A case study of gait compensations for hip muscle weakness in idiopathic inflammatory myopathy

Polymyositis is an idiopathic inflammatory myopathy, characterized by proximal muscle weakness and sometimes extramuscular manifestations. We report the case of a 51-year-old male, with history of complete heart block, which required pacemaker implantation, and subsequently heart failure, presenting to the emergency department with worsening of dyspnea and peripheral edema. He was admitted to the Internal Medicine ward with acute heart failure and started on diuretic therapy. During hospitalization, he was discovered to have marked rhabdomyolysis. Examination revealed proximal symmetrical muscle weakness and arthralgia. The immunological study, electromyography and muscle biopsy confirmed polymyositis. The patient was started on prednisolone with clinical improvement and resolution of rhabdomyolysis. The presence of conduction defect, ventricular dysfunction, mitral valve regurgitation, segmental hypokinesia (myocardial scintigraphy without perfusion defects) and pulmonary hypertension, as well as elevated troponin with improvement after specific therapy, points to cardiac involvement. Polymyositis is a rare entity, with an insidious evolution and a myriad of extramuscular features that can mimic other conditions. In particular, cardiac involvement may be the first and only recognized manifestation. The key point for the diagnosis is to contemplate the possibility of polymyositis.

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Statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like features: A case report

SAGE Open Medical Case Reports ◽

10.1177/2050313x20984120 ◽

2020 ◽

Vol 8 ◽

pp. 2050313X2098412

Author(s):

Darosa Lim ◽

Océane Landon-Cardinal ◽

Benjamin Ellezam ◽

Annie Belisle ◽

Annie Genois ◽

...

Keyword(s):

Creatine Kinase ◽

Muscle Weakness ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Intravenous Immunoglobulins ◽

Proximal Muscle Weakness ◽

Proximal Muscle ◽

Therapeutic Implications ◽

Necrotizing Myopathy ◽

Immune Mediated

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) immune-mediated necrotizing myopathy is a subtype of idiopathic inflammatory myopathy which may be associated with statin exposure. It presents with severe proximal muscle weakness, high creatine kinase levels and muscle fiber necrosis. Treatment with intravenous immunoglobulins and immunosuppressants is often necessary. This entity is not commonly known among dermatologists as there are usually no extramuscular manifestations. We report a rare case of statin-associated anti-HMGCR immune-mediated necrotizing myopathy with dermatomyositis-like cutaneous features. The possibility of anti-HMGCR immune-mediated necrotizing myopathy should be considered in patients with cutaneous dermatomyositis-like features associated with severe proximal muscle weakness, highly elevated creatine kinase levels and possible statin exposure. This indicates the importance of muscle biopsy and specific autoantibody testing for accurate diagnosis, as well as significant therapeutic implications.

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Role of Panchakarma in the Management of Masavritavata w.r.t Dermatomyositis - A Case Study

International Journal of Ayurvedic Medicine ◽

10.47552/ijam.v11i4.1666 ◽

2020 ◽

Vol 11 (4) ◽

pp. 780-787

Author(s):

Imtiaz Hossain Md ◽

Mahesh M Parappagoudra ◽

Chimanda L Kamar

Keyword(s):

Immune System ◽

Skeletal Muscles ◽

Inflammatory Myopathy ◽

Signs And Symptoms ◽

Idiopathic Inflammatory Myopathy ◽

Autoimmune Disorder ◽

Muscular Tissue ◽

Small Blood Vessel

Dermatomyositis (DM) is the most frequently occurring Idiopathic Inflammatory Myopathy (IIM) of skeletal muscles causing proximal muscles weakness. The average age at which this disease affects is 40 years and almost twice as many women are affected as men. Its prevalence rate is 2-10 per 1,00,000 in the general population. The actual cause is unknown but the disease has much common with autoimmune disorder in which your immune system mistakenly attacks our body tissue. Small blood vessel in muscular tissue are particularly affected in dermatomyositis. In this disease the ESR and CPK is usually raised. Due to similarity in the signs and symptoms, we can correlate this disease with Mamsavrita-Vata and its treatment can be planned according to it. Here is the case study of 40 years old female patient, diagnosed as Dermatomyositis since 3 months. Patient admitted in Panchakarma ward of Parul Ayurved Hospital, Vadodara. The Panchakarma procedures like Udvartana, Nitya Virechana, Basti Chikitsa, Shasti Shali Pinda Sweda and Shamana Aushadi like Cap Palsineuron and Guduchi Rasayana etc. are given. At the end of the treatment marked improvements were seen in the patient, like reduced ESR and CPK levels. Also the symptoms of the disease reduced significantly.

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Clinical manfestation of dermatomyositis and difficulties in its differential diagnosis

Bőrgyógyászati és Venerológiai Szemle ◽

10.7188/bvsz.2021.97.3.3 ◽

2021 ◽

Vol 97 (3) ◽

pp. 145-149

Author(s):

Ágnes Kinyó ◽

◽

Anita Hanyecz ◽

Rolland Gyulai

Keyword(s):

Differential Diagnosis ◽

Muscle Weakness ◽

Early Recognition ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Muscle Inflammation ◽

Skin Symptoms ◽

Limb Muscles

Dermatomyositis (DM) is an idiopathic inflammatory myopathy with muscle weakness and specific cutaneous findings. The proximal weakness of the limb muscles is present in the majority of the patients, in a proportion of them however the muscle inflammation is preceded by the skin symptoms with several months, and in 40%, skin symptoms are the only manifestations of the disease. While, there may have many difficulties in differential diagnosis of DM without myositis, dermatologists have a major role in the early recognition of the disorder and in the initiation of the proper therapy

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Rituximab and intravenous immunoglobulin treatment in PM/Scl antibody-associated disease: case-based review

Rheumatology International ◽

10.1007/s00296-021-05075-z ◽

2022 ◽

Author(s):

Denesh Srikantharajah ◽

Mark E. Lloyd ◽

Patrick D. W. Kiely

Keyword(s):

Intravenous Immunoglobulin ◽

Clinical Features ◽

Muscle Weakness ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Immunoglobulin Therapy ◽

Rapid Onset ◽

Calcinosis Cutis ◽

Intravenous Immunoglobulin Therapy ◽

Disease Case

AbstractAutoantibodies to the 75-kDa and 100-kDa subunits of the PM/Scl nucleolar protein complex are associated with an overlap syndrome, manifesting with clinical features of systemic sclerosis and idiopathic inflammatory myopathy. We describe the diverse clinical features in a series of 4 cases with anti-PM/Scl-75 and/or anti-PM/Scl-100 antibodies, including severe proximal muscle weakness, oesophageal dysfunction, respiratory weakness requiring mechanical ventilation, Raynaud’s, calcinosis cutis, sclerodactyly and critical digital ischaemia. Despite the severity of striated and oesophageal muscle weakness, all patients responded very well to immune suppression, and calcinosis cutis in one case regressed substantially. We highlight the efficacy of Rituximab and intravenous immunoglobulin therapy (IVIg) in these cases, enabling return to normal muscle function within six months. Rituximab was preferentially chosen for cases with hyper-gammaglobulinemia and multiple autoantibodies in addition to anti-PM/Scl, and IVIg was utilised for cases where a rapid onset of effect was required, such as severe ventilator-dependent respiratory muscle weakness and oesophageal dysfunction.

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Dermatomyositis as a paraneoplastic phenomenon in oesophageal cancer

BMJ Case Reports ◽

10.1136/bcr-2018-227387 ◽

2018 ◽

Vol 11 (1) ◽

pp. e227387

Author(s):

Nicholas Kevin Laidler

Keyword(s):

Muscle Weakness ◽

Clinical Symptoms ◽

Inflammatory Myopathy ◽

Oral Prednisolone ◽

Idiopathic Inflammatory Myopathy ◽

Oesophageal Adenocarcinoma ◽

Tertiary Institution ◽

Multisystem Disease ◽

Positron Emission ◽

New Diagnosis

Dermatomyositis is a multisystem disease manifesting as an idiopathic inflammatory myopathy, characterised predominantly by cutaneous and muscular abnormalities. Patients with dermatomyositis display characteristic skin changes in addition to muscle weakness. Many consider dermatomyositis a paraneoplastic syndrome, as up to 32% of patients with dermatomyositis will develop cancer. Discussed here is the case of a 69-year-old man with a new diagnosis of dermatomyositis who was referred to a tertiary institution with an 8-month history of rash, proximal muscle weakness, fatigue and weight loss. In the absence of clinical symptoms, negative cancer markers and a negative CT chest, abdomen and pelvis, positron emission tomography–CT prompted a gastroscopy, which led to the discovery of a stage T1b oesophageal adenocarcinoma. The oesophageal adenocarcinoma was treated with a gastrointestinal endoscopic mucosal resection and subsequent oesophajectomy. The patient’s dermatomyositis was treated successfully with oral prednisolone and methotrexate.

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Újabb adatok a myositisspecifikus és -asszociált antitestekről juvenilis és felnőttkori myositisekben

Orvosi Hetilap ◽

10.1556/650.2016.30404 ◽

2016 ◽

Vol 157 (30) ◽

pp. 1179-1184 ◽

Cited By ~ 1

Author(s):

Andrea Váncsa ◽

Katalin Dankó

Keyword(s):

Muscle Weakness ◽

Immunosuppressive Treatment ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

General Term ◽

Response To Treatment ◽

Disease Course ◽

Inflammatory Myopathies ◽

Disease Response ◽

Systemic Symptoms

Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179–1184.

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A rare case of NXP-2 positive dermatomyositis

Archive of Clinical Cases ◽

10.22551/2020.29.0704.10176 ◽

2020 ◽

Vol 7 (4) ◽

pp. 68-71

Author(s):

Nariman Khan ◽

Keyword(s):

Muscle Weakness ◽

Nuclear Matrix ◽

Conventional Therapy ◽

Rare Case ◽

Matrix Protein ◽

Inflammatory Myopathy ◽

Idiopathic Inflammatory Myopathy ◽

Nuclear Matrix Protein ◽

Cutaneous Manifestations ◽

Distal Muscle

Dermatomyositis is an idiopathic inflammatory myopathy with variable cutaneous manifestations. Several autoantibodies each with distinct clinical phenotypes are associated with the disease. Here we present the case of a 36-year-old Laotian woman with hypothyroidism who presented with severe proximal and distal muscle weakness, dysphagia, diffuse rash, and anasarca that was diagnosed with NXP-2 (nuclear matrix protein 2) antibody positive dermatomyositis. The patient’s hospitalization was complicated by disease resistant to conventional therapy.

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