general population, particularly within the first year of diagnosis and in older individuals. The overall ACS incidence rate in IIM was 15.6 (95% CI 11.7–20.4) per 1000 person-years, with an HR of 2.4 (95% CI 1.8–3.2) compared with the general population. When accounting for the competing risk of death, the risk of ACS in IIM remained increased with a cumulative incidence of 7% at 5 years compared to 3.3% in the general population.Conclusion.IIM individuals are at higher risk of ACS, particularly within the first year after diagnosis.

Histopathological features of the idiopathic inflammatory myopathies

2018 ◽  
Author(s):  
Marianne de Visser and Eleonora M.A. Aronica
Keyword(s):  
Inclusion Body ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Inflammatory Myopathy ◽  
Adult Patients ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Histopathological Features ◽  
Muscle Biopsies

In adult patients with presumed idipathic inflammatory myopathy (IIM) without a characteristic and diagnostic dermatomyositis rash, muscle biopsy is mandatory to confirm the IIM diagnosis and to exclude a myopathy which would not respond to glucocorticoids or other immunosuppressants, including inclusion body myositis. This chapter discusses when, where, and how to undertake muscle biopsies, when to repeat them, how to interpret their results, and how these relate to IIM subtypes and disease processes.

scholarly journals 13. Idiopathic inflammatory myopathy, everything it should not be: asymmetrical, normal CK, high fever

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Emer Gates ◽  
Ben Faber ◽  
Steve Hepple ◽  
Harsha Gunawardena
Keyword(s):  
Clinical Features ◽  
Muscle Biopsy ◽  
Lower Limb ◽  
Conflicts Of Interest ◽  
Vastus Lateralis ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Thigh Pain ◽  
Immunomodulatory Therapy ◽  
Immune Mediated

Abstract Introduction Myositis can be infective, metabolic or immune-mediated. Idiopathic inflammatory myopathy, which is immune-mediated, tends to be subacute, with symmetrical symptoms, overlap clinical features and positive autoimmune serology. We present a case of acute onset lower limb seronegative inflammatory myopathy with a normal creatinine kinase (CK), a marked acute phase response that responded promptly to immunomodulatory therapy. Case description A 68-year-old man presented with a 2-week history of lower limb pain with subjective weakness, on a background of well-controlled type 2 diabetes mellitus and hypertension. He was admitted with worsening symptoms of marked left thigh pain, night sweats, and fevers. On admission, he had swinging pyrexia (above >39 °C) while remaining haemodynamically stable. He had focal tenderness over the left anterolateral thigh, with a good range of movement, normal power and no signs of focal collection or cellulitis. There were no extra-muscular features to suggest systemic infection or overlap connective tissue disease. Bloods showed C-reactive protein (CRP) 225, normal CK 212 and negative blood cultures. X-rays knee, femur and pelvis were normal. Magnetic resonance imaging (MRI) on T2, fat-suppressed STIR sequences demonstrated increased signal/oedema both thighs throughout the anterior muscle compartment and along the fascial plane, notably most severe in the left vastus lateralis. He was treated empirically for infective myositis. Despite 14 days of broad-spectrum antibiotics, he remained febrile with persistently elevated CRP. There was no focal collection, lymphadenopathy or occult malignancy on CT abdomen and pelvis. Trans-oesophageal echocardiogram showed no evidence of infective endocarditis but revealed incidental moderate aortic stenosis. The patient described persistent now bilateral thigh pain with continued normal CK and high CRP. Full autoimmune screen (ANA, ANCA, ACE and complement studies) was negative. Despite negative nuclear and cytoplasmic HEp-2 immunofluorence, extended myositis immunoblot was negative. Muscle biopsy from the left vastus lateralis demonstrated inflammation within the perimysium and perivasculature. In view of biopsy findings and no response to anti-microbial therapy, prednisolone (0.5mg/kg) with significant clinical response (resolution of fever and pain) with concurrent normalisation in CRP. The patient remains in remission following steroid reduction with no additional immunomodulatory therapy required. Discussion We report a case of idiopathic inflammatory myopathy presenting with predominantly asymmetrical symptoms, normal CK, marked inflammatory response and negative myositis autoantibodies. Diagnosis was confirmed on MRI and muscle biopsy. The normal CK can be explained by the histology demonstrating inflammation in perivascular regions and around muscle fibres, rather than inflammation or necrosis in the muscle fascicles and fibres themselves. Idiopathic inflammatory myopathy including sporadic inclusion-body myositis, dermatomyositis, overlap CTD myositis and polymyositis/necrotising myopathy subsets are distinguishable based on clinical features, autoantibodies, MRI and biopsy features. The table below summarises the atypical aspects of this case. Differential diagnoses for this case include atypical infection, sarcoid myopathy and amyloid myonecrosis secondary to diabetes. Table: Features of typical idiopathic inflammatory myopathy compared with this atypical case.Idiopathic inflammatory myopathiesOur patient- typical featuresOur patient- atypical featuresSymptomsPain, fever, weakness.Pain and fever.Normal power.Clinical distributionSymmetrical, proximal muscle groups.Predominantly asymmetrical (worse on left), only in thighs.AntibodiesMyositis associated autoantibodiesSeronegativeMuscle enzymesElevated CKNormal CK.Inflammatory markersNormal to slightly elevated CRPMarkedly raised CRP and WCC.MR imaging resultsFocal muscle oedema in affected musclesDiffuse and speckled muscle oedema Key learning points Early idiopathic inflammatory myopathy can have inflammation around the muscle fascicles in the perimysium. Normal CK does not rule out a diagnosis of idiopathic inflammatory myopathy. Idiopathic inflammatory myopathies can present atypically with fevers >39 °C, significantly raised inflammatory markers, and asymmetrical symptoms and MRI findings. In the absence of overlap features, normal CK and negative serology, MRI and biopsy can delineate the type of myositis and direct management. Conflicts of interest The authors have declared no conflicts of interest.

Clinical Characteristics of Immune-Mediated Necrotizing Myopathy with Autoantibodies Recognizing the Signal Recognition Particle: A Retrospective Study of a Chinese Cohort

2021 ◽  
Author(s):  
Qi Tang ◽  
Jinshen He ◽  
Feng Li ◽  
Jinwei Chen ◽  
Jing Tian ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Inflammatory Myopathy ◽  
Signal Recognition Particle ◽  
Idiopathic Inflammatory Myopathy ◽  
Lower Limbs ◽  
Hunan Province ◽  
Signal Recognition ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Chinese Cohort

Abstract Objective: Immune-mediated necrotizing myopathy (IMNM) with autoantibodies recognizing the signal recognition particle (SRP) patients tend to have prominent proximal weakness and infrequent extra-muscular involvement, especially interstitial lung disease (ILD). However, we reported a Chinese cohort of anti-SRP IMNM patients with relatively more frequent ILD.Methods: Anti-SRP IMNM patients from September 2016 to November 2019 were included according to the most recent European Neuromuscular Center criteria for IMNM. All sera for anti-SRP autoantibody and other myositis-related autoantibodies detection were obtained before the treatment initiation. Muscle strength, coexisting autoimmunity, complications including ILD, treatment and follow-up outcomes were also recorded. Univariate logistic regression was performed to determine variables predicting bad outcomes.Results: Of 271 patients with idiopathic inflammatory myopathy tested, we diagnosed 23 (8.5%) patients with anti-SRP IMNM. Muscle weakness was presented in 23 patients (100%) and generally worse in the lower limbs. ILD was observed in 50% anti-SRP IMNM patients. Predictor of bad outcomes identified by univariate logistic regression analysis was complicated ILD (odds ratio, 3.8).Conclusion: ILD tends to be more frequent in this Chinese anti-SRP IMNM cohort from Hunan province. Complicated ILD represents a risk factor for bad outcomes for anti-SRP IMNM.

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