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1.
Medicine (Baltimore) ; 100(9): e24372, 2021 Mar 05.
Artigo em Inglês | MEDLINE | ID: mdl-33655912

RESUMO

RATIONALE: Severe refractory idiopathic inflammatory myopathy (IIM) represents a challenge for the clinician. The lack of efficacy of available tools reflects our incomplete insight into the molecular events sustaining the inflammatory tissue damage in these patients. We present the first case of refractory IIM treated with anti-dipeptidyl peptidase-4 (DPP-4)/cluster of differentiation 26 (CD26) monoclonal antibody. PATIENT CONCERNS: A 55-year old man presented with proximal muscle weakness, diffuse erythematous skin lesions which rapidly evolved into ulcerations, dysphagia and dysphonia. DIAGNOSIS: Increased serum creatine kinase levels and histological findings at muscle and skin biopsies were compatible with the diagnosis of dermatomyositis (DM). Several lines of treatment failed to control the disease including steroids, mycophenolate mofetil, tacrolimus, intravenous immunoglobulins and rituximab. Despite therapy, the patient also had recurrent intestinal vasculitis causing bowel perforation. Concurrently, DPP-4/CD26 expression in the patient's skin and skeletal muscle was observed. INTERVENTIONS: The patient was treated with begelomab, a murine immunoglobulin G2b monoclonal antibody against DPP-4/CD26. OUTCOMES: Dysphagia, skin lesions and intestinal vasculitis resolved and the patient experienced a significant improvement of his quality of life. CONCLUSION: Blockade of DPP-4/CD26, which is expressed on T cells and mediates T cell activation and function, is safe and might be effective in patients with refractory DM.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Dermatomiosite/tratamento farmacológico , Dipeptidil Peptidase 4/imunologia , Inibidores da Dipeptidil Peptidase IV/imunologia , Imunoglobulina G/imunologia , Dermatomiosite/imunologia , Humanos , Masculino , Pessoa de Meia-Idade
4.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 46(1): 104-107, 2021 Jan 28.
Artigo em Inglês, Chinês | MEDLINE | ID: mdl-33678644

RESUMO

Dermatomyositis (DM) is a kind of idiopathic inflammatory myopathy characterized by chronic proximal skeletal muscle weakness and unique skin lesions. However, DM with exfoliation of esophageal mucosa is rare. A 36-year-old male patient complained of muscular soreness of extremities, dysphagia, and pharyngalgia was diagnosed with DM with exfoliation of esophageal mucosa. After treatment with glucocorticoid, immunosuppressant, acupuncture, and endoscopic submucosal dissection (ESD), the above symptoms were disappeared. During the 3-year follow-up period, the results of routine physical examination, laboratory examination, gastroscopy, and imaging examination were normal. High-dose of corticosteroid is needed in the initial treatment, but it must be reduced regularly to avoid adverse reactions. Acupuncture and ESD are also effective as adjuvant therapy.


Assuntos
Dermatomiosite , Ressecção Endoscópica de Mucosa , Neoplasias Esofágicas , Adulto , Dermatomiosite/complicações , Mucosa Esofágica , Gastroscopia , Humanos , Masculino , Resultado do Tratamento
5.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 29(1): 253-258, 2021 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-33554830

RESUMO

OBJECTIVE: To present one patient initially diagnosed with dermatomyositis(DM) who was eventually revealed to be diffuse large B-cell lymphoma(DLBCL) complicated with hemophagocytic syndrome(HPS), and to improve the understanding of the disease. METHODS: The clinical characteristics, diagnostic approach, treatment of the patient were retrospectively analyzed, and some related literatures were reviewed. RESULTS: A 52-year-old female patient suffered from muscle weakness, elevated serum creatine kinase activity, electromyography changes and characteristic skin rashes and diagnosed as DM. The patient was treated with glucocorticoid therapy and the muscle strength, skin rashes, and creatine kinas index turns into remission. Subsequently, subcutaneous nodules appeared during treatment, and the patient was confirmed as DLBCL based on pathological biopsy; And the patient was considered HPS because of presenting with repeated fever, splenomegaly, cytopenias, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, high levels of sCD25, low NK-cell activity and hemophagocytosis in bone marrow. But the patient refused chemotherapy, and only treated with "DXM+VP-16" to control hemophagocytic syndrome, and unfortunately died due to the disease progression. CONCLUSION: Cutaneous involvement in diffuse large B-cell lymphoma and hemophagocytic syndrome patients with first presentation of dermatomyositis is relatively rare. Malignacy screening should be performed as soon as possible after newly diagnosed DM, so that the patient can get early diagnosis and effective treatment to improve survival rate.


Assuntos
Dermatomiosite , Linfo-Histiocitose Hemofagocítica , Linfoma Difuso de Grandes Células B , Dermatomiosite/complicações , Etoposídeo , Feminino , Humanos , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfoma Difuso de Grandes Células B/complicações , Pessoa de Meia-Idade , Estudos Retrospectivos
6.
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
7.
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
8.
Medicine (Baltimore) ; 100(1): e23677, 2021 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-33429735

RESUMO

INTRODUCTION: Dermatomyositis (DM) is an inflammatory myopathy characterized by distinct skin manifestations and muscle weakness. Intravenous immunoglobulin (IVIg) has been used off-label as adjuvant therapy in DM, but is not indicated for DM, due to lack of proven efficacy in a large randomized controlled trial. The objective of the ProDERM (Progress in DERMatomyositis) study was to evaluate the efficacy, safety and long-term tolerability of IVIg (Octagam 10%) in patients with DM in a randomized, placebo-controlled, double-blind, Phase III study. METHODS: Adult patients with active DM who were continuing standard therapy at a stable dose were eligible for this study. Patients were randomized 1:1 to receive either 2 g/kg of IVIg or placebo, administered every 4 weeks until week 16 (First Period). Patients were switched to the alternate treatment if they showed clinical deterioration in the First Period. After response assessment at week 16, all patients on placebo and those without deterioration on IVIg entered the open-label Extension Period, receiving 2 g/kg IVIg every 4 weeks for 24 weeks. RESULTS: The primary efficacy endpoint was the proportion of responders in the IVIg vs placebo arm at week 16, where response was defined per 2016 ACR/EULAR Myositis Response Criteria of at least minimal improvement [Total Improvement Score (TIS) ≥20] and without deterioration at 2 consecutive visits up to week 16. TIS consists of composite response criteria, combining weighted improvement in 6 core set measures (CSMs), Global Disease Activity (Physician and Patient), manual muscle testing-8 (MMT-8), Health Assessment Questionnaire, extra-muscular disease activity, and muscle enzymes. Secondary endpoints included the mean change in individual CSMs, time to improvement in TIS, time to confirmed deterioration in the First Period, and the overall proportion of patients with deteriorations. Adverse events, including infusion reactions and thromboembolic events, were recorded. CONCLUSIONS: The ProDERM study was the first to assess the long-term efficacy and safety of IVIg (Octagam 10%) in a placebo-controlled, blinded, randomized trial in DM. The study aimed to inform on the use of IVIg in the treatment of DM, and results are expected in Q3 2020. CLINICALTRIALSGOV IDENTIFIER: NCT02728752.


Assuntos
Dermatomiosite/tratamento farmacológico , Imunoglobulinas Intravenosas/normas , Adulto , Método Duplo-Cego , Feminino , Humanos , Imunoglobulinas Intravenosas/farmacologia , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Pessoa de Meia-Idade , Placebos , Estudos Prospectivos , Resultado do Tratamento
10.
BMJ Case Rep ; 14(1)2021 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-33472800

RESUMO

Staphylococcus-associated glomerulonephritis (SAGN) occurs as a complication of staphylococcal infection elsewhere in the body. Dermatomyositis (DM) can be associated with glomerulonephritis due to the disease per se. We report a case of a 40-year-old male patient with DM who presented with acute kidney injury, and was initially pulsed with methylprednisolone for 3 days, followed by dexamethasone equivalent to 1 mg/kg/day prednisolone. He was subsequently found to have SAGN on kidney biopsy along with staphylococcus bacteraemia and left knee septic arthritis. With proof of definitive infection, intravenous immunoglobulin 2 g/kg over 2 days was given and steroids were reduced. He was treated with intravenous vancomycin. With treatment, the general condition of the patient improved. On day 38, he developed infective endocarditis and died of congestive heart failure subsequently. Undiagnosed staphylococcal sepsis complicating a rheumatological disease course can lead to complications like SAGN, infective endocarditis and contribute to increased morbidity and mortality, as is exemplified by our case.


Assuntos
Lesão Renal Aguda/diagnóstico , Artrite Infecciosa/diagnóstico , Bacteriemia/diagnóstico , Dermatomiosite/tratamento farmacológico , Glomerulonefrite/patologia , Glucocorticoides/uso terapêutico , Infecções Estafilocócicas/diagnóstico , Lesão Renal Aguda/etiologia , Lesão Renal Aguda/terapia , Adulto , Antibacterianos/uso terapêutico , Artrite Infecciosa/tratamento farmacológico , Bacteriemia/tratamento farmacológico , Dermatomiosite/fisiopatologia , Endocardite Bacteriana , Evolução Fatal , Glomerulonefrite/etiologia , Glomerulonefrite/terapia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Infecções Estafilocócicas/complicações , Infecções Estafilocócicas/tratamento farmacológico , Vancomicina/uso terapêutico
11.
Mod Rheumatol Case Rep ; 5(1): 101-107, 2021 01.
Artigo em Inglês | MEDLINE | ID: mdl-33019894

RESUMO

Anti-melanoma differentiation-associated gene 5 juvenile dermatomyositis (anti-MDA5 JDM) is associated with high risk of developing rapidly progressive interstitial lung disease (RP-ILD). Here we report an 11-year-old girl with anti-MDA5 JDM and RP-ILD which led to a fatal outcome, further aggravated by SARS-CoV-2 infection. She was referred to our hospital after being diagnosed with anti-MDA5 JDM and respiratory failure due to RP-ILD. On admission, fibrobronchoscopy with bronchoalveolar lavage (BAL) revealed Pneumocystis jirovecii infection so treatment with intravenous trimethoprim-sulfamethoxazole was initiated. Due to RP-ILD worsening, immunosuppressive therapy was intensified using methylprednisolone pulses, cyclophosphamide, tofacitinib and intravenous immunoglobulin without response. She developed severe hypoxemic respiratory failure, pneumomediastinum and pneumothorax, further complicated with severe RP-ILD and cervical subcutaneous emphysema. Three real-time RT-PCR for SARS-CoV-2 were made with a negative result. In addition, she was complicated with a secondary hemophagocytic lymphohistiocytosis and a fourth real-time PCR for SARS-CoV-2 performed in BAS sample was positive. Despite aggressive treatment of RP-ILD due to anti-MDA5 JDM, there was no improvement of respiratory failure in the following days and patient developed refractory septic shock and died. Anti-MDA5 JDM patients with RP-ILD have a poor prognosis with a high mortality rate. For this reason, intensive immunosuppressive therapy is essential including the use of promising drugs such as tofacitinib. COVID-19 in children with underlying health conditions like anti-MDA5 JDM may still be at risk for disease and severe complications.


Assuntos
/complicações , Dermatomiosite/complicações , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais/complicações , Linfo-Histiocitose Hemofagocítica/etiologia , Pneumonia por Pneumocystis/complicações , Monofosfato de Adenosina/análogos & derivados , Monofosfato de Adenosina/uso terapêutico , Alanina/análogos & derivados , Alanina/uso terapêutico , Antibacterianos/uso terapêutico , Anticorpos Monoclonais Humanizados/uso terapêutico , Antivirais/uso terapêutico , Autoanticorpos/imunologia , Broncoscopia , Criança , Ciclofosfamida/uso terapêutico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/imunologia , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Hospedeiro Imunocomprometido , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Helicase IFIH1 Induzida por Interferon/imunologia , Pulmão/diagnóstico por imagem , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/terapia , Linfo-Histiocitose Hemofagocítica/imunologia , Enfisema Mediastínico/etiologia , Metilprednisolona/uso terapêutico , Piperidinas/uso terapêutico , Pneumonia por Pneumocystis/imunologia , Pneumotórax/etiologia , Pirimidinas/uso terapêutico , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Choque Séptico/etiologia , Enfisema Subcutâneo/etiologia , Tomografia Computadorizada por Raios X , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
12.
Clin Dermatol ; 38(6): 731-733, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33341205

RESUMO

In late 2019, the coronavirus disease 2019 (COVID-19) broke out in Wuhan and then spread over China, which greatly affected the medical practices and health care systems. With most of the hospital's outpatient services closed, the routine clinical diagnosis and treatment for patients with dermatomyositis has been disturbed. We conducted telephone follow-up for 52 patients to know the changes in the condition and the continuation of drug therapy and to ensure the continuity, safety, and effectiveness of the treatment of patients with dermatomyositis during COVID-19.


Assuntos
/epidemiologia , Fármacos Dermatológicos/uso terapêutico , Dermatomiosite/tratamento farmacológico , Adulto , Idoso , China/epidemiologia , Fármacos Dermatológicos/efeitos adversos , Surtos de Doenças , Feminino , Humanos , Masculino , Adesão à Medicação , Pessoa de Meia-Idade , Telemedicina
13.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(6): 995-1000, 2020 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-33331304

RESUMO

OBJECTIVE: To investigate and analyse the clinical and immunological features of patients with myositis complicated with thromboembolism. METHODS: We identified a cohort of 390 myositis patients diagnosed with myositis admitted to People's Hospital of Peking University from 2003 to 2019. The patients were retrospectively enrolled in this investigation. According to the outcome of the color Doppler ultrasound, CT pulmonary angiography, pulmonary ventilation and perfusion scan patients were divided into myositis with and without thromboembolism group. Demographic, clinical (heliotrope rash, Gottron's sign/papules, periungual erythema, skin ulceration, subcutaneous calcinosis, Mechanic's hands, myalgia, interstitial lung disease, pulmonary arterial hypertension), laboratory, immunological [anti-autoantibodies including melanoma differentiation associated gene 5 (anti-MDA5), anti-Mi-2, anti-transcription intermediary factor-1γ (anti-TIF-1γ, anti-nuclear matrix protein 2 (anti-NXP2), anti-small ubiquitin-like modifier activating enzyme (anti-SAE), anti-synthetase], imaging and therapeutic status data of the patients at the diagnosis of myositis with and without thromboembolism were collected and the differences in these data were analyzed. Logistic regressive analysis was used to identify the risk factors of thromboembolism. RESULTS: In the retrospective study, 390 myositis patients were investigated. The mean age of onset was (49.6±13.4) years, male to female ratio was 0.31 :1. Thromboembolism was identified in 4.62% (18/390) of the myositis patients, which was lower than the published reports. Out of 18 patients with thromboembolism, 55.6% (10/18) of them were deep venous thrombosis, followed by cerebral infarction (22.2%, 4/18), pulmonary embolism (11.1%, 2/18), renal artery embolism (5.6%, 1/18) and embolism of upper extremity (5.6%, 1/18). Fifty percent of thromboembolism events occurred 6 months after the diagnosis of myositis, 38.9% of thromboembolism events occurred 6 months within the diagnosis of myositis, 11.1% of thromboembolism events occurred 6 months before the diagnosis of myositis. As compared with the myositis patients without thromboembolism, the myositis patients complicated with thromboembolism were older [(58.3±11.7) years vs. (49.3±13.4) years, P=0.006]. C-reaction protein (CRP) (12.2 mg/L vs. 4.1 mg/L, P < 0.001), ferritin (20 085.5 µg/L vs. 216.6 µg/L, P < 0.001) and D-dimer (529.0 µg/L vs. 268.0 µg/L, P=0.002) were significantly higher in thromboembolism group. Diabetes (44.4% vs. 16.4%, P=0.006), coronary heart disease (22.2% vs. 3.0%, P=0.003) and surgery (16.7% vs. 3.5%, P=0.032) were observed more common in thromboembolism group than those without thromboembolism. Activated partial thromboplastin time (APTT) (26.9 s vs. 28.7 s, P=0.049) and albumin (32.4 g/L vs. 36.5 g/L, P=0.002) was lower in thromboembolism group. The risk factors of thromboembolism in the myositis patients were low level of albumin (OR=0.831, 95%CI: 0.736-0.939, P=0.003), diabetes (OR=4.468, 95%CI: 1.382-14.448, P=0.012), and coronary heart disease (OR=22.079, 95%CI: 3.589-135.837, P=0.001) were independent significant risk factors for thromboembolism in the patients with myositis. There was no significant difference in clinical manifestations, myositis-specific antibodies or myositis-associated antibodies between the two groups. CONCLUSION: Thromboembolism is a complication of myositis. Lower levels of albumin, diabetes, and coronary heart disease might be risk factors of thromboembolism in myositis patients.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Miosite , Tromboembolia , Adulto , Autoanticorpos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/complicações , Estudos Retrospectivos
14.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(6): 1001-1008, 2020 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-33331305

RESUMO

OBJECTIVE: To study the differences between clinically amyopathic dermatomyositis (CADM) and typical dermatomyositis (DM) on clinical and immunological features. METHODS: By collecting clinical data of 106 CADM patients and 158 DM patients from January 2010 to June 2019 in the department of Rheumatology and Immunology, Peking University People's Hospital, the clinical characteristics and immunological features in the two groups were compared, and the distribution characters and the clinical meanings of myositis autoantibodies were discussed in the two groups respectively. Myositis autoantibodies were measured by immunoblotting according to the manufacturers' instructions. RESULTS: In the aspects of clinical manifestations, CADM presented more with onset of interstial lung diseases (ILD) compared with DM (20.7% vs. 7.6%, P=0.002), and CADM-ILD was more likely to be acute ILD (58.3% vs. 26%, P < 0.001), and there were no differences between CADM and DM in cutaneous manifestations, accompanied with connective tissue disease (CTD) and malignancy. In CADM, the positive rate of rheumatoid factors and antinuclear antibodies was lower in DM. The most common myositis specific autoantibodies (MSAs) in CADM were anti-MDA5 (36%), anti-PL-7 (11.2%) and anti-TIF-1γ (10.1%). The most common MSAs in DM were anti-Jo-1 (19.2%), anti-TIF-1γ (11.5%) and anti-MDA5 (11.5%). Anti-MDA5 was correlated with acute ILD and skin ulceration both in CADM and DM; in CADM, skin ulceration was not associated with the titer of anti-MDA5; while in DM, skin ulceration was associated with high titer of anti-MDA5. In DM, anti-TIF-1γ was correlated with heliotrope eruption, V/shawl neck sign, perionychia erythma and malignancy, and higher rate of malignancy was seen in all titers of the anti-TIF-1γ positive patients. In CADM, anti-TIF1-γ showed no correlation with clinical manifestations. The most common myositis associated autoantibody was anti-Ro-52 both in CADM and DM. In CADM, anti-Ro-52 was associated with Raynaud's phenomenon and chronic ILD, while in DM, anti-Ro-52 was associated with mechanic's hands, noninfectious fever and accompanied CTD. CONCLUSION: Compared with DM, ILD is more likely to be acute in CADM. It is different between CADM and DM about the distribution of myositis autoantibodies and the clinical significance of the same myositis antibody, and the clinical significance of some myositis antibodies is related to titers.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Neoplasias , Autoanticorpos , Dermatomiosite/complicações , Humanos
17.
J Assoc Physicians India ; 68(11): 20-24, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33187031

RESUMO

Viruses have been shown to modify the clinical picture of several autoimmune diseases, including type 1 diabetes, systemic lupus erythematosus (SLE), rheumatoid arthritis and multiple sclerosis. Viral infections have also been considered as a possible trigger for autoimmune disorders like myositis through myositis specific antibodies. Dermatomyositis is an acquired inflammatory myopathy which is relatively rare with incidence of 9.3 per 1 million persons. Usually we come across 1-2 patients of dermatomyositis per year, amongst 800-1000 new patients in our tertiary care rheumatology services. A surge in the incidence was noted this year during the months of April-August of 2020, the period coinciding with the occurrence of corona virus (COVID-19) pandemic in the city of Mumbai, the total number of cases encountered being five in a span of six months. The following case series includes five such cases with review of available literature on virus-triggered autoimmunity with special reference to SARS-CoV-2 and the challenges of immunosuppression during this pandemic.


Assuntos
Betacoronavirus , Infecções por Coronavirus , Dermatomiosite , Lúpus Eritematoso Sistêmico , Pandemias , Pneumonia Viral , Dermatomiosite/epidemiologia , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia
18.
BMC Pulm Med ; 20(1): 304, 2020 Nov 19.
Artigo em Inglês | MEDLINE | ID: mdl-33213393

RESUMO

BACKGROUND: Coronavirus disease 2019 (COVID-19) has reach pandemic proportions globally. For patients with symptoms of fever and cough accompanied by rapid lung damage progression, COVID-19 needs to be distinguished from interstitial lung disease (ILD) attributed to connective tissue disease (CTD), especially dermatomyositis (DM)/clinical amyopathic dermatomyositis (CADM) associated rapidly progressive interstitial lung disease (RP-ILD). CASE PRESENTATION: We report a case of a woman observed with fever, cough, and rapid lung damage during the epidemic. The patient had a suspicious epidemiological history, and her chest CT scans showed lung damage similar to that caused by COVID-19, but anti-Ro52 antibody was strongly positive. She was diagnosed with CADM associated RP-ILD and died 1 month later. CONCLUSIONS: During the COVID-19 epidemic, it is critical to carefully assess patients with CTD related ILD, especially RP-ILD associated with CADM. Repeated nucleic acid tests for COVID-19 are necessary to achieve accurate case diagnosis. High-resolution CT (HRCT) of the chest is presently deemed an inefficient technique to distinguishing between COVID-19 and CADM associated RP-ILD. The characteristic rashes of dermatomyositis require careful observation and can often provide diagnostic clues. For patients with CADM, a high titers of anti-Ro52 antibody may be related to the pathogenesis of RP-ILD, suggesting a poor prognosis.


Assuntos
Betacoronavirus , Infecções por Coronavirus/complicações , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/etiologia , Pneumonia Viral/complicações , Infecções por Coronavirus/diagnóstico , Dermatomiosite/terapia , Diagnóstico Diferencial , Evolução Fatal , Feminino , Humanos , Doenças Pulmonares Intersticiais/terapia , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/diagnóstico
20.
Isr Med Assoc J ; 22(10): 623-627, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-33070486

RESUMO

BACKGROUND: The reported mortality rates of patients with polymyositis and dermatomyositis are highly variable worldwide. The excess mortality of patients with polymyositis/dermatomyositis has not been evaluated in an Israeli population. OBJECTIVES: To investigate the overall mortality in a large and well-established cohort of patients with polymyositis/dermatomyositis as compared to the mortality expected in the matched general population in a tertiary medical center. METHODS: In this retrospective cohort study, the mortality of 166 patients with polymyositis/dermatomyositis was compared to age- and sex-matched control subjects in the general population. All-cause standardized mortality ratios (SMRs) were estimated. RESULTS: Overall, 47 (28.3%) deaths were observed among patients with polymyositis/dermatomyositis during a mean follow-up period of 5.8 ± 4.8 years, which was 7 times higher than in the control group (SMR 7.4, 95% confidence interval [95%CI] 5.5-9.8). The SMRs were comparable in patents with polymyositis (7.7, 95%CI 4.8-12.3) and dermatomyositis (7.2, 95%CI 5.0-10.3). The 1-, 5-, 10-, and 15-year overall survival rates were 90.0%, 82.8%, 51.5%, and 26.1%, respectively, in patients with polymyositis, and 80.3%, 59.6%, 40.0%, and 17.1%, respectively, in patients with dermatomyositis. CONCLUSIONS: The overall mortality among Israeli patients with polymyositis/dermatomyositis is 7.4 times greater than for the general population. Although long-term mortality was comparable between patients with dermatomyositis and polymyositis, patients in the former group died at a notably earlier stage.


Assuntos
Causas de Morte , Dermatomiosite/diagnóstico , Dermatomiosite/mortalidade , Polimiosite/diagnóstico , Polimiosite/mortalidade , Adulto , Fatores Etários , Estudos de Casos e Controles , Dermatomiosite/terapia , Feminino , Humanos , Israel , Masculino , Pessoa de Meia-Idade , Polimiosite/terapia , Prognóstico , Valores de Referência , Estudos Retrospectivos , Índice de Gravidade de Doença , Fatores Sexuais , Análise de Sobrevida , Centros de Atenção Terciária , Reino Unido
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