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1.
Clin Exp Rheumatol ; 2021 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-33938790

RESUMO

People with cryoglobulinaemic vasculitis (CV) have an increased risk of infections, attributed to different causes: impairment of the immune system due to the disease itself, comorbidities, and immunosuppressive therapy. Therefore, these patients may be at high risk for a more severe course of COVID-19, including hospitalisation and death. Concerns about efficacy, immunogenicity and safety of vaccines, as well as doubts, not yet fully clarified in patients with systemic autoimmune diseases, represent other important factors for a low vaccination rate in people with (CV). Indeed, providing an expert position on the issues related to SARS-CoV-2 vaccination in patients suffering from CV is of critical relevance in order to help both patients and clinicians who are treating them in making the best choice in each case. A multidisciplinary task force of the Italian Group for the Study of Cryoglobulinaemia (GISC) was convened, and through a Delphi technique produced provisional recommendations regarding SARS-CoV-2 vaccination in cryoglobulinaemic patients.

3.
J Clin Med ; 10(6)2021 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-33799655

RESUMO

Primary Sjögren's syndrome (pSS) is a chronic and heterogeneous disorder characterized by a wide spectrum of glandular and extra-glandular features. The hallmark of pSS is considered to be the immune-mediated involvement of the exocrine glands and B-cell hyperactivation. This leads pSS patients to an increased risk of developing lymphoproliferative diseases, and persistent (>2 months) major salivary gland enlargement is a well-known clinical sign of possible involvement by B cell lymphoma. Better stratification of the patients may improve understanding of the mechanism underlying the risk of lymphoproliferative disorder. Here, we summarize the role of different imaging techniques and a bioptic approach in pSS patients, focusing mainly on the role of salivary gland ultrasonography (SGUS) and a US-guided core needle biopsy (Us-guided CNB) as diagnostic and prognostic tools in pSS patients with persistent parotid swelling.

4.
Front Immunol ; 12: 615656, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33841398

RESUMO

Purpose: The prevalence of peripheral nervous system (PNS) involvement in primary Sjögren's syndrome (pSS) has been reported to range from 2% to over 50%. Bias in study designs, including low number of patients and unclearly defined rheumatological and neurological diagnosis could explain such variability. Consequently, the exact depiction of PNS involvement in pSS is still lacking. This study aimed at analyzing the prevalence and the clinical and laboratory factors associated with PNS involvement in a very large cohort of well-characterized pSS patients with a clearly defined neurological diagnosis. Methods: Clinical and serological data of 1,695 pSS patients with specific and accurate information on PNS involvement were analyzed. Comparisons between patients with and without PNS involvement and between patients with distinct subsets of PNS manifestations were performed. Results: Prevalence of PNS involvement was 3.7%. The most frequent types observed were pure sensory neuropathies and axonal sensorimotor polyneuropathies (SMP). Patients with PNS involvement exhibited a more active disease profile and were more frequently treated with immunosuppressants. Intriguingly, clinical and serological negative prognostic factors, including purpura, extra-glandular manifestations, leukopenia, low complement and cryoglobulinemia, principally characterized patients with SMP, while subjects with pure sensory neuropathy displayed a milder phenotype. Conclusion: Our results highlight that PNS involvement is rather rare, but prognostically relevant in pSS. Main adverse prognostic features characterize patients with SMP, while pure sensory neuropathies are usually associated with a mild clinical picture. These findings, useful for patient stratification, may suggest protean pathogenic pathways underlying different types of PNS manifestations in pSS.

5.
Ann Rheum Dis ; 2021 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-33547062

RESUMO

OBJECTIVES: Severe systemic inflammation associated with some stages of COVID-19 and in fatal cases led therapeutic agents developed or used frequently in Rheumatology being at the vanguard of experimental therapeutics strategies. The aim of this project was to elaborate EULAR Points to consider (PtCs) on COVID-19 pathophysiology and immunomodulatory therapies. METHODS: PtCs were developed in accordance with EULAR standard operating procedures for endorsed recommendations, led by an international multidisciplinary Task Force, including rheumatologists, translational immunologists, haematologists, paediatricians, patients and health professionals, based on a systemic literature review up to 15 December 2020. Overarching principles (OPs) and PtCs were formulated and consolidated by formal voting. RESULTS: Two OPs and fourteen PtCs were developed. OPs highlight the heterogeneous clinical spectrum of SARS-CoV-2 infection and the need of a multifaceted approach to target the different pathophysiological mechanisms. PtCs 1-6 encompass the pathophysiology of SARS-CoV-2 including immune response, endothelial dysfunction and biomarkers. PtCs 7-14 focus on the management of SARS-CoV-2 infection with immunomodulators. There was evidence supporting the use of glucocorticoids, especially dexamethasone, in COVID-19 cases requiring oxygen therapy. No other immunomodulator demonstrated efficacy on mortality to date, with however inconsistent results for tocilizumab. Immunomodulatory therapy was not associated with higher infection rates. CONCLUSIONS: Multifactorial pathophysiological mechanisms, including immune abnormalities, play a key role in COVID-19. The efficacy of glucocorticoids in cases requiring oxygen therapy suggests that immunomodulatory treatment might be effective in COVID-19 subsets. Involvement of rheumatologists, as systemic inflammatory diseases experts, should continue in ongoing clinical trials delineating optimal immunomodulatory therapy utilisation in COVID-19.

7.
Clin Exp Rheumatol ; 2021 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-33635212

RESUMO

OBJECTIVES: The main purpose was to investigate the intra- and inter-rater reliability of the Assessment of SpondyloArthritis international Society (ASAS) definition of positive MRI for active sacroiliitis (ASAS-positive MRI), in a sample of patients with inflammatory back pain (IBP) and suspected axial spondyloarthritis (axSpA), who underwent sacroiliac joints (SIJ) MRI. We also evaluated the intra- and inter-rater reliability for the detection of the recently ASAS-refined findings indicating inflammatory activity. METHODS: We retrospectively identified 105 consecutive patients with IBP and suspected axSpA who underwent SIJ MRI. Two radiologists in two distinct reading sessions assessed the prevalence of ASAS-positive MRI and of ASAS-defined signs of inflammatory activity. We determined the intra-rater and inter-rater reliability of the above-mentioned variables by means of prevalence-adjusted bias-adjusted kappa (PABAK) statistic, and verified whether there was any significant difference in providing the diagnosis of ASAS-positive MRI on an inter-rater basis (McNemar test). RESULTS: We observed substantial reliability in assessing a SIJ MRI as ASAS-positive both on intra-rater basis (PABAK ranging 0.70-0.77) and inter-rater basis (PABAK 0.71 for the first reading, and 0.64 for the second reading). No significant difference in the rate of diagnosis between raters was found (p>0.99 for both reading sets). Intra-rater and inter-rater reliability for inflammatory activity signs ranged from moderate to almost perfect. CONCLUSIONS: The substantial intra- and inter-rater reliability in assessing the ASAS-positive MRI supports its use for classification purposes. The variable reliability of inflammatory activity signs suggests they are suboptimal as a complement to the current definition of ASAS-positive MRI.

8.
Neuromuscul Disord ; 31(2): 134-138, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33461845

RESUMO

We describe the clinical response to long-term subcutaneous immunoglobulins (SCIg) in anti-3­hydroxy-3-methyl-glutaryl-coenzyme-A-reductase (anti-HMCGR) myopathy previously treated with intravenous immunoglobulins (IVIg). We collected data from patients affected by anti-HMGCR myopathy, switched from IVIg to SCIg therapy, after achieving clinical stabilization. The Medical Research Council sum score, creatine kinase (CK) levels, and anti-HMGCR antibodies were used to assess the response. We identified three patients with anti-HMGCR myopathy treated with SCIg with a favourable clinical course, allowing the maintenance of clinical stability, the reduction or suspension of steroids therapy and in two of them a complete CK normalization. Finally, anti-HMGCR antibodies tested in all patients after 12 months from SCIg starting, showed a global decrease. SCIg represent an useful alternative to long-term IVIg as already well known in several autoimmune neuromuscular disorders and inflammatory myopathies with advantages of lower side effects and home self-administration.

9.
Cytokine ; 140: 155438, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33493861

RESUMO

BACKGROUND: Patients infected by SARS-CoV-2 can develop interstitial pneumonia, requiring hospitalisation or mechanical ventilation. Increased levels of inflammatory biomarkers are associated with development of acute respiratory distress syndrome (ARDS). The aim of the present study was to determine which cytokines are associated with respiratory insufficiency in patients hospitalised for COVID-19. PATIENTS AND METHODS: Data on 67 consecutive patients were collected between March 8 and March 30, 2020. PaO2/FiO2 ratio (P/F) was calculated at hospital admission. The following cytokines were analysed: interleukin (IL)-6, IL-1α, IL-18, tumour necrosis factor (TNF)-ß, macrophage colony-stimulating factor (M-CSF), macrophage migration inhibitory factor (MIF), soluble IL-2 receptor alpha (sIL-2Rα; CD25), IL-12ß, IL-3, interferon (IFN) α2a, monokine induced by gamma interferon (MIG), monocyte-chemotactic protein 3 (MCP3) and hepatocyte growth factor (HGF). RESULTS: P/F lower than 300 was recorded in 22 out of 67 patients (32.8%). P/F strongly correlated with IL-6 (r = -0.62, P < 0.0001), M-CSF (r = -0.63, P < 0.0001), sIL-2Rα (r = -0.54, P < 0.0001), and HGF (r = -0.53, P < 0.0001). ROC curve analyses for IL-6 (AUC 0.83, 95% CI 0.73-0.93, P < 0.0001), M-CSF (AUC 0.87, 95% CI 0.79-0.96, P < 0.0001), HGF (AUC 0.81, 95% CI 0.70-0.93, P < 0.0001), and sIL-2Rα (AUC 0.80, 95% CI, 0.69-0.90, P < 0.0001) showed that these four soluble factors were highly significant. All four soluble factors correlated with LDH, white blood cell count, neutrophil count, lymphocyte count, and CRP. CONCLUSION: IL-6, M-CSF, sIL-2Rα, and HGF are possibly involved in the main biological processes of severe COVID-19, mirroring the level of systemic hyperinflammatory state, the level of lung inflammation, and the severity of organ damage.


Assuntos
/sangue , Citocinas/sangue , Imunidade Inata/imunologia , Inflamação/sangue , Subunidade alfa de Receptor de Interleucina-2/sangue , Insuficiência de Múltiplos Órgãos/sangue , Pneumonia/sangue , Idoso , /virologia , Feminino , Fator de Crescimento de Hepatócito/sangue , Interações Hospedeiro-Patógeno , Humanos , Inflamação/complicações , Interleucina-6/sangue , Fator Estimulador de Colônias de Macrófagos/sangue , Masculino , Pessoa de Meia-Idade , Insuficiência de Múltiplos Órgãos/complicações , Pneumonia/complicações , Pneumonia/virologia , Estudos Retrospectivos , /fisiologia
10.
Minerva Med ; 2020 Dec 02.
Artigo em Inglês | MEDLINE | ID: mdl-33263372

RESUMO

INTRODUCTION: Hepatitis C Virus (HCV) is a well-known worldwide infection, responsible for hepatic and extrahepatic complications. Among extrahepatic manifestation, the rheumatologic are the most common ones. With the arrival of Direct Antiviral Agents (DAA), the treatment and the clinical perspective have rapidly changed, permitting to achieve a sustained virological response (SVR) and preventing complications of chronic infection. EVIDENCE ACQUISITION: We performed on PubMed a literature search for the articles published by using the search terms "HCV infection", "HCV syndrome", "HCV-related rheumatologic disorders", "cryoglobulinaemia", "cryoglobulinaemic vasculitis", "mixed cryoglobulinaemia". EVIDENCE SYNTHESIS: Mixed cryoglobulinaemia (MC) is the prototype of HCVassociated rheumatologic disorder. HCV-related MC is typically considered by physicians as a human model disease to linking infection with autoimmune diseases. Chronic HCV infection can lead to a multistep process from a simple serological alteration (presence of circulating serum cryoglobulins) to frank systemic vasculitis (cryoglobulinaemic vasculitis (CV)) and ultimately to overt malignant B lymphoproliferation (such as non-Hodgkin lymphoma (NHL)). Antiviral therapy is indicated to eradicate the HCV infection and to prevent the complications of chronic infection. Immunosuppressive therapy is reserved in case of organ threatening manifestations of CV. In this review, we discuss the main clinical presentation, diagnostic approach and treatment of rheumatologic manifestations of HCV infection. CONCLUSIONS: Chronic HCV infection is responsible for complex clinical condition, ranging from hepatic to extra-hepatic disorders. Cryoglobulins are the result of this prolonged immune system stimulation, caused by tropism of HCV for B-lymphocyte.

11.
J Transl Autoimmun ; 3: 100072, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33305250

RESUMO

Objective: Glucocorticoids (GC) are associated with side effects in giant cell arteritis (GCA). Immunosuppressive therapies (ITs) have given conflicting results in GCA, regarding GC sparing effect. Primary endpoint is to evaluate whether very early introduction of ITs in GCA minimize the rate of GC-induced adverse events, in terms of infections, new onset systemic arterial hypertension, GC-induced diabetes and osteoporotic fractures. Methods: A multicenter retrospective case-control study included 165 patients. One group included 114 patients who were treated with at least one IT given at diagnosis or within 3 months from the start of GC. A second group included 51 GCA who received only GC or an IT more than 3 months later. Results: The most frequently used ITs were: methotrexate (138 patients), cyclophosphamide (48 patients) and tocilizumab (27 patients). No difference was observed as concerns the follow-up time between groups [48.5 (IQR 26-72) vs 40 (IQR 24-69), p â€‹= â€‹0.3)]. The first group showed a significantly lower incidence of steroid-induced diabetes (8/114, 7% vs 12/51, 23.5%; p â€‹= â€‹0.003) and no differences for the rate of infections (p â€‹= â€‹0.64). The group was also exposed to lower doses of GC at first (p â€‹< â€‹0.0001) and third (p â€‹< â€‹0.0001, rank-sum test) month. Forty-four patients in the first group (38.6%) compared with 34 in the second one (66.7%) experienced at least one relapse (p â€‹= â€‹0.001). Conclusion: Very early introduction of IT in GCA lowered the incidence of steroid-induced diabetes, possibly due to the lower doses of GC in the first three months. Relapse rate was even lower.

12.
Clin Exp Rheumatol ; 2020 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-33338001

RESUMO

OBJECTIVES: Baricitinib, an oral Janus kinase (JAK) 1-2 inhibitor, is currently used along biologic DMARDs (bDMARDs) after the failure of methotrexate (MTX) in rheumatoid arthritis (RA). We investigated the efficacy and safety of baricitinib in real life. METHODS: We prospectively enrolled 446 RA patients treated with baricitinib from 11 Italian centres. Patients were evaluated at baseline and after 3, 6, and 12 months. They were arrayed based on previous treatments as bDMARD-naïve and bDMARD-insufficient responders (IR) after the failure or intolerance to bDMARDs. A sub-analysis differentiated the effects of methotrexate (MTX) and the use of oral glucocorticoids (OGC). RESULTS: Our cohort included 150 (34%) bDMARD-naïve and 296 (66%) bDMARD-IR patients, with 217 (49%) using baricitinib as monotherapy. Considering DAS-28-CRP as the primary outcome, at 3 and 6 months, 114/314 (36%) and 149/289 (51.6%) patients achieved remission, while those in low disease activity (LDA) were 62/314 (20%) and 46/289 (15.9%), respectively; finally at 12 months 81/126 (64%) were in remission and 21/126 (17%) in LDA. At all-timepoints up to 12 months, bDMARDs-naïve patients demonstrated a better clinical response, independently of MTX. A significant reduction in the OGC dose was observed at 3 and 12 months in all groups. The serum positivity for both rheumatoid factors (RF) and anti-citrullinated protein antibodies (ACPA) conferred a lower risk of stopping baricitinib due to inefficacy. Fifty-eight (13%) patients discontinued baricitinib due to adverse events, including thrombotic events and herpes zoster reactivation. CONCLUSIONS: Real-life data confirm the efficacy and safety profiles of baricitinib in patients with RA and provide evidence that drug survival is higher in bDMARDs-naïve and seropositive patients.

13.
Clin Exp Rheumatol ; 38 Suppl 126(4): 53-56, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33095137

RESUMO

OBJECTIVES: Immunological parameters exert a relevant diagnostic and prognostic role in primary Sjögren's syndrome (pSS) and may identify specific disease phenotypes. Among disease-associated immunological features, anti-La/SSB are rarely found without concomitant anti-Ro/SSA and their clinical significance in patients with pSS has been poorly investigated. Thus, we aimed to characterise the value of anti-La/SSB analysing clinical and serologic features of a wide cohort of pSS patients with both circulating anti-Ro/SSA and positive salivary gland biopsy (SGB). METHODS: Clinical and serological data of 600 pSS patients with both anti-Ro/SSA and SGB positivity and categorised according to anti-La/SSB status were retrospectively analysed. Comparisons between patients with and without circulating anti-La/SSB were performed. RESULTS: Among the whole cohort, 319 (53%) of patients were anti-La/SSB negative and 281 (47%) were anti-La/SSB positive. Anti-La/SSB positive patients were younger at disease diagnosis and had a longer disease duration. Moreover, anti-La/SSB positive patients had a higher prevalence of hypergammaglobulinaemia and circulating rheumatoid factor and of lymphoproliferative disorders in comparison to seronegative group. At multivariate analysis, hypergammaglobulinaemia (OR=1,7; 95% CI 1.17, 2.43), rheumatoid factor (OR=2.3; 95% CI 1.6, 3.3) and lymphoma (OR=2.6; 95% CI 1.12, 5.96) were identified as independent variables significantly associated with anti-La/SSB positivity. CONCLUSIONS: In patients with pSS and concomitant anti-Ro/SSA and SGB positivity, the presence of anti-La/SSB may help in identifying a disease subset with distinct prognostic features, especially in terms of higher risk of lymphoproliferative complications.


Assuntos
Síndrome de Sjogren , Anticorpos Antinucleares , Biópsia , Humanos , Estudos Retrospectivos , Glândulas Salivares , Síndrome de Sjogren/diagnóstico
14.
Clin Exp Rheumatol ; 38 Suppl 126(4): 189-194, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33095153

RESUMO

OBJECTIVES: Thymic stromal lymphopoietin (TSLP) has been implicated in primary Sjögren's syndrome (pSS) and related B-cell lymphoproliferation and lymphoma (NHL) by studies on salivary pathologic tissues and serum. The purpose of this work was to validate serum TSLP as biomarker of pSS and related lymphoproliferation by the study of two additional independent cohorts. METHODS: Serum TSLP was measured by ELISA in the original published Cohort-1 from Udine, Italy, including 91 patients. Two additional cohorts were then studied for validation: Cohort-2, including 4 sub-cohorts comprising 125 patients from the Universities of Roma, L'Aquila, Pisa and Perugia, belonging to the Italian SS Study Group (GRISS), and Cohort-3, including 59 patients from the University of Athens, Greece. Overall, 159 control subjects were enrolled. Active pSS-NHL, as well as pre-lymphomatous conditions, i.e. persistent salivary gland swelling and mixed cryoglobulinaemia, were investigated in detail. In addition, serum samples from pSS-NHL in complete remission were analysed (n=27). RESULTS: TSLP serum levels were confirmed to be significantly higher in pSS compared to controls in both Cohort-2 and Cohort-3, in particular in patients with lymphoproliferation. Serum TSLP was much higher in pSS pre-lymphomatous conditions. Finally, active NHL showed the highest TSLP serum levels, while in NHL in remission TSLP resulted undetectable or significantly lower than in benign pSS. CONCLUSIONS: By the study of independent cohorts, it was again demonstrated that serum TSLP levels are increased in pSS, above all in more advanced B-cell lymphoproliferation and NHL. Serum TSLP can therefore represent a novel biomarker for pSS-related lymphoproliferation.


Assuntos
Síndrome de Sjogren , Biomarcadores , Citocinas , Grécia , Humanos , Itália , Síndrome de Sjogren/diagnóstico
15.
Artigo em Inglês | MEDLINE | ID: mdl-33083815

RESUMO

OBJECTIVES: To verify the level of adherence to the influenza vaccination program in a population of patients suffering from RA, PsA or AS undergoing immunosuppressive treatment. METHODS: Administrative databases from the Regional Health Information System of Friuli Venezia Giulia (FVG), Italy, were used. Subjects were residents in FVG, suffered from chronic inflammatory arthritis and had at least one prescription for a DMARD in the 9 months before the start of the vaccination season (from 1 October to 31 December). The observation ranged from 2006 to 2018. Logistic regression was used to assess the association between vaccination and the patient's characteristics in the 2018-2019 influenza season. RESULTS: Overall, vaccination adherence decreased from the highest value of 35.7% (662/1853) in 2006 to the lowest value of 25.3% (926/3663) in 2014; in people ≥65 years of age it also decreased over time from 61.6% (577/936) in 2008 to 43.9% (701/1595) in the 2014. By logistic analysis on the 2018-2019 season, which included 4460 patients, older subjects were more likely to be vaccinated [people 65-74 years, odds ratio (OR) 4.58 (95% CI 3.72, 5.64); people 75-84 years, OR 6.47 (95% CI 5.04, 8.32); both vs <65] as were those with diabetes [OR 1.66 (95% CI 1.05, 2.64)]. Treatment with a biologic agent alone [OR 0.64 (95% CI 0.52, 0.80)] and RA diagnosis [OR 0.69 (95% CI 0.51, 0.93)] were associated with lower adherence. CONCLUSION: Influenza vaccination adherence is alarmingly low in a population at higher risk of infectious complications, in particular in elderly patients.

16.
J Clin Med ; 9(11)2020 Oct 27.
Artigo em Inglês | MEDLINE | ID: mdl-33121112

RESUMO

Statin-associated autoimmune myopathy is a rare muscle disorder, characterized by autoantibodies against HMGCR. The anti-HMGCR myopathy persists after statin, and often requires immunosuppressive therapy. However, there is not a standardized therapeutic approach. The purpose of this study is to report the effectiveness of the immunosuppressive treatment employed in a multi-center and multi-disciplinary cohort of patients affected by anti-HMGCR myopathy, in which an immunoglobulin (IVIG)-based treatment strategy was applied. We collected 16 consecutive patients with a diagnosis of anti-HMGCR myopathy, between 2012 and 2019, and recorded data on clinical and laboratory presentation (i.e., muscle strength, serum CK levels, and anti-HMGCR antibody titer) and treatment strategies. Our results highlight the safety and efficacy of an induction therapy combining IVIG with GCs and/or methotrexate to achieve persistent remission of the disease and steroid-free maintenance. Under IVIG-based regimens, clinical improvement and CK normalization occurred in more than two thirds of patients by six months. Relapse rate was low (3/16) and 2/3 relapses occurred after treatment suspension. Nearly 90% of the patients who successfully discontinued GCs were treated with a triple immunosuppressive regimen. In conclusion, an IVIG-based regimen, which particularly includes high-dose immunoglobulin, GCs and methotrexate, can provide a fast remission achievement with GC saving.

18.
Clin Exp Rheumatol ; 2020 Sep 16.
Artigo em Inglês | MEDLINE | ID: mdl-32940209

RESUMO

OBJECTIVES: The AQUEOUS (Anti-phospholipid syndrome: a QUEstionnaire for yOUng patientS) study aimed to assess how the diagnosis of primary anti-phospholipid syndrome (PAPS) affects the psychosocial status of young patients. METHODS: Subjects with PAPS aged 18-45 years were invited to compile an ad hoc designed questionnaire and the Short Form-12 to assess quality of life (QoL). RESULTS: Ninety-two patients (83.7% females) were recruited in 10 Italian centres. Vascular and obstetric manifestations were equally represented. Nearly half of the patients perceived the need for psychological support, 89.2% when considering women after pregnancy complications. Social activities and working efficiency were reduced in APS patients, also intimacy was threatened. In all cases, fatigue appeared to be the main determinant. PAPS affected family planning, due to fears of treatment side-effects, disease hereditariness, inability to care for the newborn child. Fertility appeared to be conserved: the median time to pregnancy was 2 months; assisted reproduction techniques were pursued by 5 women. Our survey documented significantly lower rates of hospitalisation and learning disabilities in 51 children born after APS diagnosis as compared to 48 children born before. PAPS patients displayed lower QoL in physical and, to a greater extent, mental scores compared to the general Italian population. Both components were significantly lower in women and in patients with fatigue. CONCLUSIONS: The AQUEOUS study assessed for the first time the unmet needs of young PAPS patients, enabling the development of a future "youth-focused" strategy to reduce disease burden.

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