scholarly journals Diagnosis of dermatomyositis and polymyositis: a study of 102 cases

2000 ◽  
Vol 58 (3B) ◽  
pp. 789-799 ◽  
Author(s):  
ROSANA HERMINIA SCOLA ◽  
LINEU CESAR WERNECK ◽  
DANIEL MONTE SERRAT PREVEDELLO ◽  
EDIMAR LEANDRO TODERKE ◽  
FÁBIO MASSAITI IWAMOTO
Keyword(s):  
Weight Loss ◽  
Creatine Kinase ◽  
Connective Tissue ◽  
Connective Tissue Diseases ◽  
Malignant Neoplasm ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Systemic Involvement ◽  
Perifascicular Atrophy

Patients with dermatomyositis (DM) or polymyositis (PM) were studied retrospectively. The patients were divided into four groups: definite PM 24, probable PM 19, definite DM 34 and mild-early DM 25 cases. PM patients complained more often proximal muscle weakness [p <0.01]. DM patients complained more arthralgia [p <0.05], dysphagia [p <0.03] and weight loss [p <0.04]. Five patients had a malignant neoplasm and 9 had other connective-tissue disease. DM presented higher ESR than PM [p <0.002]. PM presented more significant increase in creatine kinase (CK) [p <0.02] and in alanine aminotransferase (ALT) [p <0.001] levels. Electromyography showed myopathic pattern in 76%. Muscle biopsy was the definitive test. Perifascicular atrophy was more frequent in definite DM than in mild-early DM group [p <0.03]. CONCLUSION: A small association with connective-tissue diseases and neoplasms was found. DM and PM are clinically different. DM presents systemic involvement affecting the skin, developing more severe arthralgia, dysphagia and weight loss and presenting higher values of ESR. PM presents a restricted and more significant involvement of muscles generating more weakness complaints and higher levels of serum muscle enzymes.

scholarly journals The Pathognomonicity of Gottron’s Sign

2016 ◽  
Vol 8 (1) ◽  
Author(s):  
Heather M. Babcock BSc ◽  
Mohammed S. Osman MD PhD ◽  
Tiffany Kwok MD ◽  
Stephen Chihrin MD ◽  
Stephanie O. Keeling MD MSc ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Connective Tissue ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Connective Tissue Diseases ◽  
Proximal Muscle Weakness ◽  
Erythematous Rash ◽  
Proximal Muscle ◽  
History Of ◽  
Distal Interphalangeal

This article presents the case of a previously healthy 43-year-old female who presented with a 3-month history of progressive, symmetrical, bilateral, proximal muscle weakness accompanied by a violaceous-to-erythematous rash involving her hands, arms, thighs, chest, and face. She had conspicuous non-edematous periorbital violaceous patches with telangiectasia and prominent warm violaceous macules overlying the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints. Muscle biopsy confirmed dermatomyositis. Gottron’s sign is the most specific cutaneous finding of dermatomyositis and is present in at least 70% of patients. The lesions begin as non-palpable flat macules or patches (Gottron’s “sign”) or are firm and raised (Gottron’s “papules”), but the lesions eventually coalesce into raised non-blanching plaques that occur over bony prominences – typically the MCP, PIP, and/or distal interphalangeal joints. Gottron’s sign (and papules) are pathognomonic for dermatomyositis, although some other conditions may have similar presentations. Gottron’s sign must always be explained, as dermatomyositis may be primary or secondary to malignancy or other connective tissue diseases, and none of the conditions that make up the differential diagnosis are benign.

scholarly journals Statin-induced necrotising autoimmune myopathy and autoimmune hepatitis presenting with dysphagia

2020 ◽  
Vol 13 (2) ◽  
pp. e232391 ◽  
Author(s):  
Osama Qasim Agha ◽  
Sukhdeep Kaur ◽  
Nirmal Vijayavel
Keyword(s):  
Creatine Kinase ◽  
Autoimmune Hepatitis ◽  
Complete Resolution ◽  
Oropharyngeal Dysphagia ◽  
Liver Function Tests ◽  
Unknown Aetiology ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Autoimmune Myopathy ◽  
Smooth Muscle Antibodies

Statin-induced necrotising autoimmune myopathy (SINAM) is a rare disease characterised by proximal muscle weakness and elevated creatine kinase levels that is usually in the thousands. Anti-3-hydroxy-3-methyl glutaryl co-enzyme A reductase (HMGCR) antibodies are associated with SINAM. Autoimmune hepatitis (AIH) is an inflammatory disease of the liver that is usually of unknown aetiology but can also be associated with concurrent extrahepatic autoimmune disorders. We are reporting a case of biopsy proven AIH associated with SINAM in a patient presenting with oropharyngeal dysphagia. The patient had elevated anti-HMGCR antibodies and anti-smooth muscle antibodies. SINAM and AIH were confirmed by muscle biopsy and liver biopsy, respectively. The patient had complete resolution of his symptoms and complete normalisation of his liver function tests after 6 months of the treatment.

Tuberculosis heralding connective tissue disorder

2020 ◽  
pp. 004947552096274
Author(s):  
U Pratap ◽  
M Ravindrachari ◽  
A RamyaPriya ◽  
Pampa Ch. Toi ◽  
R Manju
Keyword(s):  
Weight Loss ◽  
Mycobacterium Tuberculosis ◽  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Lung Biopsy ◽  
Connective Tissue Diseases ◽  
Connective Tissue Disorder ◽  
Current Smoker ◽  
Tuberculosis Therapy ◽  
A Current

Connective tissue diseases and infections are amongst the causes for organising pneumonia. However, organising pneumonia preceding other connective tissue disease manifestations is rare. Mycobacterium tuberculosis is rarely associated with organising pneumonia. We report such a case. A 50-year-old diabetic male, a roadside shop keeper, a current smoker presented with fever, breathlessness, cough and weight loss for four months. Chest radiography demonstrated areas of consolidation with halo signs. Anti-nuclear antibody blot was positive for Scl-70 and Jo-1 suggestive of a syndrome of systemic sclerosis and polymyositis overlap. Fibre-optic bronchoscopy guided lung biopsy was suggestive of organising pneumonia, and broncho-alveolar lavage detected Mycobacterium tuberculosis. Mycobacterium tuberculosis should be investigated as an aetiology of organising pneumonia, as this may occur in unestablished cases of connective tissue disease even before clinical and radiological manifestations appear, as response can be achieved with anti-tuberculosis therapy alone, without additional use of systemic steroids.

scholarly journals Symptomatic muscle involvement in neurosarcoidosis: a clinicopathological study of 5 cases

2001 ◽  
Vol 59 (2B) ◽  
pp. 347-352 ◽  
Author(s):  
Rosana Herminia Scola ◽  
Lineu Cesar Werneck ◽  
Daniel Monte Serrat Prevedello ◽  
Priscila Greboge ◽  
Fábio Massaiti Iwamoto
Keyword(s):  
Side Effects ◽  
Normal Values ◽  
Clinical Course ◽  
The Other ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Clinicopathological Study ◽  
Steroid Side Effects

We report on the clinical course and histopathologic muscle alterations of five patients diagnosed with neurosarcoidosis, who underwent biopsy due to their muscle manifestations. The five patients were females and only one was less than 40 years of age. Proximal muscle weakness was presented by all and only two patients complained of myalgia. Only normal values of serum muscle enzymes were detected. Electromyography revealed diverse findings such as normal, myopathic and neuropathic patterns. Granuloma was not present in one muscle biopsy. Two patients thoroughly recovered by taking only prednisone and one patient required a methotrexate addition for 3 months before becoming asymptomatic. The other two patients received azathioprine, one due to steroid side effects but without a satisfactory evolution, and the other to strengthen the prednisone régime, with excellent results.

Immune-mediated necrotising myopathy in asymptomatic patients with high creatine kinase

2020 ◽  
Vol 13 (10) ◽  
pp. e235457
Author(s):  
Izadora Fonseca Zaiden Soares ◽  
Victoria Fernandez Comprido ◽  
Bianca Raquel Ruoh Harn Scovoli Hsu ◽  
Alzira Alves de Siqueira Carvalho
Keyword(s):  
Creatine Kinase ◽  
Muscle Biopsy ◽  
Signal Recognition Particle ◽  
Disease Onset ◽  
Delayed Diagnosis ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Asymptomatic Patients ◽  
Immune Mediated

Subacute symmetrical proximal muscle weakness and persistent elevated creatine kinase levels are typical of immune-mediated necrotising myopathy (IMNM). These conditions are accompanied by copious myofibre necrosis, degeneration and regeneration with minimal to no inflammation on muscle biopsy. We report two cases (case 1 and case 2) of asymptomatic IMNM from different families with hyperCKaemia associated with positive anti-signal recognition particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) antibodies, respectively, and we also reviewed the literature. There are only a few previous descriptions of patients with asymptomatic IMNM.The disease onset could be insidious and lead to delayed diagnosis and treatment. We recommend testing for the anti-HMGCR and anti-SRP antibodies in patients with idiopathic hyperCKaemia because they could show no symptoms of this disorder.

Polymyositis and Dermatomyositis

2019 ◽  
pp. 187-189
Author(s):  
Josephina A. Vossen
Keyword(s):  
Muscle Biopsy ◽  
Clinical Examination ◽  
Immune Suppression ◽  
Imaging Modality ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Enzyme

Chapter 42 discusses polymyositis and dermatomyositis, which are idiopathic inflammatory myopathies characterized by muscle inflammation, proximal muscle weakness, and elevated muscle enzymes. Idiopathic inflammatory myopathies represent a heterogeneous group of muscle diseases. Diagnosis is based on clinical examination, muscle enzyme laboratory values, electromyography (EMG), and muscle biopsy. MRI is the most important imaging modality used in diagnosis and management. Radiographs and CT may detect calcinosis. Treatment is primarily by immune suppression.

scholarly journals Interstitial lung disease and inflammatory myopathy in antisynthetase syndrome with PL-12 antibody

2018 ◽  
pp. bcr-2018-226119
Author(s):  
Ameen Jubber ◽  
Mudita Tripathi ◽  
James Taylor
Keyword(s):  
Weight Loss ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Muscle Weakness ◽  
Inflammatory Myopathy ◽  
Antisynthetase Syndrome ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Progressive Dyspnoea ◽  
Sicca Symptoms

We report the case of an 80-year-old Caucasian man with PL-12 antibody positive antisynthetase syndrome. He presented with progressive dyspnoea and weight loss, later developing dysphagia, mild proximal muscle weakness and mild sicca symptoms. Investigations revealed interstitial lung disease, inflammatory myopathy and an immunology profile consistent with PL-12 antisynthetase syndrome. Prednisolone and cyclophosphamide resulted in a significant improvement of all his symptoms.

scholarly journals Rituximab in the treatment of immune-mediated necrotizing myopathy: a review of case reports and case series

2021 ◽  
Vol 14 ◽  
pp. 175628642199891
Author(s):  
Anji Xiong ◽  
Guancui Yang ◽  
Zhuoyao Song ◽  
Chen Xiong ◽  
Deng Liu ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Case Reports ◽  
Signal Recognition Particle ◽  
High Dose ◽  
Signal Recognition ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Coa Reductase

Immune-mediated necrotizing myopathy (IMNM) is a group of immune-related myopathies characterized by progressive proximal muscle weakness, extremely high serum creatine kinase (CK) levels, and necrotic muscle fibers with a relative lack of inflammation. Treatment of IMNM is challenging, with most cases refractory to high-dose steroids in combination with multiple immunotherapies. The role of rituximab (RTX) for IMNM has been explored in isolated case reports and small series. The aim of this article was to perform a literature review of patients with IMNM treated with RTX and to evaluate RTX efficacy and safety. A total of 34 patients with IMNM were reviewed: 52.9% (18/34) with anti-signal recognition particle (SRP) antibodies and 47.1% (16/34) with anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibodies. Patient age at onset varied from 11 years to 81 years (mean 41 years). The majority of patients presented as a severe proximal muscle weakness and the peak level of CK varied from 3900 IU/L to 56,000 IU/L (mean 18,440 IU/L). Prior to RTX administration, all patients were treated with high-dose steroids and most were treated with multiple immunotherapies. The reason for initiating RTX was that 64.7% (22/34) of patients showed no improvement after previous treatments, and 35.3% (12/34) of patients relapsed when attempting to wean steroids or other immunosuppressive agents. With regard to RTX efficacy, 61.8% (21/34) of patients presented a response to RTX. Our data may support the use of RTX as an effective treatment strategy against IMNM resistant to steroids and multiple immunotherapies. Meanwhile, RTX as a first-line therapy could be a choice in IMNM, particularly in African Americans with anti-SRP antibody-positive subsets. ANA, antinuclear antibody; CK, creatine kinase; HMGCR, 3-hydroxy-3-methylglutaryl-CoA reductase; IMNM, immune-mediated necrotizing myopathy; MAC, membrane attack complex; MHC-I, major histocompatibility complex-I; RTX, rituximab; SRP, signal recognition particle.

scholarly journals Statin and fibrate associed myopathy: study of eight patients

2004 ◽  
Vol 62 (2a) ◽  
pp. 257-261 ◽  
Author(s):  
Alzira A. Siqueira Carvalho ◽  
Ürsula Waleska Poti Lima ◽  
Raul Alberto Valiente
Keyword(s):  
Creatine Kinase ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Clinical Course ◽  
Lipid Lowering ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Infiltration ◽  
Lipid Lowering Drugs ◽  
Ragged Red Fibers

Lipid-lowering drugs have been occasionally associated with neuromuscular symptoms and muscle biopsy changes. We reported the clinical course and the muscle biopsy in eight patients with hyperlipoproteinemia, treated with lipid -lowering drugs (statins/fibrates). Five patients had myalgias while; in two cases there was proximal muscle weakness. All patients became asymptomatic after the withdrawal of the drug, although creatine kinase remained elevated. We performed muscle biopsy in six cases from three months to two years after suspension of the drug. We found variation in fibers diameters in all cases, with necrosis of fibers in five cases, inflammatory infiltration in one case, the presence of vacuolated fiber in one patient and ragged-red fibers in three subjects. We concluded that although the muscle biopsy findings were not specific, the prolonged use of statins and or fibrates might induce a chronic myopathy even in the absence of symptoms.

scholarly journals Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Naomi Fei ◽  
Sarah Sofka
Keyword(s):  
Polycythemia Vera ◽  
Muscle Weakness ◽  
Case Reports ◽  
Inflammatory Myopathy ◽  
Primary Myelofibrosis ◽  
Clinical Findings ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
First Case

Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association.

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