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1.
Pan Afr Med J ; 38: 101, 2021.
Artigo em Francês | MEDLINE | ID: mdl-33889267

RESUMO

Dermatomyositis (DM) and polymyositis (PM) are rare but serious conditions. The purpose of this study was to investigate, by a review of hospital cases, their epidemiological, clinical and evolutionary profile. We conducted a retrospective study over a 15-year period, between January 2004 and December 2019. All cases with possible or definite diagnosis according to Bohan and Peter's criteria were retained. A total of 14 patients were enrolled (8 DM and 6 PM), with an average age of 48.7 years. Sex ratio was 13F/ 1H. General signs were reported in 71% of cases. Motor deficit affected the girdle muscles in 71% of cases; 85.7% of patients had arthralgia and 14% arthritis. Erythema and periorbital edema were the predominant skin signs. Patients' assessment showed increased sedimentation rate in all cases and increased muscle enzymes in 80% of cases. Antinuclear antibodies were positive in 63% of cases. Muscle biopsy objectified inflammatory myositis in 75% of cases. Heart disease was reported in 14% of cases and lung disease in 21%. Cancer was found in 21.4% of cases. All patients received corticosteroid therapy. Improvement was reported in 88% of patients, with a relapse in 4 patients. In our context, DM is more frequent than PM, with a clear female predominance. Pulmonary disease is a heavy complication. Its association with cancers occurs commonly, hence the need for a systematic cancer screening at diagnosis and follow-up.


Assuntos
Dermatomiosite/epidemiologia , Miosite/epidemiologia , Polimiosite/epidemiologia , Adolescente , Corticosteroides/administração & dosagem , Adulto , Idoso , Biópsia , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Edema/epidemiologia , Eritema/epidemiologia , Eritema/etiologia , Feminino , Cardiopatias/epidemiologia , Humanos , Masculino , Pessoa de Meia-Idade , Marrocos , Miosite/diagnóstico , Miosite/tratamento farmacológico , Neoplasias/epidemiologia , Polimiosite/diagnóstico , Polimiosite/tratamento farmacológico , Estudos Retrospectivos , Adulto Jovem
2.
BMJ Case Rep ; 14(4)2021 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-33849864

RESUMO

The COVID-19 pandemic caused by the SARS-CoV-2 virus has affected millions of people around the globe. The most common presentation of COVID-19 is fever and upper and lower respiratory tract infection. Myalgia is fairly common in the prodromal phase of the viral illness which self-resolves. There is very scant literature on autoimmune myositis triggered by COVID-19 infection. We report a case of SARS-CoV-2 infection, who presented with progressive muscle weakness with rhabdomyolysis and necrotizing autoimmune myopathy on muscle biopsy. This case report imposes awareness of musculoskeletal autoimmune processes triggered by COVID-19 which requires clinical suspicion for early diagnosis and initiation of treatment.


Assuntos
Doenças Autoimunes/virologia , Miosite/virologia , Anticorpos Antivirais/sangue , Doenças Autoimunes/terapia , Diagnóstico Diferencial , Feminino , Humanos , Imunoglobulina G/sangue , Pessoa de Meia-Idade , Debilidade Muscular/virologia , Mialgia/virologia , Miosite/terapia , Necrose/virologia , Prednisona/uso terapêutico , Rabdomiólise/virologia
3.
Yonsei Med J ; 62(5): 424-430, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33908213

RESUMO

PURPOSE: To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients. MATERIALS AND METHODS: We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Immunoblot assay with Euroline strip (EUROIMMUN, Germany) was performed using the sera of dermatomyositis (DM, n=56), polymyositis (PM, n=45), amyopathic DM (n=5), DM sine dermatitis (n=1), and immune mediated necrotizing myopathy (n=1) patients. Patients were classified based on two classifications: 2017 EULAR/ACR and novel clinicoserologic classification. RESULTS: According to 2017 EULAR/ACR criteria, DM and PM were the most and the second most frequent entities. Overlap myositis was the major entity of IIM, and the frequency of PM was significantly lower when applying clinicoserologic classification criteria. Sixty-nine (63.9%) patients had one or more MSA, and 61 (56.5%) patients had one or more MAA. Interstitial lung disease was closely associated with anti-MDA5 and anti-ARS, and DM-specific skin lesions were frequently observed in patients with anti-TIF1γ, anti-SRP, and anti-MDA5. CONCLUSION: The clinicoserologic criteria based on MSA/MAA positivity could reflect more precise clinical features of IIM. Establishment of a laboratory system routinely available to screen for MSA/MAA status will be beneficial to provide precise diagnosis and proper management of IIM patients.


Assuntos
Doenças Autoimunes , Miosite , Adolescente , Adulto , Autoanticorpos , Humanos , Miosite/diagnóstico , República da Coreia , Estudos Retrospectivos
4.
Chest ; 159(3): e151-e154, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33678283

RESUMO

CASE PRESENTATION: A 64-year-old previously healthy man presented with 8 weeks of progressive dyspnea on exertion and cough. Prior to presentation, the patient was able to bicycle > 60 miles per week and work full-time in a home improvement store. He was up-to-date with age-appropriate cancer screening and immunizations, and home medications included famotidine for reflux and nonsteroidal antiinflammatories for osteoarthritis, both as-needed. He had no significant respiratory exposure, aside from previous work as an electrician. His symptoms began in mid-February 2020 amid the coronavirus disease 2019 pandemic, although he had no known exposure to the virus.


Assuntos
/diagnóstico , Frutose-Bifosfato Aldolase/sangue , Glucocorticoides/administração & dosagem , Pulmão/diagnóstico por imagem , Miosite , Troca Plasmática/métodos , Rituximab/administração & dosagem , Treonina-tRNA Ligase/imunologia , Autoanticorpos/sangue , Diagnóstico Diferencial , Progressão da Doença , Humanos , Imunossupressores/administração & dosagem , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/fisiopatologia , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/diagnóstico , Debilidade Muscular/etiologia , Miosite/sangue , Miosite/diagnóstico , Miosite/fisiopatologia , Miosite/terapia , Oxigenoterapia/métodos , Prognóstico , Resultado do Tratamento
5.
Rev Med Suisse ; 17(729): 482-486, 2021 Mar 10.
Artigo em Francês | MEDLINE | ID: mdl-33689244

RESUMO

Inflammatory myopathies are characterized by muscle weakness, occasionally pain, and an inflammatory infiltrate of the skeletal muscle. Despite the description of novel autoantibodies and advances in imaging, the lack of universal consensus on classification criteria and routine clinical use of validated outcome measures has direct implications on treatment trials and observational registries. In this article we discuss the differential diagnosis and prognosis of the idiopathic inflammatory myopathies.


Assuntos
Miosite , Autoanticorpos , Diagnóstico Diferencial , Humanos , Músculo Esquelético , Miosite/diagnóstico , Prognóstico
8.
Brain Nerve ; 73(2): 117-125, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561826

RESUMO

The classification criteria of idiopathic inflammatory myopathy in the past include the criteria defined phenotypically by Peter and Bohan in 1975 and the criteria defined based on pathological findings in affected muscles. New criteria were defined by international researchers, including the author of this article, from multiple fields to be used widely in different fields in 2017. The criteria were approved by rheumatological societies in Europe and the United States, and validated as excellent criteria in a Japanese cohort. It is known that the criteria used for etiological studies and those for diagnosis should be different in their stringency. In the new criteria, the stringency can be set freely by the users to be used for a wide range of purposes.


Assuntos
Miosite , Estudos de Coortes , Europa (Continente) , Humanos , Miosite/diagnóstico
9.
Brain Nerve ; 73(2): 127-136, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561827

RESUMO

Immune-mediated necrotizing myopathy (IMNM) is a heterogeneous disease that presents with subacute progressive proximal dominant muscle weakness clinically and prominent necrotic muscle fibers without invasion of inflammatory cells pathologically. IMNM is a disease that exclusively affects skeletal muscles, so the serum creatine kinase level usually increases prominently. It is thought to be an autoimmune disease because of the presence of two autoantibodies, anti-SRP and anti-HMGCR. Because muscle involvement is more severe than that in other forms of myositis, its functional prognosis is worse. Several immune-modifying therapies can improve the symptoms; therefore, intensive treatments should be started soon after diagnosis with IMNM.


Assuntos
Doenças Autoimunes , Doenças Musculares , Miosite , Autoanticorpos , Doenças Autoimunes/diagnóstico , Humanos , Músculo Esquelético , Doenças Musculares/terapia , Necrose
10.
Brain Nerve ; 73(2): 137-146, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561828

RESUMO

Dermatomyositis and antisynthetase syndrome are among idiopathic inflammatory myopathies (myositis) that have skin lesions. In these disorders, skin symptoms often appear as the initial manifestations, and thus, their evaluation is critical for diagnosis. Two components, "site" and "properties," are important for understanding the skin manifestations in myositis. The rash in these disorders is induced by Koebner's phenomenon, and is most frequently observed on the hands and face. Recent studies have shown that myositis-specific autoantibodies are useful tools for the diagnosis and classification of these diseases, as they are closely correlated with the clinical and histopathological phenotypes of myositis. It has recently become clear that each antibody is associated with characteristic skin manifestations, especially related to the properties of the skin lesions.


Assuntos
Dermatomiosite , Miosite , Autoanticorpos , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Humanos , Miosite/complicações , Miosite/diagnóstico , Fenótipo
11.
Brain Nerve ; 73(2): 147-159, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561829

RESUMO

In the field of neurology, idiopathic inflammatory myopathy has been classified into four sub-categories, namely, dermatomyositis, anti-synthetase syndrome, inclusion body myositis, and immune-mediated necrotizing myopathy (IMNM), based upon histological and serological findings. Among them, IMNM has features similar to muscular dystrophy, and it may thus be difficult to differentiate between these two conditions, not only clinically but also pathologically, especially in chronic cases and pediatric patients. This is partly because the main pathological feature of both IMNM and muscular dystrophy is myofiber necrosis and regeneration. Furthermore, IMNM patients with anti-SRP antibodies tend to have more prominent muscle atrophy, especially in the shoulder girdle, which mimics the muscle involvement pattern in facioscapulohumeral muscular dystrophy. Some IMNM patients with anti-HMGCR antibodies have onset in their childhood or even in infancy, and may be misdiagnosed with muscular dystrophy. On the other hand, some muscular dystrophies have been reported to show more prominent lymphocyte infiltration than others, which may also mislead muscle pathologists. Nevertheless, these conditions can be distinguished using the relevant histological evaluations, including muscle immunohistochemistry for the MHC and C5b-9 antigens, in addition to appropriate clinical and laboratory examinations including muscle MRI and autoantibody testing.


Assuntos
Doenças Musculares , Distrofias Musculares , Miosite de Corpos de Inclusão , Miosite , Autoanticorpos , Criança , Humanos , Músculo Esquelético , Distrofias Musculares/diagnóstico , Miosite/diagnóstico
12.
Brain Nerve ; 73(2): 161-169, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561830

RESUMO

Inflammatory myopathies are heterogeneous disorders characterized by muscle inflammation. They are frequently accompanied by extra-muscular manifestations that affect the skin, lungs, heart, and joints. Owing to its low prevalence, wide phenotypic heterogeneity, and variable disease course, it is difficult to make clear recommendations for the treatment of inflammatory myopathies. Corticosteroids are administered as first-line treatment based on clinical experience rather than controlled trial findings. Empirically, addition of an immunosuppressive drug might offer a steroid-sparing effect or an additional benefit. Administration of intravenous immunoglobulins has been shown to be effective as second-line treatment. Recently, there has been a growing interest in assessing the potential of several biological agents in the treatment of inflammatory myopathies. There are multiple ongoing clinical trials that will lead to more treatment options for inflammatory myopathies.


Assuntos
Miosite , Corticosteroides , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Imunoterapia , Miosite/tratamento farmacológico
13.
Brain Nerve ; 73(2): 183-187, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561833

RESUMO

A 66-year-old woman visited our hospital complaining of shortness of breath during exertion and progressive weakness in all her limb muscles. On admission, we noted muscle weakness in her trunk and in her proximal limb muscles, although, her muscle MRI showed no remarkable findings. However, her serum CK level (2,747U/L) was above the normal range. Histopathological examination of muscle biopsy, performed from the left biceps brachii muscle, revealed immune-mediated necrotizing myopathy (IMNM). Her serum samples were negative for myositis-associated autoantibodies (MAAs), anti-SRP, and HMGCR antibodies. However, as the anti-SS-A antibody level in her serum was high (53.2U/mL), we conducted the salivary gland biopsy and the Schirmer test on her eyes. We found lymphocytes infiltration in her salivary gland tissue, and thus, she was diagnosed with primary Sjögren syndrome (SjS). We also observed necrotizing myopathy associated with the SjS. Following her treatment with oral steroids, her symptoms and CK level improved. Although, inflammatory myositis frequently occurs in association with general collagen diseases, necrotizing myopathy has rarely been observed secondary to SjS. We report here this rare case study along with the review of the relevant literature. (Received June 24, 2020; Accepted October 12, 2020; Published February 1, 2021).


Assuntos
Doenças Autoimunes , Doenças Musculares , Miosite , Síndrome de Sjogren , Idoso , Autoanticorpos , Feminino , Humanos , Doenças Musculares/etiologia , Miosite/complicações , Síndrome de Sjogren/complicações
14.
Neuroepidemiology ; 55(2): 109-118, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33631765

RESUMO

BACKGROUND: COVID-19 can be accompanied by acute neurological complications of both central and peripheral nervous systems (CNS and PNS). In this study, we estimate the frequency of such complications among hospital inpatients with COVID-19 in Assiut and Aswan university hospitals. MATERIALS AND METHODS: We screened all patients with suspected COVID-19 admitted from 1 June to 10 August 2020 to the university hospitals of Assiut and Aswan in Upper Egypt. Clinical and laboratory tests, CT/MRI of the chest and brain, and neurophysiology study were performed for each patient if indicated. RESULTS: 439 patients had confirmed/probable COVID-19; neurological manifestations occurred in 222. Of these, 117 had acute neurological disease and the remainder had nonspecific neuropsychiatric symptoms such as headache, vertigo, and depression. The CNS was affected in 75 patients: 55 had stroke and the others had convulsions (5), encephalitis (6), hypoxic encephalopathy (4), cord myelopathy (2), relapse of multiple sclerosis (2), and meningoencephalitis (1). The PNS was affected in 42 patients: the majority had anosmia and ageusia (31) and the others had Guillain-Barré syndrome (4), peripheral neuropathy (3), myasthenia gravis (MG, 2), or myositis (2). Fever, respiratory symptoms, and headache were the most common general symptoms. Hypertension, diabetes mellitus, and ischemic heart disease were the most common comorbidities in patients with CNS affection. CONCLUSION: In COVID-19, both the CNS and PNS are affected. Stroke was the most common complication for CNS, and anosmia and/or ageusia were common for PNS diseases. However, there were 6 cases of encephalitis, 2 cases of spinal cord myelopathy, 2 cases of MG, and 2 cases of myositis.


Assuntos
/fisiopatologia , Doenças do Sistema Nervoso Central/fisiopatologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Acidente Vascular Cerebral/fisiopatologia , Adulto , Idoso , Encéfalo/diagnóstico por imagem , /epidemiologia , Doenças do Sistema Nervoso Central/diagnóstico , Doenças do Sistema Nervoso Central/epidemiologia , Progressão da Doença , Egito/epidemiologia , Encefalite/epidemiologia , Encefalite/fisiopatologia , Feminino , Síndrome de Guillain-Barré/epidemiologia , Síndrome de Guillain-Barré/fisiopatologia , Hospitais Universitários , Humanos , Hipóxia Encefálica/epidemiologia , Hipóxia Encefálica/fisiopatologia , Pulmão/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Esclerose Múltipla Recidivante-Remitente/fisiopatologia , Miastenia Gravis/epidemiologia , Miastenia Gravis/fisiopatologia , Miosite/epidemiologia , Miosite/fisiopatologia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/epidemiologia , Convulsões/epidemiologia , Convulsões/fisiopatologia , Medula Espinal/diagnóstico por imagem , Doenças da Medula Espinal/epidemiologia , Doenças da Medula Espinal/fisiopatologia , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/epidemiologia , Tomografia Computadorizada por Raios X
15.
Autoimmun Rev ; 20(4): 102782, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33609795

RESUMO

OBJECTIVE: To review the extant literature relating to bone health in the idiopathic inflammatory myopathies (IIM) including both adult and juvenile patients. METHODS: A PubMed search® identified relevant studies from 1966 to 2020 in accordance with PRISMA guidelines. Two independent reviewers screened and extracted the abstracts/full manuscripts, and a third author was consulted in the case of disagreement. RESULTS: We identified 37 articles (3 review articles, 2 RCTs, 9 cross-sectional, 16 cohort and 7 case-control studies). The prevalence of osteopenia (n = 7) ranges from 7 to 75% and osteoporosis (n = 7) between 13% to 27%. The prevalence of vertebral fractures ranged from 11 to 75%. Systemic inflammation likely contributes to reduced bone mineral density (BMD) in children with IIM but data is currently lacking in adult patients. Association between with impaired BMD and Vitamin D or calcium intake and physical activity has not been demonstrated in IIM. There is no clear consensus regarding the impact of age, menopause or BMI on bone health. Gender, smoking status, disease activity and inflammatory markers are not obvious independent predictors of low BMD. Several studies have demonstrated that glucocorticoids are associated with an increased risk of low BMD. There are no specific guidelines relating to the management of bone health in adult and juvenile patients with IIM. CONCLUSION: Both adult and juvenile patients with IIM are at high risk of impaired bone health and fracture. The mechanisms behind this are likely multifactorial including systemic inflammation, glucocorticoid treatment, reduced mobility and impaired calcium/vitamin D homeostasis. There are a lack of guidelines and studies relating to the screening, prevention and treatment of impaired bone health in adult and juvenile patients with IIM. Future research is required to understand the complexity of bone health in IIM including to develop much needed disease-specific management recommendations.


Assuntos
Miosite , Osteoporose , Adulto , Densidade Óssea , Criança , Estudos Transversais , Feminino , Humanos , Miosite/complicações , Miosite/epidemiologia , Osteoporose/epidemiologia , Fatores de Risco , Vitamina D
16.
BMC Pulm Med ; 21(1): 57, 2021 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-33579248

RESUMO

BACKGROUND: Anti-synthetase syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, and interstitial lung disease (ILD). ASSD is highly heterogenous due to different organ involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behaviour of ASSD associated ILD. METHODS: Data of 108 cases of ASSD associated ILD were retrospectively collected in Beijing Chaoyang Hospital from December 2017 to March 2019. Data were obtained from the Electronic Medical Record system. Patients were divided into 5 groups according to distinct aminoacyl tRNA synthetase (ARS) antibodies. RESULTS: Overall, 108 consecutive patients were recruited. 33 were JO-1 positive, 30 were PL-7 positive, 23 were EJ positive, 13 were PL-12 positive and 9 were OJ positive. The JO-1 (+) group had a significant higher rate of mechanic's hand (57.6%) than other 4 groups. Polymyositis/dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The PL-7 (+) group had a higher frequency of UIP pattern (13.3%) than the other 4 groups but the difference was not significant, and the EJ (+) group had the most frequent OP pattern (78.2%), which was significantly higher than the PL-7 (+) (P < 0.001) and PL-12 (+) groups (P = 0.025). The median follow-up time was 10.7 months, during which no patients died. All received prednisone treatment, with or without immunosuppressants. At the 6-month follow-up, 96.3% of all patients (104/108) had a positive response to therapy, the JO-1 (+) and EJ (+) groups had a significantly higher improvement of forced vital capacity than the other 3 groups (P < 0.05), and the PL-7 group had the lowest FVC improvement (P < 0.05). The JO-1 (+) group and EJ (+) group had significantly higher anti-Ro-52 positive occurrence than the other 3 groups (P < 0.05). CONCLUSION: Anti PL-7 antibody had the same frequency as anti-JO-1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. Most ASSD-ILD had a positive response to steroid therapies, with or without immunosuppressants. The PL-7 (+) group had the highest occurrence of UIP pattern, and a significantly lower response to therapy.


Assuntos
Autoanticorpos/imunologia , Dermatomiosite/fisiopatologia , Doenças Pulmonares Intersticiais/fisiopatologia , Miosite/fisiopatologia , Adulto , Idoso , Alanina-tRNA Ligase/imunologia , Anticorpos Antinucleares/imunologia , China , Estudos de Coortes , Dermatomiosite/tratamento farmacológico , Dermatomiosite/imunologia , Feminino , Glucocorticoides/uso terapêutico , Glicina-tRNA Ligase/imunologia , Humanos , Fibrose Pulmonar Idiopática/tratamento farmacológico , Fibrose Pulmonar Idiopática/imunologia , Fibrose Pulmonar Idiopática/fisiopatologia , Imunossupressores/uso terapêutico , Isoleucina-tRNA Ligase/imunologia , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/imunologia , Masculino , Pessoa de Meia-Idade , Miosite/tratamento farmacológico , Miosite/imunologia , Prednisona/uso terapêutico , Prognóstico , Estudos Retrospectivos , Treonina-tRNA Ligase/imunologia , Resultado do Tratamento , Capacidade Vital
18.
Autoimmun Rev ; 20(3): 102757, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33476815

RESUMO

BACKGROUND: despite the absence of specific guidelines, the treatment with intravenous immunoglobulins (IvIg) is considered effective in patients with refractory idiopathic inflammatory myopathies (IIM). The aim of our study is to evaluate the effectiveness and the safety of IvIg and define the possible profile of IIM patients candidate to IvIg treatment. METHODS: we performed a retrospective study of IIM pts. treated with IvIg (2 g/kg/month). We collected demographic, epidemiological, laboratory and clinical data. Additionally, to evaluate the toxicity, the adverse events occurred during the treatment were collected. RESULTS: 123 patients with IIM were included in the study. The main indications for the prescription of IvIg were muscle (83.7% of patients) and esophageal involvement (45.5% of patients). IvIg were started mainly for refractory disease. At the end of treatment (mean duration 14 months), muscular necrosis enzymes decreased significantly and dysphagia VAS decreased significantly (p < 0.001), while MMT value increased (104.6 ± 24.2 vs. 127.0 ± 22.2 p < 0.001). Ninety-six pts. (78%) responded to IvIg. They had a shorter disease duration (p < 0.001), higher creatine kinase levels (p < 0.001), and higher prevalence of myalgias at the baseline (p = 0.023) compared to non-responders. The presence of Raynaud's phenomenon (p = 0.023-odds ratio 0.28 [0.11-0.72]) and skin involvement (p = 0.004, odds ratio 0.18 [0.06-0.55]), were associated to a worse response. Adverse events were mostly mild and transitory. CONCLUSIONS: Despite their high cost, IvIg confirmed their effectiveness in refractory IIM pts., particularly in muscular and esophageal manifestations. Specific clinical characteristics at the baseline may identify the patients with higher probability of response to the treatment.


Assuntos
Imunoglobulinas Intravenosas , Miosite , Humanos , Imunoglobulinas Intravenosas/efeitos adversos , Imunoglobulinas Intravenosas/uso terapêutico , Estudos Multicêntricos como Assunto , Miosite/tratamento farmacológico , Estudos Retrospectivos
19.
BMJ Case Rep ; 14(1)2021 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-33414112

RESUMO

Influenza A and B commonly cause benign respiratory disease in humans, but can cause more severe illness in high-risk populations. We report an unusual case of a previously healthy adult patient who presented with myositis and severe rhabdomyolysis secondary to influenza A infection that resulted in atraumatic compartment syndrome of all four extremities, each requiring emergent fasciotomy. The patient was subsequently managed with delayed primary closure and skin grafting in the operating room. Prompt recognition of this rare complication by the team resulted in no limb amputations. On his first follow-up appointment, 1 month after discharge, he had regained full functionality in both his hands and his feet were both close to 50% of baseline and improving with physical therapy.


Assuntos
Extremidades/patologia , Influenza Humana/complicações , Miosite/complicações , Rabdomiólise/complicações , Síndromes Compartimentais/etiologia , Síndromes Compartimentais/cirurgia , Extremidades/cirurgia , Humanos , Vírus da Influenza A , Masculino , Pessoa de Meia-Idade , Miosite/virologia , Rabdomiólise/virologia , Transplante de Pele
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