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3.
Scand J Rheumatol ; 49(3): 239-243, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31928291

RESUMO

Objective: Despite being a cardinal clinical sign of systemic sclerosis (SSc), digital pitting has been little studied. Our objective was to test, in a pilot study, the hypothesis that pitting is painful and associated with digital vascular disease severity.Method: Fifty patients with SSc were recruited: 25 with and 25 without digital pitting. Fingertip pain was assessed on a 0-10 scale. Thermography of both hands assessed surface temperature, allowing calculation of the distal-dorsal difference (temperature gradient) for each finger. Nailfold capillaroscopy was performed in each finger using a dermatoscope, and graded on a 0-3 scale (0 = normal; 3 = grossly abnormal).Results: In the 25 patients with digital pitting, 65 fingers in total were affected (mainly the index and middle fingers). Pain scores were higher in 'pitting' patients [median 4 (interquartile range 3-8) vs 0 (0-2), p < 0.001], and pitting patients reported that pitting impacted on activities of everyday living. Temperature gradients along the fingers did not differ significantly between patients with and without pitting (p = 0.248). Pitting patients were more likely to have 'grossly abnormal' capillaries than those without pitting, and less likely to have 'no/mild' nailfold capillary changes.Conclusions: Digital pitting is painful and impacts on hand function. Capillaroscopy findings provide further support for an association between pitting and severity of digital vascular change. Larger, more comprehensive studies are required to examine the pathophysiology of pitting and to pave the way to therapeutic intervention, ideally including preventive strategies.


Assuntos
Dedos/fisiopatologia , Dor/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/fisiopatologia , Idoso , Estudos de Casos e Controles , Feminino , Dedos/irrigação sanguínea , Dedos/patologia , Humanos , Masculino , Angioscopia Microscópica , Pessoa de Meia-Idade , Dor/etiologia , Projetos Piloto , Esclerodermia Difusa/complicações , Esclerodermia Difusa/patologia , Esclerodermia Limitada/complicações , Esclerodermia Limitada/patologia , Escleroderma Sistêmico/fisiopatologia , Venenos de Escorpião , Índice de Gravidade de Doença , Úlcera Cutânea/etiologia , Termografia
4.
Am J Gastroenterol ; 114(12): 1847-1856, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31805016

RESUMO

OBJECTIVES: Although systemic sclerosis (SSc) is known to affect the gastrointestinal (GI) tract, most of the literature focuses on esophageal, small intestinal, or anorectal manifestations. There have been no reviews focused on large bowel SSc complications in over 30 years. The aim of this study is to perform a systematic review of colonic manifestations and complications of SSc. METHODS: An experienced librarian conducted a search of databases, including English and Spanish articles. The search used keywords including "systemic sclerosis," "scleroderma," and "colon." A systematic review was performed using Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines. Case reports/series were screened for validity by adapting from criteria published elsewhere. RESULTS: Of 1,890 articles, 74 met selection criteria. Fifty-nine of the 77 articles were case reports/series. The most common article topics on colonic SSc complications were constipation/dysmotility (15), colonic volvulus (8), inflammatory bowel disease (7), microscopic colitis (6), megacolon (6), and telangiectasia (6). Colonic manifestations constituted 24% of articles on GI complications of SSc. There were a total of 85 cases (84% women, with a median age of onset of colon complication of 52 years). Limited cutaneous SSc phenotype (65.6%) was more common than diffuse (26.2%). Patients frequently had poor outcomes with high mortality related to colonic complications (27%). Recent studies explore contemporary topics such as the microbiome in SSc and prucalopride for chronic constipation in SSc. DISCUSSION: Colonic complications comprise a large proportion of the published reports on GI symptoms afflicting patients with SSc and require raised diagnostic suspicion and deliberate action to avoid potentially serious complications including death.


Assuntos
Doenças do Colo/fisiopatologia , Escleroderma Sistêmico/fisiopatologia , Colite Microscópica/etiologia , Colite Microscópica/fisiopatologia , Doenças do Colo/etiologia , Constipação Intestinal/etiologia , Constipação Intestinal/fisiopatologia , Humanos , Doenças Inflamatórias Intestinais/etiologia , Doenças Inflamatórias Intestinais/fisiopatologia , Volvo Intestinal/etiologia , Volvo Intestinal/fisiopatologia , Megacolo/etiologia , Megacolo/fisiopatologia , Esclerodermia Difusa/complicações , Esclerodermia Difusa/fisiopatologia , Esclerodermia Limitada/complicações , Esclerodermia Limitada/fisiopatologia , Escleroderma Sistêmico/complicações , Telangiectasia/etiologia , Telangiectasia/fisiopatologia
5.
BMJ Case Rep ; 12(11)2019 Nov 26.
Artigo em Inglês | MEDLINE | ID: mdl-31776158

RESUMO

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/enzimologia , Mieloblastina , Doenças do Sistema Nervoso Periférico/complicações , Esclerodermia Difusa/complicações , Idoso , Humanos , Masculino
6.
Drug Discov Ther ; 13(5): 297-298, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31723102

RESUMO

Myocardial injury with systemic sclerosis (SSc) causes pericarditis and arrhythmia, and polymyositis-induced muscle inflammation causes myocarditis. We report a rare case of overlap syndrome (SSc and polymyositis) who presented with sudden chest pain secondary to myocardial fibrosis. Although the etiology of chest symptoms in collagen disease was difficult to identify, cardiac magnetic resonance imaging (MRI) revealed not myocarditis but myocardial fibrosis in our case. Synthetic judgement of serum brain natriuretic peptide/ troponin T levels and cardiac MRI is useful in the search for the cause of chest symptoms even in patients with collagen diseases.


Assuntos
Dor no Peito/diagnóstico , Fibrose/diagnóstico por imagem , Fibrose/patologia , Coração/diagnóstico por imagem , Miocárdio/patologia , Polimiosite/complicações , Esclerodermia Difusa/complicações , Dor no Peito/complicações , Fibrose/complicações , Humanos , Imagem por Ressonância Magnética , Masculino , Peptídeo Natriurético Encefálico/sangue , Polimiosite/sangue , Polimiosite/diagnóstico , Esclerodermia Difusa/sangue , Esclerodermia Difusa/diagnóstico , Síndrome , Troponina T/sangue , Adulto Jovem
7.
Mycopathologia ; 184(6): 787-793, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31729682

RESUMO

Pneumocystis pneumonia (PCP) is a life-threatening fungal infection occurring in immunocompromised patients such as HIV-positive patients with low CD4 cell count or patients under heavy immunosuppressive therapy. We report the case of a 59-year-old male with severe diffuse cutaneous systemic sclerosis presenting with asthenia, dry cough and worsening shortness of breath for the last 15 days. Biological studies were remarkable for PTH-independent severe hypercalcemia with low 25-hydroxyvitamin D and a paradoxically elevated 1,25-dihydroxyvitamin D. Early bronchoalveolar lavage allowed for PCP diagnosis and targeted treatment. We discuss the underlying physiopathology and difficulties regarding prophylaxis and treatment.


Assuntos
Hipercalcemia/fisiopatologia , Pneumocystis carinii , Pneumonia por Pneumocystis , Esclerodermia Difusa/complicações , Humanos , Hospedeiro Imunocomprometido , Imunoterapia/efeitos adversos , Masculino , Pessoa de Meia-Idade , Infecções Oportunistas/diagnóstico , Pneumocystis carinii/efeitos dos fármacos , Pneumocystis carinii/patogenicidade , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/tratamento farmacológico , Pneumonia por Pneumocystis/fisiopatologia , Combinação Trimetoprima e Sulfametoxazol/administração & dosagem , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico
8.
Osteoporos Int ; 30(8): 1679-1691, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31030240

RESUMO

Radius and tibia bone microarchitecture, analyzed through a high-resolution peripheral quantitative computed tomography, were significantly impaired in female patients with diffuse systemic sclerosis compared with healthy controls. Acroosteolysis, quality of life-grip strength, hand disability, and disease duration were significantly associated with this bone deterioration. INTRODUCTION: The effect of diffuse systemic sclerosis (dSSc) on the bone is not completely understood. The objective of this study was to analyze the volumetric bone mineral density (vBMD), microarchitecture, and biomechanical parameters at the distal radius and tibia using high-resolution peripheral quantitative computed tomography (HR-pQCT, XtremeCT) in female patients with dSSc and identify clinical and laboratory variables associated with these parameters. METHODS: Thirty-eight women with dSSc and 76 healthy controls were submitted to HR-pQCT at the distal radius and tibia. Clinical and laboratory findings, bone mineral density(BMD), nailfold capillaroscopy (NC), total passive range of motion(ROM), and quality of life (health assessment questionnaire-HAQ) were associated with HR-pQCT (Scanco Medical AG, Brüttisellen, Switzerland) parameters. Multiple linear regression models adjusted for clinical and laboratory variables, ROM and HAQ, were performed. RESULTS: Density, microarchitecture, and biomechanical parameters at the distal radius and tibia were significantly impaired in dSSc patients compared with healthy controls (p < 0.001). Multiple linear regression models showed that lower trabecular density (Tb.vBMD) (radius R2 = 0.561, p = 0.002; and tibia R2 = 0.533, p = 0.005), and lower trabecular number (Tb.N) (tibia R2 = 0.533, p = 0.005) were significantly associated with acroosteolysis. Higher trabecular separation (Tb.Sp) was associated with disease duration and higher HAQ-grip strength (radius R2 = 0.489, p = 0.013), while cortical density (Ct.vBMD) was associated with ROM (radius R2 = 0.294, p = 0.002). CONCLUSION: Bone microarchitecture in patients with dSSc, analyzed through HR-pQCT, showed impairment of trabecular and cortical bone at distal radius and tibia. Variables associated with hand involvement (acroosteolysis, quality of life-grip strength, and ROM) and disease duration may be considered prognostic factors of this bone impairment.


Assuntos
Densidade Óssea/fisiologia , Rádio (Anatomia)/fisiopatologia , Esclerodermia Difusa/fisiopatologia , Tíbia/fisiopatologia , Acro-Osteólise/etiologia , Acro-Osteólise/fisiopatologia , Adolescente , Adulto , Antropometria/métodos , Fenômenos Biomecânicos/fisiologia , Estudos de Casos e Controles , Feminino , Articulações dos Dedos/fisiopatologia , Força da Mão/fisiologia , Humanos , Angioscopia Microscópica , Pessoa de Meia-Idade , Qualidade de Vida , Rádio (Anatomia)/diagnóstico por imagem , Amplitude de Movimento Articular/fisiologia , Esclerodermia Difusa/complicações , Esclerodermia Difusa/diagnóstico por imagem , Tíbia/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto Jovem
9.
Ann Rheum Dis ; 78(5): 648-656, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30852552

RESUMO

OBJECTIVES: To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). METHODS: We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. RESULTS: Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). CONCLUSIONS: Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice.


Assuntos
Esclerodermia Difusa/mortalidade , Esclerodermia Difusa/patologia , Dermatopatias/mortalidade , Dermatopatias/fisiopatologia , Pele/patologia , Adulto , Estudos de Coortes , Bases de Dados Factuais , Progressão da Doença , Europa (Continente) , Feminino , Fibrose , Humanos , Estimativa de Kaplan-Meier , Pulmão/fisiopatologia , Masculino , Pessoa de Meia-Idade , Esclerodermia Difusa/complicações , Índice de Gravidade de Doença , Dermatopatias/etiologia , Análise de Sobrevida , Fatores de Tempo
11.
Rheumatology (Oxford) ; 58(7): 1221-1226, 2019 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-30690570

RESUMO

OBJECTIVES: To screen for significant arrhythmias with an implantable loop recorder (ILR) in patients with SSc and no known cardiovascular disease, and identify associated disease phenotype, blood and cardiovascular magnetic resonance (CMR) biomarkers. METHODS: Twenty patients with SSc with no history of primary SSc heart disease, traditional cardiovascular disease, diabetes or maximum one traditional cardiovascular risk factor underwent clinical assessment, contrast-enhanced CMR and ILR insertion. RESULTS: ILR data were available for 19 patients: 63% female, mean (s.d.) age of 53 (12) years, 32% diffuse SSc. Eight patients had significant arrhythmias over 3 years: one complete heart block, two non-sustained ventricular tachycardia [all three dcSSc, two anti-topoisomerase antibodies (Scl70) positive, three interstitial lung disease and two previous digital ulceration] and five atrial arrhythmias of which four were with limited SSc. These required interventions with one permanent pacemaker implantation, four anti-arrhythmic pharmacotherapy, one anticoagulation.Patients with significant arrhythmia had higher baseline high-sensitivity troponin I and N-terminal pro-brain natriuretic peptide [mean difference (95% CI) 117 (-11, 245) and 92 (-30, 215) ng/l, respectively], and CMR-extracellular volume [mean (s.d.) 32 (2) vs 29 (4)%]. Late gadolinium enhancement was observed in five patients, only one with significant arrhythmia. CONCLUSION: This first ILR study identified potentially life-threatening arrhythmias in asymptomatic SSc patients attributable to a primary SSc heart disease. Disease phenotype, CMR-extracellular volume (indicating diffuse fibrosis) and cardiac biomarkers may identify at-risk patients that would benefit from ILR screening. Future studies can inform a risk model and provide insights into SSc-associated arrhythmia pathogenesis.


Assuntos
Arritmias Cardíacas/etiologia , Miocárdio/patologia , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Esclerodermia Difusa/complicações , Troponina I/sangue , Adulto , Idoso , Arritmias Cardíacas/diagnóstico , Biomarcadores/sangue , Eletrocardiografia Ambulatorial/métodos , Feminino , Fibrose , Coração/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética/métodos , Masculino , Programas de Rastreamento/métodos , Pessoa de Meia-Idade , Projetos Piloto , Prognóstico , Estudos Prospectivos , Esclerodermia Difusa/sangue
12.
JNMA J Nepal Med Assoc ; 57(220): 457-459, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-32335662

RESUMO

Systemic sclerosis is a connective tissue disease characterized by wide-spread vascular lesions and fibrosis of the skin and internal organs. It is an immune mediated rheumatic disease with the presence of an immunological dysfunction of T lymphocytes, especially Th1 and Th17 subtypes. It affects gastrointestinal, pulmonary, vascular, musculoskeletal, cardiac and various other systems. This disease is rare but has high morbidity and mortality with less known effective management. We report a case of 70-year-old female with systemic sclerosis presented with pain along with swelling over multiple joints since 18 months which exacerbated since last 6 months and wound over finger tips since last 2 weeks. We present here other various signs, investigations and management of this uncommon disease systemic sclerosis, also known as scleroderma. Various systems are evident to be involved including cardiac (left ventricular diastolic dysfunction) and peripheral vascular system (Raynaud's phenomenon). Keywords: connective tissue disease; immune-mediated; rheumatic disease; scleroderma; systemic sclerosis.


Assuntos
Diástole/fisiologia , Esclerodermia Difusa/diagnóstico , Disfunção Ventricular Esquerda/diagnóstico por imagem , Idoso , Ecocardiografia Doppler , Feminino , Humanos , Esclerodermia Difusa/complicações , Esclerodermia Difusa/patologia , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/fisiopatologia
14.
Am J Dermatopathol ; 41(2): 135-136, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30235169

RESUMO

We report a case of a 65-year-old man who developed an asymptomatic bluish spot that affected the flank and left lumbar region with the onset 10 years prior. He had a history of diffuse systemic sclerosis with anti-Scl-70-positive antibodies. The appearance of the skin lesion coincided with the onset of his disease. The skin biopsy was consistent with the diagnosis of acquired dermal melanocytosis. The relationship between the appearance of acquired pigmented macules and spots and systemic sclerosis has been known for years, although it is an infrequent finding.


Assuntos
Melanose/complicações , Esclerodermia Difusa/complicações , Idoso , Grupo com Ancestrais do Continente Europeu , Humanos , Masculino
15.
Clin Exp Rheumatol ; 36 Suppl 113(4): 68-75, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30277860

RESUMO

OBJECTIVES: To describe differences in clinical presentation between men and women in a large group of patients with early (<3 years' duration) systemic sclerosis (SSc) according to disease subsets. METHODS: A cross-sectional analysis of the prospective EULAR Scleroderma Trial and Research database (EUSTAR) was performed. Patients fulfilling preliminary ACR 1980 classification criteria for SSc, with less than 3 years from the first non-Raynaud's symptom at first entry, were selected. A group of patients with less than 3 years from the first SSc symptom, including Raynaud's phenomenon, was also analysed. SSc related variables, including antibodies, SSc subsets, disease activity and organ involvement were included. Descriptive and bivariate analyses were performed. RESULTS: A total of 1,027 patients were included, 90% Caucasian, 80% women, and 40% with diffuse cutaneous disease. In early stages of SSc, men showed more frequently than women active disease, diffuse cutaneous subset, anti-Scl-70 antibodies, elevated acute phase reactants, muscular and pulmonary involvement. Differences between men and women were confirmed in the limited, but not in the diffuse SSc subset. The results were similar when 650 patients with less than three years from the first SSc symptom, including Raynaud's phenomenon, were analysed. CONCLUSIONS: In early stages of SSc, men present signs and symptoms of more severe disease. In the limited disease subset, men might appear with clinical features and organ involvement similar to those of the diffuse subgroup. In clinical practice, the identification of such differences might help to select the appropriate management for each particular patient.


Assuntos
Disparidades nos Níveis de Saúde , Esclerodermia Difusa/diagnóstico , Esclerodermia Limitada/diagnóstico , Proteínas da Fase Aguda/análise , Autoanticorpos/sangue , Biomarcadores/sangue , Estudos Transversais , Bases de Dados Factuais , Progressão da Doença , Feminino , Humanos , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Masculino , Proteínas Nucleares/imunologia , Prognóstico , Doença de Raynaud/diagnóstico , Doença de Raynaud/etiologia , Fatores de Risco , Esclerodermia Difusa/sangue , Esclerodermia Difusa/complicações , Esclerodermia Difusa/imunologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/complicações , Esclerodermia Limitada/imunologia , Índice de Gravidade de Doença , Fatores Sexuais
16.
Arq Gastroenterol ; 55Suppl 1(Suppl 1): 47-51, 2018 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-30304292

RESUMO

BACKGROUND: Scleroderma or progressive systemic sclerosis is characterized by a chronic inflammatory process with proliferation of fibrous connective tissue and excessive deposition of collagen and extracellular matrix in the skin, smooth muscle, and viscera. The smooth muscle most involved in scleroderma is that of the esophagus, and dysphagia is the most commonly reported symptom. However, the internal anal sphincter may also be impaired by degeneration and fibrosis, leading to concomitant anal incontinence in scleroderma patients. These patients may neglect to complain about it, except when actively questioned. OBJECTIVE: To assess anorectal function and anatomy of female scleroderma patients with symptoms of anal incontinence through Cleveland Clinic Florida Fecal Incontinence Score (CCFIS), anorectal manometry and endoanal ultrasound at the outpatient clinic of colorectal and anal physiology, Clinics Hospital, University of São Paulo Medical School (HC-FMUSP). METHODS: Female scleroderma patients were prospectively assessed and questioned as to symptoms of anal incontinence. The anorectal manometry and endoanal ultrasound results were correlated with clinical data and symptoms. RESULTS: In total, 13 women were evaluated. Their mean age was 55.77 years (±16.14; 27-72 years) and their mean disease duration was 10.23 years (±6.23; 2-23 years). All had symptoms of fecal incontinence ranging from 1 to 15. Seven (53.8%) patients had fecal incontinence score no higher than 7; three (23.1%) between 8 and 13; and three (23.1%) 14 or higher, corresponding to mild, moderate, and severe incontinence, respectively. Ten (76.92%) patients had hypotonia of the internal anal sphincter. Three-dimensional endoanal ultrasound showed tapering associated with muscle atrophy of the internal sphincter in six cases and previous muscle defects in three cases. CONCLUSION: A functional and anatomical impairment of the sphincter is an important factor to assess in patients with progressive systemic sclerosis and it should not be underestimated.


Assuntos
Endossonografia/métodos , Incontinência Fecal/diagnóstico por imagem , Distúrbios do Assoalho Pélvico/diagnóstico por imagem , Esclerodermia Difusa/complicações , Adulto , Idoso , Incontinência Fecal/etiologia , Incontinência Fecal/fisiopatologia , Feminino , Humanos , Imageamento Tridimensional , Manometria , Pessoa de Meia-Idade , Distúrbios do Assoalho Pélvico/etiologia , Distúrbios do Assoalho Pélvico/fisiopatologia , Estudos Prospectivos , Esclerodermia Difusa/fisiopatologia , Índice de Gravidade de Doença
17.
Rheumatol Int ; 38(12): 2279-2288, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30206672

RESUMO

The objective of this study is to investigate the impact of skin sclerosis burden on an internal organ involvement over a 1-year period, as measured by time-adjusted accrual-modified Rodnan skin score (TA-mRSS), and to evaluate association between TA-mRSS patterns and laboratory tests in patients with systemic sclerosis (SSc). This prospective study was conducted at Siriraj Hospital (Bangkok, Thailand) during the November 2013-November 2016. SSc patients by ACR/EULAR 2013 or ACR 1980 criteria were eligible. TA-mRSS was classified as low, intermediate, or high, and then compared between groups. Correlation between the arithmetic mean of laboratory tests and TA-mRSS was assessed by multiple linear regression analysis. A total of 118 patients, with 81.4% women, median (IQR) age 49.8 (43.8, 55.1) years, disease duration from onset of non-Raynaud symptoms to first visit of 3.3 (1, 6.8) years, 78% dcSSc, and 75.3% anti-Scl-70 positivity, were analyzed. TA-mRSS over 1 year ranged from 0 to 37.44. The high skin sclerosis burden group had a median TA-mRSS > 7.26 (> 67th percentile). Patients with high TA-mRSS were dcSSc, high initial and average mRSS, and had tendon friction rub, digital ischemic complications, usual interstitial pneumonia, diastolic dysfunction, gastrointestinal dysmotility, and low serum albumin. In multiple linear regression analysis, the arithmetic mean of hemoglobin (B = - 1.007, 95% CI - 1.779 to - 0.236), erythrocyte sedimentation rate (B = - 0.078, 95% CI - 0.126 to - 0.029), serum glutamic oxaloacetic transaminase (B = 0.073, 95% CI 0.026-0.12), creatine phosphokinase (B = 0.012, 95% CI 0.003-0.021), and albumin (B = - 4.117, 95% CI - 6.958 to - 1.276) were associated with TA-mRSS. This study found a higher cumulative course of mRSS over a 1-year period to be significantly associated with severe internal organ involvement.


Assuntos
Esclerodermia Difusa/complicações , Esclerodermia Limitada/complicações , Pele/patologia , Adulto , Biomarcadores/sangue , Progressão da Doença , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Indução de Remissão , Esclerodermia Difusa/sangue , Esclerodermia Difusa/tratamento farmacológico , Esclerodermia Difusa/patologia , Esclerodermia Limitada/sangue , Esclerodermia Limitada/tratamento farmacológico , Esclerodermia Limitada/patologia , Índice de Gravidade de Doença , Síndrome , Tailândia , Fatores de Tempo , Resultado do Tratamento
18.
Clin Nephrol ; 90(6): 413-418, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30106367

RESUMO

AIMS: Overlap syndrome of ANCA-associated vasculitis (AAV) and scleroderma (SSc) is rare with conflicting data on renal outcomes. We describe the clinical characteristics and treatment outcome of ANCA-associated glomerulonephritis (AAG) in SSc patients followed at a single center. MATERIALS AND METHODS: We conducted a retrospective study of 3,570 patients in our SSc database to identify SSc patients who subsequently developed AAV with renal involvement. Patient demographics, serology, renal function, renal histology, and treatment outcomes were assessed. RESULTS: Of the 3,570 patients, we identified 7 patients who developed acute glomerulonephritis, and all were ANCA positive. The mean age at SSc diagnosis was 47 years, 4 patients were female, and 6 had diffuse SSc. Anti-nuclear antibody (ANA) was positive in all. Mean time of onset of AAV from time of diagnosis of SSc was 6 years, and all were myeloperoxidase (MPO) positive. Patients presented with hematuria, proteinuria, with or without rise in serum creatinine, and all patients had biopsy-proven crescentic glomerulonephritis. One patient required dialysis at presentation. Five patients were treated with cyclophosphamide and steroids, and 2 were treated with rituximab and steroids. Of the 7 patients, 4 did not receive maintenance immunosuppression. Three patients died, and 1 of them experienced relapse with fulminant alveolar hemorrhage. CONCLUSION: AAG in SSc is rare, with disease manifestation and course similar to that of AAV. This case series demonstrates that disease remission can be achieved with standard induction therapy. Vasculitis relapse can occur, and similar to idiopathic AAV, maintenance immunosuppression should be initiated to maintain remission.
.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/complicações , Glomerulonefrite/etiologia , Glomerulonefrite/terapia , Esclerodermia Difusa/complicações , Adulto , Idoso , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/sangue , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos/sangue , Creatinina/sangue , Ciclofosfamida/uso terapêutico , Feminino , Glomerulonefrite/sangue , Glomerulonefrite/patologia , Hematúria/etiologia , Humanos , Fatores Imunológicos/uso terapêutico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Peroxidase/sangue , Proteinúria/etiologia , Recidiva , Diálise Renal , Estudos Retrospectivos , Rituximab/uso terapêutico , Esclerodermia Difusa/sangue , Esteroides/uso terapêutico , Resultado do Tratamento , Adulto Jovem
20.
Rheumatology (Oxford) ; 57(12): 2106-2113, 2018 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-30053212

RESUMO

Objectives: SSc is characterized by fibrotic changes in the skin and lung, and the mainstay of treatment has been CYC. B cell involvement suggests that rituximab (RTX) may also be of therapeutic benefit. The aim of the study was to compare the efficacy and safety of RTX compared with CYC in retarding the progression of interstitial lung disease and skin manifestations of primary SSc. Methods: We randomly assigned 60 patients of dcSSc, age 18-60 years with skin and lung involvement, to monthly pulses of CYC 500 mg/m2 or RTX 1000 mg × 2 doses at 0, 15 days. Primary outcomes were forced vital capacity (FVC) percent predicted at 6 months. Secondary outcomes were: absolute change in litres (FVC-l) at 6 months; modified Rodnan skin scores at 6 months, 6-min walk test, Medsgers score and new onset or worsening of existing pulmonary hypertension by echocardiographic criteria. Results: The FVC [%mean (s.d.)] in the RTX group improved from 61.30 (11.28) to 67.52 (13.59), while in the CYC group it declined from 59.25 (12.96) to 58.06 (11.23) at 6 months (P = 0.003). The change of FVC was 1.51 (0.45) l to 1.65 (0.47) l in the RTX group, compared with 1.42 (0.49) to 1.42 (0.46) l in the CYC group. The mRSS changed from 21.77 (9.86) to 12.10 (10.14) in the RTX group and 23.83 (9.28) to 18.33 (7.69) in the CYC group after 6 months. Serious adverse events were more common in the CYC group. Conclusion: RTX is a safe and effective alternative to CYC in the primary therapy of skin and lung manifestations of scleroderma. Trial registration: Clinical Trials Registry - India, www.ctri.nic.in, CTRI/2017/07/009152.


Assuntos
Antirreumáticos/uso terapêutico , Ciclofosfamida/uso terapêutico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Rituximab/uso terapêutico , Esclerodermia Difusa/tratamento farmacológico , Adulto , Progressão da Doença , Feminino , Humanos , Pulmão/patologia , Doenças Pulmonares Intersticiais/etiologia , Masculino , Esclerodermia Difusa/complicações , Esclerodermia Difusa/patologia , Índice de Gravidade de Doença , Pele/patologia , Resultado do Tratamento , Capacidade Vital/efeitos dos fármacos , Teste de Caminhada
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