RESUMO
OBJECTIVES: ACA-positive/primary Sjögren's syndrome (pSS) represents a distinct overlapping entity with intermediate features in between limited systemic sclerosis (lSSc) and pSS. Few data are available on their general risk for lymphoproliferative complications, specifically regarding adverse predictors at the level of minor salivary gland (MSG) histology. The objectives of this work are: a) to characterise, through a detailed immunohistochemistry study, the organisation of the lymphomonocitic infiltrates in ACA-positive/pSS patient vs. ACA-negative/pSS patients focusing on the presence of GC-like structures in minor salivary gland biopsies; b) to compare the frequency of traditional clinical and serological risk factors for lymphoma between the two subgroups. METHODS: We analysed 28 MSG samples from ACA-positive/pSS patients and 43 consecutive MSGs from ACA-negative/pSS, using sequential IHC staining for CD3, CD20 and CD21 in order to define the T/B cell segregation within the periductal infiltrates and presence of ectopic GC-like on the detection of GC-like structures. Clinical and serological data of all the patients were retrieved and analysed. RESULTS: Ectopic lymphoid structures (ELS) with GC-like structures were observed in 7 out of 28 ACA-positive/pSS patients (25%) and in 13 out of 43 ACA-negative/pSS patients (30.2%). Similarly, no statistical significant difference was found between the two groups as far as the classical pSS risk factors for lymphoproliferative complications was concerned (i.e. salivary gland enlargement, purpura, low C4, leukocytopenia, clonal gammopathy). Finally, the 3 cases of non-Hodgkin's lymphoma observed were equally distributed between the two subsets. CONCLUSIONS: Overall, this study indicates that ACA-positive/and ACA-negative pSS patients apparently present a similar risk for lymphoproliferative complications as suggested indirectly by the analogies between the two groups observed at the histopathology level.
Assuntos
Anticorpos Antinucleares/imunologia , Centrômero/imunologia , Transtornos Linfoproliferativos/imunologia , Glândulas Salivares Menores/patologia , Síndrome de Sjogren/imunologia , Síndrome de Sjogren/patologia , Adulto , Idoso , Anticorpos Antinucleares/sangue , Antígenos CD20/análise , Biomarcadores/sangue , Biópsia , Complexo CD3/análise , Progressão da Doença , Feminino , Humanos , Imuno-Histoquímica , Itália , Londres , Linfoma/imunologia , Linfoma/patologia , Transtornos Linfoproliferativos/sangue , Transtornos Linfoproliferativos/patologia , Pessoa de Meia-Idade , Fenótipo , Receptores de Complemento 3d , Estudos Retrospectivos , Fatores de Risco , Neoplasias das Glândulas Salivares/imunologia , Neoplasias das Glândulas Salivares/patologia , Glândulas Salivares Menores/imunologia , Síndrome de Sjogren/sangueRESUMO
AIMS: (i) To analyze the in vivo corneal structure and sub-basal plexus nerves in patients with primary Sjögren's syndrome (pSS) and no-SS dry eye by confocal scanning laser microscopy (CSLM) and (ii) to correlate CSLM findings with tear function tests and with patients' subjective dryness. METHODS: Seventeen patients with pSS, 16 no-SS dry eye, and 20 healthy volunteers were included. CSLM parameters taken into consideration included: basal epithelial integrity, corneal thickness, epithelial cellular density, keratocyte activation, and sub-basal plexus morphology. Statistical analysis was carried out using SPSS-13 (Chicago IL, USA). RESULTS: CSLM pachymetric data and the superficial epithelium cell density were significantly lower in pSS versus no-SS dry eye (p < 0.0001); keratocyte activation and sub-basal nerve abnormalities were also more frequent in pSS patients (p < 0.0001). CSLM findings well correlated with both the ocular test results and the patients' perception of ocular dryness at the baseline and over the follow-up. CONCLUSION: CSLM might be a useful novel tool in the assessment of the involvement of the lachrymal functional unit in pSS.
Assuntos
Córnea/patologia , Doenças da Córnea/patologia , Síndrome de Sjogren/patologia , Contagem de Células , Feminino , Humanos , Masculino , Microscopia Confocal/métodos , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Índice de Gravidade de DoençaRESUMO
OBJECTIVES: We aimed to assess the prevalence of patients with either primary Sjögren's syndrome (pSS) and positive anticentromere antibodies (ACA) and secondary Sjögren's syndrome (sSS) and limited cutaneous ACA positive-systemic sclerosis (SSc) in two large cohorts of patients with pSS and SSc¸ and also to compare the clinical features of these two subsets with those of patients affected by 'ACA-positive SSc without sicca symptoms' and 'pSS'. METHODS: In this retrospective monocentric study, the case records of 'overlap' patients fulfilling both the classification criteria for SS and the LeRoy criteria for early SSc were identified from two datasets of patients with limited cutaneous ACA positive SSc (209 subjects) and with pSS (402 subjects) who attended our Rheumatology Unit in the years between 1989 and 2011. Control groups were represented by SSc subjects without sicca symptoms ('SSc group') and ACA negative Pss patients ('pSS group'). SSc patients with sicca symptoms ('Sicca-SSc group') who did not complete the diagnostic algorithm for SS were excluded from the analysis. Demographic, clinical and immunological data of the patients enrolled were collected cumulatively over the entire follow up period. Statistical analysis was performed using SPSS 13 (SPSS Inc., Chicago IL, USA). RESULTS: Out of the two datasets 41 'overlap' patients were selected. The control groups were represented by 102/209 SSc subjects without sicca symptoms ('SSc group') and 387/402 pSS patients ('pSS group'). Eighty-one 'sicca-SSc' with an incomplete work-up for SS were excluded from the analysis. The prevalence of ACA positive pSS patients among pSS was 3.7% (15/402), while the frequency of patients with definite sSS in the SSc cohort was 20% (26/128). No differences were detected between 'overlap' patients and control groups, relatively to demographic characteristics. 'Overlap patients' were characterised by a milder SSc disease (i.e. lower frequency of sclerodactily, negative evolution of the capillaroscopy pattern or absence of severe systemic involvement) whereas, as far as the SS-related manifestations were concerned, although often lacking in specific autoantibodies (i.e. rheumatoid factor, anti-Ro/SSA, anti-La/SSB), the 'overlap patients' displayed a full blown SS phenotype with recurrent salivary gland enlargement, purpura, fatigue, arthralgias, and leukocytopenia. It is noteworthy that the prevalence of non-Hodgkin's lymphoma in the 'overlap patients' was higher than in pSS. CONCLUSIONS: Taken together, the results of our work emphasise the existence of a novel distinct clinical entity which might tentatively be called 'ACA-positive limited scleroderma/SS overlap syndrome' characterised by a benign SSc clinical course but at a high risk of non-Hodgkin's lymphoma.
Assuntos
Anticorpos Antinucleares/sangue , Linfoma não Hodgkin/epidemiologia , Esclerodermia Limitada/diagnóstico , Síndrome de Sjogren/diagnóstico , Análise de Variância , Biomarcadores/sangue , Distribuição de Qui-Quadrado , Humanos , Itália , Modelos Logísticos , Linfoma não Hodgkin/imunologia , Valor Preditivo dos Testes , Prevalência , Prognóstico , Estudos Retrospectivos , Fator Reumatoide/sangue , Medição de Risco , Fatores de Risco , Esclerodermia Limitada/sangue , Esclerodermia Limitada/epidemiologia , Esclerodermia Limitada/imunologia , Índice de Gravidade de Doença , Síndrome de Sjogren/sangue , Síndrome de Sjogren/epidemiologia , Síndrome de Sjogren/imunologiaRESUMO
Purpose: This study aims to describe the functional status of a cohort of subacute COVID-19 patients treated in a dedicated rehabilitation unit and to compare functional outcomes between patients previously hospitalized in the intensive care unit (ICU group) and patients assisted in the medical care unit (MCU group). Materials and methods: Clinical and functional evaluations were performed at admission and discharge. The functional status was assessed using Barthel index (BI), functional ambulation categories (FAC), trunk control test (TCT), and dysphagia outcome and severity score (DOSS). All patients received multidisciplinary tailored rehabilitation. Results: We evaluated 171 patients (with a mean age of 67.7 ± 11.9 years, 117 were males), 110 coming from the ICU (with a mean age of 63.24 ± 10.9 years), and 61 coming from the MCU (with a mean age of 75.75 ± 9.09 years). The ICU group showed a worse functional status at admission compared with the MCU group [BI 2.5 (0-20) vs. 20 (10-60), FAC 0 (0-0) vs. 0 (0-2), TCT 61 (42-100) vs. 100 (61-100), DOSS 5 (1-7) vs. 7 (7-7)] and had significantly longer hospital stay. At discharge, all functional scales were improved with no statistically significant differences between the two groups. Conclusion: Early rehabilitation of COVID-19 survivors improves functional recovery closing the initial gap between the ICU and MCU groups. In addition, it is effective to improve the functional outcome reducing the costs for longer-term assistance of COVID-19 patients.
RESUMO
BACKGROUND: Adherence to treatment is one of the most common problems in patients suffering from chronic disease such as osteoporosis, and special commitment is required to patients, especially regarding rehabilitation. There is increasing evidence that physical interventions aimed at relieving pain and reducing physical impairments could play a crucial role in improving the quality of life and reducing the risk of fractures in patients with severe osteoporosis. OBJECTIVE: The aim of this study was to assess the compliance and determine the acceptability of a home-self-managed exercises program in patients with vertebral fractures, one of the most frequent and serious consequences of osteoporosis. METHODS: We conducted a retrospective observational study of patients undergoing a home exercise program, monitoring them with clinical scales, questionnaires, and routine visits. RESULTS: 62.86% of the patients were compliant with the treatment; the absence of supervision by health personnel was the primary cause of non-compliance, followed by the lack of time and the lack of motivation. Compliant patients showed a significant reduction in lumbar pain (p 0.011), an improvement in posture with a reduction of dorsal kyphosis (occipital-wall distance T0-T1, p-value = 0.02) and an improvement in QoL (p-value = 0.001) and physical performance at the 20 m walking test (p-value = 0.003). CONCLUSIONS: A home exercise program is feasible and could improve signs and symptoms in patients with vertebral fractures due to OP.
Assuntos
Dor Lombar , Osteoporose , Fraturas por Osteoporose , Fraturas da Coluna Vertebral , Terapia por Exercício , Humanos , Dor Lombar/complicações , Osteoporose/complicações , Osteoporose/terapia , Fraturas por Osteoporose/terapia , Qualidade de Vida , Fraturas da Coluna Vertebral/terapiaAssuntos
Antimaláricos/administração & dosagem , Imunossupressores/administração & dosagem , Síndrome de Sjogren/tratamento farmacológico , Adulto , Idoso , Antimaláricos/efeitos adversos , Esquema de Medicação , Substituição de Medicamentos , Feminino , Humanos , Imunossupressores/efeitos adversos , Masculino , Adesão à Medicação , Pessoa de Meia-Idade , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/imunologia , Fatores de Tempo , Resultado do TratamentoRESUMO
Giant coronary artery aneurysm (CAA) is a rare clinicopathologic entity, and there is a dearth of information regarding presentation, diagnosis, and subsequent management. We present a case of a giant aneurysm of the proximal right coronary artery (RCA) that was 1.5 × 1.5 cm with direct communication between the aneurysmal cavity and the aortic sinus. The deficit in the RCA ostium was closed with a patch of bovine pericardium. The distal communication of the aneurysm to the mid-RCA was clearly delineated and tied off. The distal RCA was grafted under bypass with a long saphenous vein. Although our case is not unique, it does represent an uncommon presentation of a rare disease process.