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[PMID]:29367532
[Au] Autor:Michitsuji T; Horai Y; Sako A; Asano T; Iwanaga N; Izumi Y; Kawakami A
[Ad] Endereço:Department of General and Internal Medicine, National Hospital Organization Nagasaki Medical Center.
[Ti] Título:[A case of mixed connective tissue disease positive for proteinase 3 antineutrophil cytoplasmic antibody in a patient with slowly progressive type 1 diabetes mellitus and chronic thyroiditis].
[So] Source:Nihon Rinsho Meneki Gakkai Kaishi;40(6):467-470, 2017.
[Is] ISSN:1349-7413
[Cp] País de publicação:Japan
[La] Idioma:jpn
[Ab] Resumo:  A female in her sixties with slowly progressive type 1 diabetes mellitus (SPT1DM) and chronic thyroiditis was referred to our rheumatology department with swelling in her fingers. A prominent atherosclerotic lesion was revealed upon brain magnetic resonance imaging, and she was found to have mixed connective tissue disease (MCTD) positive for proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA). This rare case of MCTD accompanying SPT1DM and PR3-ANCA suggested that a synergy between MCTD and PR3-ANCA triggers atherosclerosis.
[Mh] Termos MeSH primário: Anticorpos Anticitoplasma de Neutrófilos
Aterosclerose/etiologia
Diabetes Mellitus Tipo 1/complicações
Doença de Hashimoto/complicações
Doença Mista do Tecido Conjuntivo/diagnóstico
Doença Mista do Tecido Conjuntivo/imunologia
Mieloblastina/imunologia
Tireoidite/complicações
[Mh] Termos MeSH secundário: Idoso
Aterosclerose/diagnóstico por imagem
Aterosclerose/imunologia
Encéfalo/diagnóstico por imagem
Progressão da Doença
Feminino
Seres Humanos
Imagem por Ressonância Magnética
Meia-Idade
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antibodies, Antineutrophil Cytoplasmic); EC 3.4.21.76 (Myeloblastin)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180227
[Lr] Data última revisão:
180227
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180126
[St] Status:MEDLINE
[do] DOI:10.2177/jsci.40.467


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[PMID]:29241348
[Au] Autor:Kim M; Sadoughi B
[Ad] Endereço:1 Sean Parker Institute for the Voice, Department of Otolaryngology-Head and Neck Surgery, Weill Cornell Medical College/NewYork-Presbyterian Hospital, New York, New York, USA.
[Ti] Título:The Voice of Autoimmunity: Antisynthetase Syndrome Manifesting as Vocal Fold Bamboo Nodes.
[So] Source:Ann Otol Rhinol Laryngol;127(2):128-130, 2018 Feb.
[Is] ISSN:1943-572X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: To describe a case of vocal fold bamboo nodes leading to the diagnosis of antisynthetase syndrome, a rare autoimmune disorder. To highlight the link between these laryngeal lesions and autoimmunity. METHODS: A case of vocal fold bamboo nodes in a patient with long-standing interstitial lung disease is presented. The presence of these characteristic lesions prompted a rheumatologic workup that led to the diagnosis of a rare autoimmune disorder. RESULTS: The patient was ultimately diagnosed with antisynthetase syndrome, a rare condition characterized by inflammatory myositis and interstitial lung disease. She was treated with steroids and immunosuppressive agents with improvement in her symptoms and clinical findings. CONCLUSIONS: Vocal fold bamboo nodes are pathognomonic signs of autoimmunity. Management consists primarily of medical treatment of the underlying systemic disorder. Intralesional steroid injection or phonomicrosurgical excision may be considered for refractory cases.
[Mh] Termos MeSH primário: Doenças Autoimunes/diagnóstico
Disfonia/diagnóstico
Doenças da Laringe/diagnóstico
Doenças Pulmonares Intersticiais/diagnóstico
Doença Mista do Tecido Conjuntivo/diagnóstico
Miosite/diagnóstico
Nódulo Reumatoide/diagnóstico
Prega Vocal/patologia
[Mh] Termos MeSH secundário: Doenças Autoimunes/tratamento farmacológico
Diagnóstico Diferencial
Disfonia/tratamento farmacológico
Disfonia/patologia
Feminino
Seres Humanos
Doenças da Laringe/tratamento farmacológico
Doenças da Laringe/patologia
Laringoscopia
Doenças Pulmonares Intersticiais/tratamento farmacológico
Doenças Pulmonares Intersticiais/patologia
Meia-Idade
Doença Mista do Tecido Conjuntivo/tratamento farmacológico
Miosite/tratamento farmacológico
Prednisona/uso terapêutico
Nódulo Reumatoide/tratamento farmacológico
Nódulo Reumatoide/patologia
Gravação em Vídeo
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
VB0R961HZT (Prednisone)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180123
[Lr] Data última revisão:
180123
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171216
[St] Status:MEDLINE
[do] DOI:10.1177/0003489417748331


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[PMID]:28904690
[Au] Autor:Missounga L; Ba JI; Nseng Nseng Ondo IR; Nziengui Madjinou MIC; Malekou D; Mouendou Mouloungui EG; Nzengue EE; Boguikouma JB; Kombila M
[Ad] Endereço:Département de Médecine Interne et Spécialités Médicales, Université des Sciences de la Santé, BP 4009 Libreville Gabon.
[Ti] Título:[Mixed connective tissue disease: prevalence and clinical characteristics in African black, study of 7 cases in Gabon and review of the literature].
[Ti] Título:La connectivite mixte: prévalence et caractéristiques cliniques chez le noir africain, étude de 7 cas au Gabon et revue de la littérature..
[So] Source:Pan Afr Med J;27:162, 2017.
[Is] ISSN:1937-8688
[Cp] País de publicação:Uganda
[La] Idioma:fre
[Ab] Resumo:The literature reports that mixed connective tissue disease seems more frequent in the black population and among Asians. This study aims to determine the prevalence of mixed connective tissue disease (MCTD) among connective tissue disorders and all rheumatologic pathologies in a hospital population in Gabon as well as to describe the clinical features of this disease. We conducted a retrospective study by reviewing the medical records of patients treated for mixed connective tissue disease (Kasukawa criteria) and other entities of connective tissue disorders (ACR criteria) in the Division of Rheumatology at the University Hospital in Libreville between January 2010 and December 2015. For each case of MCTD the parameters studied were articular and extra-articular manifestations, anti-U1RNP antibodies levels, patient's evolution. Over a period of 6 years, data were collected by medical records of 7 patients out of 6050 patients and 67 cases of connective tissue disorders, reflecting a prevalence of 0.11% and 10.44% respectively. the 7 patients were women (100%), with an average age of 39.5 years. Articular manifestations included: polyarthritis, myalgias, chubby fingers and Raynaud's phenomenon in 87.5%, 87.5%, 28.6% and 14% respectively. The 7 patients had high anti-U1RNP antibodies levels, ranging between 5 and 35N (N≤ 7 IU). A case of death due to pulmonary arterial hypertension (PAH) was certified. This is the largest case series of MCTD reported in Black Africa. The disease seems to be rare among the black Africans; the reason could be genetic. The demographic and clinical aspects appear similar to those in Caucasians, Asians and Blacks except for a low frequency of Raynaud?s phenomenon among Blacks.
[Mh] Termos MeSH primário: Autoanticorpos/imunologia
Doença Mista do Tecido Conjuntivo/fisiopatologia
Ribonucleoproteína Nuclear Pequena U1/imunologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Grupo com Ancestrais do Continente Africano
Feminino
Gabão/epidemiologia
Seres Humanos
Meia-Idade
Doença Mista do Tecido Conjuntivo/epidemiologia
Doença Mista do Tecido Conjuntivo/imunologia
Prevalência
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Autoantibodies); 0 (Ribonucleoprotein, U1 Small Nuclear)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170926
[Lr] Data última revisão:
170926
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170915
[St] Status:MEDLINE
[do] DOI:10.11604/pamj.2017.27.162.12572


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[PMID]:28700492
[Au] Autor:Kelkar AH; Shah AA; Yong SL; Ahmed Z
[Ad] Endereço:aDepartment of Internal Medicine bDepartment of Pathology, University of Illinois College of Medicine at Peoria, Peoria cDepartment of Gastroenterology, University of Illinois College of Medicine at Chicago, Chicago, IL.
[Ti] Título:An unusual association between hemophagocytic lymphohistiocytosis, mixed connective tissue disease, and autoimmune hemolytic anemia: A case report.
[So] Source:Medicine (Baltimore);96(28):e7488, 2017 Jul.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: In the adult patient, hemophagocytic lymphohistiocytosis (HLH) is uncommon and frequently difficult to diagnose due to its nonspecific presentation and numerous complications. PATIENT CONCERNS: Herein, we present the case of a 25-year-old female who initially presented for evaluation of persistent fevers and fatigue. She was found to have splenomegaly, generalized lymphadenopathy, pancytopenia, and acute hepatic failure. DIAGNOSES, INTERVENTIONS, AND OUTCOMES: Her course was further complicated by the development of nephrotic syndrome and autoimmune hemolytic anemia (AIHA). Antinuclear antibody and ribonucleoprotein were positive, with concurrent physical examination findings, indicating underlying mixed connective tissue disease (MCTD). Ferritin was greater than 40,000 ng/dL. Viral studies, including hepatitis A, B, and C, cytomegalovirus, and Epstein-Barr virus were negative. On the basis of her clinical presentation, a diagnosis of HLH secondary to MCTD was made. This was later confirmed on liver biopsy. She was started on high-dose prednisone and her symptoms completely resolved. She was then transitioned to azathioprine, hydroxychloroquine, prophylactic antibiotics, and a prednisone taper for long-term management. LESSONS: This case is notable for the association of both AIHA and MCTD with HLH, providing support for a possible relationship between these 3 conditions.
[Mh] Termos MeSH primário: Anemia Hemolítica Autoimune/complicações
Anemia Hemolítica Autoimune/diagnóstico
Linfo-Histiocitose Hemofagocítica/diagnóstico
Linfo-Histiocitose Hemofagocítica/etiologia
Doença Mista do Tecido Conjuntivo/complicações
Doença Mista do Tecido Conjuntivo/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Anemia Hemolítica Autoimune/tratamento farmacológico
Anemia Hemolítica Autoimune/patologia
Diagnóstico Diferencial
Feminino
Seres Humanos
Linfo-Histiocitose Hemofagocítica/tratamento farmacológico
Linfo-Histiocitose Hemofagocítica/patologia
Doença Mista do Tecido Conjuntivo/tratamento farmacológico
Doença Mista do Tecido Conjuntivo/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170730
[Lr] Data última revisão:
170730
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170713
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000007488


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[PMID]:28289001
[Au] Autor:Ashraf M; Gopikrishnan K; Umamahesvaran B; Sambandam SN
[Ad] Endereço:Department of Orthopedics, KG Hospital, Coimbatore, Tamil Nadu, India.
[Ti] Título:Presentation of in mixed connective tissue disorder: an encounter during hip arthroplasty.
[So] Source:BMJ Case Rep;2017, 2017 Mar 13.
[Is] ISSN:1757-790X
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:A woman aged 23 years with a diagnosis of mixed connective tissue disorder presented with left groin pain extending over 6 months. Workup revealed avascular necrosis of the femoral head (Grade 3) secondary to systemic lupus erythematosus and chronic steroid intake. An uncemented total hip arthroplasty was considered as the patient was only in the third decade of life. During the preop workup, careful clinical assessment had revealed multiple subcutaneous nodules affecting the extensor musculature limited to the gluteal region, anterior and posterior aspects of the thigh. The diagnosis of was made after a CT revealed calcified nodules in the subcutaneous, subfascial and muscular planes. A total hip arthroplasty using the posterior approach was performed with minimal trauma to the calcified nodules and thereby preventing a source of persistent drainage and reducing morbidity due to infection.
[Mh] Termos MeSH primário: Artroplastia de Quadril
Calcinose/diagnóstico
Necrose da Cabeça do Fêmur/diagnóstico
Necrose da Cabeça do Fêmur/cirurgia
Lúpus Eritematoso Sistêmico/complicações
Lúpus Eritematoso Sistêmico/diagnóstico
Doença Mista do Tecido Conjuntivo/complicações
Dermatopatias/diagnóstico
[Mh] Termos MeSH secundário: Calcinose/complicações
Feminino
Necrose da Cabeça do Fêmur/etiologia
Virilha/cirurgia
Seres Humanos
Doença Mista do Tecido Conjuntivo/diagnóstico
Dermatopatias/complicações
Tomografia Computadorizada por Raios X
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170322
[Lr] Data última revisão:
170322
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170315
[St] Status:MEDLINE


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[PMID]:28279287
[Au] Autor:Biswas A; Ataya A; Salgado JC; Chandrasekharan S; Machuca TN; Emtiazjoo AM
[Ad] Endereço:Division of Pulmonary Critical Care and Sleep Medicine, University of Florida, Gainesville, FL.
[Ti] Título:A 42-Year-Old Woman With Anemia, Shock, and Ischemic Stroke After Lung Transplantation.
[So] Source:Chest;151(3):e63-e68, 2017 Mar.
[Is] ISSN:1931-3543
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:CASE PRESENTAION: A 42-year-old woman with mixed connective tissue disease-associated interstitial lung disease underwent bilateral lung transplantation. She had an uneventful surgery and was extubated 3 h later. Induction immunosuppression therapy included methylprednisolone 500 mg intraoperatively, basiliximab (anti-IL-2 monoclonal antibody) on days 0 and 4 after transplantation, and methylprednisolone 125 mg intravenously bid for 2 days following surgery. Maintenance immunosuppression therapy consisted of prednisone 20 mg daily, mycophenolate mofetil 750 mg bid, and enteral tacrolimus 0.5 mg bid. Both the donor and the recipient were seropositive for cytomegalovirus. Infectious disease prophylaxis consisted of valganciclovir, trimethoprim-sulfamethoxazole, and voriconazole.
[Mh] Termos MeSH primário: Anemia/diagnóstico
Síndrome Hemolítico-Urêmica Atípica/diagnóstico
Infarto da Artéria Cerebral Média/diagnóstico por imagem
Doenças Pulmonares Intersticiais/cirurgia
Transplante de Pulmão
Complicações Pós-Operatórias/diagnóstico
Choque/diagnóstico
Acidente Vascular Cerebral/diagnóstico por imagem
[Mh] Termos MeSH secundário: Lesão Renal Aguda/diagnóstico
Lesão Renal Aguda/etiologia
Adulto
Anemia/etiologia
Anemia/terapia
Síndrome Hemolítico-Urêmica Atípica/complicações
Transfusão de Sangue
Ecocardiografia
Feminino
Rejeição de Enxerto/prevenção & controle
Seres Humanos
Imunossupressores/uso terapêutico
Doenças Pulmonares Intersticiais/etiologia
Imagem por Ressonância Magnética
Doença Mista do Tecido Conjuntivo/complicações
Choque/etiologia
Acidente Vascular Cerebral/etiologia
Trombocitopenia/diagnóstico
Trombocitopenia/etiologia
Trombocitopenia/terapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunosuppressive Agents)
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170529
[Lr] Data última revisão:
170529
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170311
[St] Status:MEDLINE


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[PMID]:28202869
[Au] Autor:Sugawara E; Kato M; Hisada R; Oku K; Bohgaki T; Horita T; Yasuda S; Atsumi T
[Ad] Endereço:Division of Rheumatology, Endocrinology and Nephrology, Hokkaido University Graduate School of Medicine, Japan.
[Ti] Título:Treatment of Vasodilator-resistant Mixed Connective Tissue Disease-associated Pulmonary Arterial Hypertension with Glucocorticoid and Cyclophosphamide.
[So] Source:Intern Med;56(4):445-448, 2017.
[Is] ISSN:1349-7235
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Pulmonary arterial hypertension (PAH) associated with systemic lupus erythematosus (SLE) or mixed connective tissue disease (MTCD), in contrast to other types of PAH, may respond to immunosuppressive therapy. Most PAH cases with an immunosuppressant response were in the early stages of the disease (WHO functional class III or less). The present case was a 34-year-old woman with MCTD-associated PAH (WHO functional class IV) who was resistant to a combination of three vasodilators. Afterwards, she was treated with glucocorticoid and cyclophosphamide. This case suggested the potential benefit of immunosuppressants in patients with severe MCTD-associated PAH.
[Mh] Termos MeSH primário: Ciclofosfamida/uso terapêutico
Glucocorticoides/uso terapêutico
Hipertensão Pulmonar/tratamento farmacológico
Doença Mista do Tecido Conjuntivo/complicações
[Mh] Termos MeSH secundário: Adulto
Resistência a Medicamentos
Quimioterapia Combinada
Feminino
Seres Humanos
Hipertensão Pulmonar/diagnóstico por imagem
Hipertensão Pulmonar/etiologia
Hipertensão Pulmonar/fisiopatologia
Imunossupressores/uso terapêutico
Radiografia Torácica
Vasodilatadores/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Glucocorticoids); 0 (Immunosuppressive Agents); 0 (Vasodilator Agents); 8N3DW7272P (Cyclophosphamide)
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170410
[Lr] Data última revisão:
170410
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170217
[St] Status:MEDLINE
[do] DOI:10.2169/internalmedicine.56.7668


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[PMID]:28039187
[Au] Autor:Coghlan JG; Galiè N; Barberà JA; Frost AE; Ghofrani HA; Hoeper MM; Kuwana M; McLaughlin VV; Peacock AJ; Simonneau G; Vachiéry JL; Blair C; Gillies H; Miller KL; Harris JHN; Langley J; Rubin LJ; AMBITION investigators
[Ad] Endereço:Cardiology Department, Royal Free Hospital, London, UK.
[Ti] Título:Initial combination therapy with ambrisentan and tadalafil in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial.
[So] Source:Ann Rheum Dis;76(7):1219-1227, 2017 Jul.
[Is] ISSN:1468-2060
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH), in particular systemic sclerosis (SSc), had an attenuated response compared with idiopathic PAH in most trials. Thus, there is uncertainty regarding the benefit of PAH-targeted therapy in some forms of CTD-PAH. OBJECTIVE: To explore the safety and efficacy of initial combination therapy with ambrisentan and tadalafil versus ambrisentan or tadalafil monotherapy in patients with CTD-PAH and SSc-PAH enrolled in the AMBITION trial. METHODS: This was a post hoc analysis of patients with CTD-PAH and SSc-PAH from AMBITION, an event-driven, double-blind trial in patients with WHO functional class II/III PAH. Treatment-naive patients were randomised 2:1:1 to once-daily initial combination therapy with ambrisentan plus tadalafil or monotherapy with ambrisentan or tadalafil, respectively. The primary endpoint was time to the first clinical failure event (first occurrence of death, hospitalisation for worsening PAH, disease progression or unsatisfactory long-term clinical response). RESULTS: In the primary analysis set (N=500), 187 patients had CTD-PAH, of whom 118 had SSc-PAH. Initial combination therapy reduced the risk of clinical failure versus pooled monotherapy in each subgroup: CTD-PAH (HR 0.43 (95% CI 0.24 to 0.77)) and SSc-PAH (0.44 (0.22 to 0.89)). The most common AE was peripheral oedema, which was reported more frequently with initial combination therapy than monotherapy in the two PAH subgroups. The relative frequency of adverse events between those on combination therapy versus monotherapy was similar across subgroups. CONCLUSIONS: This post hoc subgroup analysis provides evidence that CTD-PAH and SSc-PAH patients benefit from initial ambrisentan and tadalafil combination therapy. TRIAL REGISTRATION NUMBER: NCT01178073, post results.
[Mh] Termos MeSH primário: Anti-Hipertensivos/uso terapêutico
Hipertensão Pulmonar/tratamento farmacológico
Fenilpropionatos/uso terapêutico
Inibidores da Fosfodiesterase 5/uso terapêutico
Piridazinas/uso terapêutico
Escleroderma Sistêmico/complicações
Tadalafila/uso terapêutico
[Mh] Termos MeSH secundário: Adulto
Idoso
Progressão da Doença
Método Duplo-Cego
Quimioterapia Combinada
Edema/induzido quimicamente
Feminino
Seres Humanos
Hipertensão Pulmonar/etiologia
Lúpus Eritematoso Sistêmico/complicações
Masculino
Meia-Idade
Doença Mista do Tecido Conjuntivo/complicações
[Pt] Tipo de publicação:JOURNAL ARTICLE; RANDOMIZED CONTROLLED TRIAL
[Nm] Nome de substância:
0 (Antihypertensive Agents); 0 (Phenylpropionates); 0 (Phosphodiesterase 5 Inhibitors); 0 (Pyridazines); 742SXX0ICT (Tadalafil); HW6NV07QEC (ambrisentan)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170821
[Lr] Data última revisão:
170821
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170101
[St] Status:MEDLINE
[do] DOI:10.1136/annrheumdis-2016-210236


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[PMID]:27788293
[Au] Autor:Ferucci ED; Johnston JM; Gordon C; Helmick CG; Lim SS
[Ad] Endereço:Alaska Native Tribal Health Consortium, Anchorage, Alaska.
[Ti] Título:Prevalence of Mixed Connective Tissue Disease in a Population-Based Registry of American Indian/Alaska Native People in 2007.
[So] Source:Arthritis Care Res (Hoboken);69(8):1271-1275, 2017 Aug.
[Is] ISSN:2151-4658
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To determine the prevalence of mixed connective tissue disease (MCTD) in 2007 in the Indian Health Service (IHS) active clinical population from 3 regions of the US. METHODS: The IHS Lupus Registry was designed to identify possible MCTD cases in addition to systemic lupus erythematosus cases. The population denominator for this report includes American Indian or Alaska Native adults within the IHS active clinical population in 2007, residing in select communities in 3 regions of the US. Potential MCTD cases were identified using a broad range of diagnostic codes and were confirmed by detailed medical record abstraction. Classification as MCTD for this analysis required both rheumatologist diagnosis of MCTD without diagnosis of other CTD, and documentation of the Alarcón-Segovia MCTD criteria in the medical record. Prevalence was also calculated using 2 alternate definitions of MCTD. RESULTS: The age-adjusted prevalence of MCTD using our primary definition was 6.4 per 100,000 (95% confidence interval 2.8-12.8). The prevalence was higher in women than in men using all 3 definitions of MCTD, and no men met the criteria for the primary definition of MCTD. CONCLUSION: The first population-based estimates of the prevalence of MCTD in the US American Indian/Alaska Native population show that the prevalence appears to be higher than in other populations. Additional population-based estimates are needed to better understand the epidemiology of MCTD.
[Mh] Termos MeSH primário: Nativos do Alasca/etnologia
Índios Norte-Americanos/etnologia
Doença Mista do Tecido Conjuntivo/diagnóstico
Doença Mista do Tecido Conjuntivo/etnologia
Vigilância da População
Sistema de Registros
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Vigilância da População/métodos
Prevalência
Estudos Retrospectivos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170831
[Lr] Data última revisão:
170831
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161028
[St] Status:MEDLINE
[do] DOI:10.1002/acr.23135


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[PMID]:27703051
[Au] Autor:Bortoluzzi A; Furini F; Campanaro F; Govoni M
[Ad] Endereço:Department of Medical Sciences, Rheumatology Unit, University of Ferrara and Azienda Ospedaliero-Universitaria Sant'Anna, Cona (Ferrara), Italy.
[Ti] Título:Application of SLICC classification criteria in undifferentiated connective tissue disease and evolution in systemic lupus erythematosus: analysis of a large monocentric cohort with a long-term follow-up.
[So] Source:Lupus;26(6):616-622, 2017 May.
[Is] ISSN:1477-0962
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Objectives The objectives of this study were to analyse the performance of the Systemic Lupus International Collaborating Clinics (SLICC) 2012 classification criteria for systemic lupus erythematosus (SLE) in a large cohort of undifferentiated connective tissue disease (UCTD) population at onset of the disease and during a long-term follow-up of 15 years (1999-2013) and to evaluate the transition from UCTD to SLE, according to American College of Rheumatology (ACR) 1997 and SLICC 2012 classification criteria. Methods A cohort of patients who met the classification criteria proposed by Mosca et al. for UCTD, were analysed. The SLICC 2012 classification criteria for SLE were retrospectively applied to each patient at the time of the diagnosis (T0) and also periodically re-applied and compared to ACR 1997 criteria at three different time points in the follow-up. Results 329 patients were enrolled. According to inclusion criteria at T0 no patient met the SLE/ACR criteria, whilst, retrospectively applying the SLE/SLICC criteria, 44 patients already satisfied this set of criteria for SLE. During the follow-up 23 new patients reached the SLE/SLICC criteria and 14 patients met the ACR criteria with a stable rate of progression to SLE over time. Acute or subacute skin rash, antiphospholipid antibody (aPL) positivity and serositis were the variables correlated to the evolution to SLE. Conclusions In our UCTD population, the application of SLICC classification criteria for SLE at disease onset allowed identification of a proportion of otherwise missed SLE cases; during follow-up, and compared with ACR criteria, SLICC criteria expanded the number of patients classifiable as SLE otherwise classified as UCTD.
[Mh] Termos MeSH primário: Lúpus Eritematoso Sistêmico/diagnóstico
Doença Mista do Tecido Conjuntivo/classificação
[Mh] Termos MeSH secundário: Adulto
Progressão da Doença
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Estudos Retrospectivos
Índice de Gravidade de Doença
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170915
[Lr] Data última revisão:
170915
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161006
[St] Status:MEDLINE
[do] DOI:10.1177/0961203316671814



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