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1.

Objective

Polymyalgia rheumatica (PMR) is often the presenting manifestation of giant cell arteritis (GCA). Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) scan often discloses the presence of large vessel vasculitis (LVV) in PMR patients. We aimed to identify predictive factors of a positive PET/CT scan for LVV in patients classified as having isolated PMR according to well-established criteria.

Methods

A set of consecutive patients with PMR from a single hospital were assessed. All of them underwent PET/CT scan between January 2010 and February 2018 based on clinical considerations. Patients with PMR associated to other diseases, including those with cranial features of GCA, were excluded. The remaining patients were categorized in classic PMR (if fulfilled the 2012 EULAR/ACR classification criteria at disease diagnosis; n = 84) or atypical PMR (who did not fulfill these criteria; n = 16). Only information on patients with classic PMR was assessed.

Results

The mean age of the 84 patients (51 women) with classic PMR was 71.4 ± 9.2 years. A PET/CT scan was positive in 51 (60.7%). Persistence of classic PMR symptoms was the most common reason to perform a PET/CT scan. Nevertheless, patients with positive PET/CT scan often had unusual symptoms. The best set of predictors of a positive PET/CT scan were bilateral diffuse lower limb pain (OR = 8.8, 95% CI: 1.7–46.3; p = 0.01), pelvic girdle pain (OR = 4.9, 95% CI: 1.50–16.53; p = 0.01) and inflammatory low back pain (OR = 4.7, 95% CI: 1.03–21.5; p = 0.04).

Conclusion

Inflammatory low back pain, pelvic girdle and diffuse lower limb pain are predictors of positive PET/CT scan for LVV in PMR.  相似文献   

2.

Objective

MRI-detected inflammation is considered of diagnostic value for rheumatoid arthritis (RA), but its evaluation involves a time-consuming scoring of 61 joint-level features. It is not clear, however, which of these features are specific for RA and whether evaluating a subset of specific features is sufficient to differentiate RA patients. This study aimed to identify a subset of RA-specific features in a case–control setting and validate them in a longitudinal cohort of arthralgia patients.

Methods

The difference in frequency of MRI-detected inflammation (bone marrow edema, synovitis, and tenosynovitis) between 199 RA patients and 193 controls was studied in 61 features across the wrist, metacarpophalangeal, and metatarsophalangeal joints. A subset of RA-specific features was obtained by applying a cutoff on the frequency difference while maximizing discriminative performance. For validation, this subset was used to predict arthritis development in 225 clinically suspect arthralgia (CSA) patients. Diagnostic performance was compared to a reference method that uses the complete set of 61 features normalized for inflammation levels in age-matched controls.

Results

Subset of 30 features, mainly (teno)synovitis, was obtained from the case–control setting. Validation in CSA patients yielded an area of 0.69 (95% CI: 0.59–0.78) under the ROC curve and a positive predictive value (PPV) of 31%, compared to 0.68 (95% CI: 0.60–0.77) and 29% PPV of the reference method with 61 features.

Conclusion

Subset of 30 MRI-detected inflammatory features, dominated by (teno)synovitis, offers a considerable reduction of scoring efforts without compromising accuracy for prediction of arthritis development in CSA patients.  相似文献   

3.

Objective

Gout is the osteoarticular expression of hyperuricemia, resulting from excessive production and/or insufficient elimination of uric acid. Emerging case reports described the deposition of mono sodium urate in the spine as a rare manifestation of gout, we aimed in revealing the full picture of reported axial joint gout (AJG).

Methods

We performed a systemic patient level review focused on characteristics of reported cases of axial joint involvement in gout.

Results

A total of 127 studies (142 cases) were identified as axial joint gout. Most of the cases were reported by neurosurgeons and orthopedic surgeons (19.7% and 17.6%, respectively),low back and neck pain and weakness of limbs were presented in 113 cases, most of the cases (77.5%) were diagnosed via operation or aspiration. Although CT and MRI was the most popular imaging method, 8 cases underwent DECT avoided surgery had marked improvement.

Conclusions

The incidence of AJG was underestimated and the IAJG exist independent of peripheral arthritis. AJG should be suspected when back pain and neurological involvement occurred in the risky populations. DECT would be a promising technique to initiate the earlier intervention complimentary to invasive procedures or operations.  相似文献   

4.

Background

There are several medications available to treat pulmonary arterial hypertension (PAH): PAH-targeted drugs. However, in patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis (PVOD/PCH), rare diseases that cause pulmonary hypertension, the effectiveness and safety of vasodilators, including PAH-targeted drugs, are unclear.

Methods

We searched English-language publications listed in three electronic databases (PubMed, Cochrane Library, and the Japan Medical Abstracts Society). Reports with efficacy outcomes (survival, improvement in 6-minute walk distance, and pulmonary vascular resistance) and data on development of pulmonary edema after administration of vasodilators to patients with PVOD/PCH were selected (1966 to August 2015).

Results

We identified 20 reports that met our criteria. No randomized controlled or prospective controlled studies were reported. The survival time ranged from 71 minutes to 4 years or more after initiation of vasodilators. Most of the reported cases showed an improvement in the 6-minute walk distance and pulmonary vascular resistance. Pulmonary edema was reported in 15 articles, some cases of which were lethal.

Conclusions

The present study demonstrates the potential efficacy and difficulties in the use of vasodilators in patients with PVOD/PCH; however, drawing a firm conclusion was difficult because of the lack of randomized controlled trials. Further research is needed to ascertain if vasodilator use is beneficial and safe in patients with PVOD/PCH.  相似文献   

5.

Background

As lung cancer development in patients with nontuberculous mycobacterial lung disease (NTM-LD) has never been reported, we investigated its incidence and clinical characteristics.

Methods

Prospective observational cohort registry (from June 2012 to June 2017), and retrospective identification by the International Classification of Diseases, tenth revision (between March 2010 and March 2018), were used to identify NTM-LD patients aged ≥20 years who developed lung cancer.

Results

Eight patients (two men and six women, one with smoking history), having Mycobacterium avium complex lung disease (MAC-LD) were identified. Four were identified from retrospective chart reviews and four from the prospective observational cohort registry (n = 361, 289 women; 311 never-smokers). All patients underwent chest computed tomography (CT) at least once a year. The incidence rate of lung cancer developing in NTM-LD patients was 124.6 per 100,000 patient-years, which was higher than the lung cancer rate in Japan. The mean age at diagnosis of MAC-LD and lung cancer was 63.6 and 74.4 years, respectively. The most common lung cancer types were adenocarcinoma (six patients) followed by squamous cell carcinoma (two patients). Lung cancer was diagnosed at early and advanced clinical stages in seven and one patients, respectively. Outcomes were favorable, except in two patients: one with advanced stage disease, and another with poor performance status.

Conclusions

We identified the clinical characteristics of eight MAC-LD patients who developed lung cancer. NTM-LD may be a risk factor for lung cancer development. Periodic follow-up with chest CT might contribute to early diagnosis and curative therapy for lung cancer.  相似文献   

6.

Background

Psoriatic arthritis (PsA) is a chronic and seronegative inflammatory arthritis occurring in patients with psoriasis. The current knowledge about the risk of malignancy associated with psoriatic arthritis (PsA) patients undergoing therapy is controversial. We focused on the relationship between malignancy and therapy and undertook a meta-analysis to address this issue.

Methods

A systematic literature search of the PubMed, EMBASE, and Web of Science databases was performed to identify relevant studies and trials. Statistical analysis was conducted using STATA 11.2 software.

Results

Nine cohort studies were included, corresponding to a total of 43,115 PsA patients undergoing therapy. A significant positive association between therapy and increased risk for overall malignancy was found relative to the general population as the reference group (pooled RR, 1.29; 95% CI: 1.04–1.60). High heterogeneity was found (I2 = 71.37%). Subgroup analysis reported that PsA patients treated with conventional synthetic disease modifying antirheumatic drugs (csDMARDs) presented increased cancer risk (pooled RR, 1.75; 95% CI: 1.40–2.18) but patients treated with biological disease modifying antirheumatic drugs (bDMARDs) did not (pooled RR, 0.957; 95% CI: 0.80–1.14). Compared to controls, patients with PsA undergoing treatment specifically are at increased risk for non-melanoma skin cancers (pooled RR, 2.46; 95% CI: 1.84–3.28).

Conclusions

This study allowed the estimation of cancer risk in PsA patients during therapy. Large-scale longitudinal studies will be essential to draw firm conclusions regarding PsA-associated risk for treatment-induced malignancy.  相似文献   

7.

Background

Elderly patients who are hospitalized due to pneumonia experience deterioration of their activities of daily living (ADL) during this period; in some cases, this loss of ADL is not recovered at the end of antibiotic treatment. In this study, we examined whether erector spinae muscle cross-sectional area (ESMCSA) measured by computed tomography (CT) could predict a low level of ADL at the end of antibiotic treatment for pneumonia.

Methods

Eighty patients (mean age 74.8 years) with pneumonia, who were admitted to Yamagata university hospital between 2015 and 2016, were analyzed retrospectively. In all cases, chest CT was performed on admission and ESMCSA was measured at the level of the 12th thoracic vertebra. Patient levels of ADL were also measured, both on admission and at the end of treatment, using the Barthel Index.

Results

Patients with lower levels of ADL at the end of treatment were significantly older and tended to have a lower body mass index, poorer nutritional status, and more severe pneumonia than did patients who were self-reliant. Significantly smaller ESMCSAs were noted in patients who required assistance at the end of treatment than in those who were self-reliant. In multivariate logistic regression analysis, smaller ESMCSA was significantly associated with a lower level of ADL at the end of treatment, independent of age, sex, severity of pneumonia, nutritional status, or dehydration status.

Conclusion

These results suggest that ESMCSA can predict ADL level after antibiotic treatment of pneumonia.  相似文献   

8.

Background

The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment.

Methods

We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date.

Results

We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%).

Conclusions

In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.  相似文献   

9.

Background

The long-term management of perianal Crohn’s disease for patients on anti-TNF-α therapy remains challenging.

Aim

To evaluate the long-term course and complications of patients with perianal fistulas treated with anti-TNF-α based on their clinical remission and healing on MRI.

Methods

Patients were evaluated clinically and by MRI. Deep remission was defined as clinical remission associated with the absence of contrast enhancement and T2 hyperintensity on MRI. Flare-free survival, surgery and hospitalizations were compared based on the presence or not of deep remission.

Results

Forty-eight consecutive patients were included with a median follow-up of 62?months after anti-TNF-α first administration. Deep remission was observed in 16 patients (33.4%). For patients in deep remission, the median time to any perianal event was 116?months (95–130) versus 42?months (8–72) in patients with pathological MRI (p?<?0.001). Sixteen patients (50%) with pathological MRI had perianal surgery versus 2 (12.5%) in the deep remission group (p?<?0.05). The mean duration of cumulative hospital stays was 0.75?±?0.52?days in the deep remission group versus 19.7?±?7.4 in the pathological group (p?<?0.05).

Conclusions

Higher flare-free survival and lower rates of surgery and hospitalization were found in patients achieving deep remission.  相似文献   

10.

Background

This study aimed to determine the radiologic predictors and clarify the clinical features related to survival in patients with combined pulmonary fibrosis and emphysema (CPFE) and lung cancer.

Methods

We retrospectively reviewed the medical chart data and high-resolution computed tomography (HRCT) findings for 81 consecutive patients with CPFE and 92 primary lung cancers (70 men, 11 women; mean age, 70.9 years). We selected 8 axial HRCT images per patient, and visually determined the normal lung, modified Goddard, and fibrosis scores. Multivariate analysis was performed using the Cox proportional hazards regression model.

Results

The major clinical features were a high smoking index of 54.8 pack-years and idiopathic pulmonary fibrosis (n = 44). The major lung cancer profile was a peripherally located squamous cell carcinoma (n = 40) or adenocarcinoma (n = 31) adjacent to emphysema in the upper/middle lobe (n = 27) or fibrosis in the lower lobe (n = 26). The median total normal lung, modified Goddard, and fibrosis scores were 10, 8, and 8, respectively. TNM Classification of malignant tumors (TNM) stage I, II, III, and IV was noted in 37, 7, 26, and 22 patients, respectively. Acute exacerbation occurred in 20 patients. Multivariate analysis showed that a higher normal lung score and TNM stage were independent radiologic and clinical predictors of poor survival at the time of diagnosis of lung cancer.

Conclusions

A markedly reduced area of normal lung on HRCT was a relevant radiologic predictor of survival.  相似文献   

11.

Background

The purpose of this study was to evaluate the diagnostic accuracy of Cryo with endobronchial ultrasonography using a guide sheath (EBUS-GS) for peripheral pulmonary lesions (PPLs) to assess the volume of specimen, determine DNA sequencing analysis, and evaluate the utility of rapid on-site evaluation (ROSE).

Methods

Out of 30 patients assessed for eligibility, 23 were enrolled in this prospective study. The histological diagnostic yield of Cryo was evaluated and the volume was compared to that of trans-bronchial biopsy (TBB). DNA analysis of Cryo was performed using next generation sequencing (NGS). ROSE was compared with the final diagnosis.

Results

The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy rate was 85%, 100%, 100%, 50%, 87% for Cryo and 80%, 100%, 100%, 42.9%, 82.6% for TBB, respectively. The mean volume was 0.078?cm3 for Cryo and 0.003?cm3 for TBB (p < 0.0001). All Cryo specimens provided sufficient quantity and quality of DNA for analysis by NGS. ROSE had a high sensitivity (70%), specificity (100%), PPV (100%), and diagnostic accuracy (73.9%). There were no clinically serious adverse events except mild bleeding in 4 cases.

Conclusions

Cryo with EBUS-GS for PPLs is a safe and potentially useful diagnostic strategy. It has a high diagnostic yield, and provides significantly larger specimens than TBB. It also provides high quantity and quality of DNA for NGS and high concordance rate between ROSE and the final diagnosis.  相似文献   

12.

Background

Idiopathic pulmonary fibrosis (IPF) is a chronically progressive lung disease with exceptionally poor prognosis. While lung transplantation (LTx) is considered the last-resort therapeutic option, dismal waitlist mortality still hampers the salvage of patients with IPF. Pirfenidone, originally designed for IPF treatment, has increasingly been utilized. This study aimed to evaluate whether Pirfenidone could influence outcomes of patients with IPF on the Japanese LTx waitlist.

Methods

This retrospective single-center cohort study included 25 consecutive patients with IPF who were registered as LTx candidates at our institution between July 1999 and August 2016. Patients with a history of pretransplant Pirfenidone therapy (Pirfenidone group) were compared with those with no history (non-Pirfenidone group).

Results

In total, 6 (24%) patients received Pirfenidone as pretransplant therapy for 45.2 (range, 18.6–66.8) months. During the treatment period, the Pirfenidone group achieved a significant reduction in the decline rate of the forced vital capacity (-6.2% vs. -0.3%, p = 0.04) and a lower lung allocation score (31 vs. 41, p = 0.013) compared with the non-Pirfenidone group. The Pirfenidone group exhibited 100% waitlist survival three years after registration that was comparable to other indications, and 66% of the patients were still alive at the time of organ availability. No patient in the Pirfenidone group developed Pirfenidone-related surgical complications postoperatively.

Conclusions

Patients with IPF successfully managed with long-term Pirfenidone therapy achieved favorable outcomes after LTx registration, comparable to other patients with LTx indications. The tolerability to antifibrotic therapy can be a predictor of waitlist survival.  相似文献   

13.

Background

There have been no reports on the relationship between lung radiological patterns and rheumatoid arthritis (RA) disease activity or RA treatment response in patients with RA-associated lung disease (RA-LD).

Methods

Patients with RA-LD who underwent treatment for RA from April 2005 to March 2015 were retrospectively evaluated. RA-LD patients were divided into three groups based on high-resolution computed tomography (HRCT) patterns [usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and bronchiolitis]. The disease activity score of 28 joints-erythrocyte sedimentation rate (DAS28-ESR) and the response of RA to treatment, as measured by the European League Against Rheumatism (EULAR) response criteria, were investigated.

Results

A total of 77 patients (21 with UIP, 23 with NSIP, and 33 with bronchiolitis) were enrolled. Median scores (interquartile range) on the DAS28-ESR at baseline were 5.27 (4.76–5.74), 5.48 (4.24–6.34), and 5.04 (3.90–5.66) for UIP, NSIP, and bronchiolitis, respectively; there were no statistical differences between the three groups (p = 0.412). One year after baseline, 19 (90%), 14 (61%), and 19 (58%) of patients in the UIP, NSIP, and bronchiolitis groups, respectively, were considered good or moderate responders, as evaluated using the EULAR response criteria; there was a significant difference between these three groups (p = 0.014). Multiple logistic regression analysis revealed that the UIP pattern was significantly associated with good or moderate response to RA treatment 1 year after baseline (p = 0.012).

Conclusions

These results suggest that NSIP and bronchiolitis HRCT patterns may be risk factors for resistance to RA therapy.  相似文献   

14.

Objectives

The association of myasthenia gravis (MG) and inflammatory myositis (IM) is rare and typically only one of the diseases is present. The management of the 2 diseases differs, therefore it is important to recognize the concomitant presentation. Here, we report a case series of 7 patients with co-existing MG and IM with review of the literature.

Method

We identified 7 patients with concurrent MG and IM who were followed at the Neuromuscular Disease Program at a tertiary referral center in Vancouver, British Columbia from 2004 to 2017.

Result

All 7 patients had ocular or bulbar involvement as manifestation of MG. Three patients had simultaneous onset of MG and IM, 2 of whom presented with myasthenia crisis and fulminant myositis. In the other 4 patients, MG was the initial presentation and IM occurred 3–11 years after MG. Among these 7 patients, 4 had underlying thymic pathology, including 2 with benign thymoma and 2 with stage IV thymoma; all 4 patients had antibodies to acetylcholine receptor (AChR). Of the 3 patients with no thymic pathology by imaging or histology, 2 had positive AChR antibody titer. For treatment, the thymoma was resected and chemotherapy was administered if appropriate. Additional immunosuppressive therapies including high-dose glucocorticoid, intravenous immunoglobulin (IVIG), methotrexate, mycophenolate, or cyclosporine were necessary to achieve remission. Two patients with no thymoma had refractory MG and IM, and both responded to rituximab. We also conducted a literature review on the clinical characteristics and management of this condition, and compared the previously reported cases to the patients in our series.

Conclusion

This is one of the largest case series of MG–IM overlap with or without thymic pathology. In this cohort, the 2 disease entities can occur simultaneously, or one presents before the other. Most of the patients responded well to steroid, acetylcholinesterase inhibitor, and immunosuppressive agents. In very refractory cases, rituximab appeared to be effective, which has not been reported for the treatment of this condition before.  相似文献   

15.

Background

To estimate patient acceptable symptom state (PASS) and minimal clinically important difference (MCID) for patient-reported outcomes in systemic sclerosis (SSc).

Methods

We conducted a secondary analysis of the SCLEREDUC trial, a 12-month randomized controlled trial comparing the efficacy of physical therapy to usual care in 220 SSc patients followed-up from September 2005 to October 2010. Self-rated state and change in patient health at 12 months were assessed by using 2 external anchors extracted from the Medical Outcomes Study 36-Item Short-Form. Patients who self-rated their health as “excellent”, “very good” or “good” were the PASS group and those who self-rated their health change as “somewhat better” were the MCID group. Main outcomes were the estimates of PASS by using the 75th percentile method and of MCID by using the mean change in scores method for pain and activity limitation.

Results

PASS (95% confidence interval) and mean (SD) MCID estimates at 12 months were 53.75 (34.00 to 68.00) and ?6.74 (32.02) for the joint-pain visual analog scale (range 0–100), 1.41 (1.13 to 1.63) and ?0.21 (0.48) for the Health Assessment Questionnaire (HAQ, range 0–3), 1.27 (1.07 to 1.62) and ?0.13 (0.45) for the scleroderma HAQ (range 0–3), 26.00 (17.00 to 37.00) and -3.38 (9.87) for the Cochin Hand Function Scale (range 0–90), and 19.40 (17.20 to 21.90) and ?5.69 (6.79) for the McMaster-Toronto Arthritis Patient Preference Disability Questionnaire (range 0–30), respectively.

Conclusions

We provide, for the first time, the PASS and MCID estimates for pain and activity limitation in SSc.

Trial registration

ClinicalTrials.gov Identifier: NCT00318188. First Posted: April 26, 2006.  相似文献   

16.

Objectives

The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet’s syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.

Methods

A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.

Results

Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study.

Conclusions

RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results.  相似文献   

17.

Background

Cigarette smoking in patients with asthma leads to poor symptom control. As patients who are current smokers have been excluded from enrollment in many clinical trials on asthma, there are few reports on the treatment in current smokers with asthma. In this study, we aimed to assess how respiratory physicians manage asthma in current smokers in Japan.

Methods

Respiratory physicians in 16 Japanese hospitals answered a questionnaire on treatment for patients with asthma between December 2014 and February 2015. Medical records were reviewed for 1756 patients with asthma.

Results

The mean patient age was 61.1 years, and 62.9% of the patients were female. A total of 102 patients (5.8%) were current smokers, and 546 patients (31.1%) were former smokers. Long-acting muscarinic antagonists (LAMA) were prescribed more frequently for current smokers with asthma than for former smokers and never smokers with asthma (10.8% vs 4.6%, p = 0.01, 10.8% vs 3.8%, p < 0.01). In contrast, macrolides were prescribed more frequently for former smokers and never smokers with asthma than for current smokers with asthma (7.7% vs 1.0%, p = 0.01, 6.4% vs 1.0%, p = 0.03). Triple therapy, i.e., inhaled corticosteroids, long-acting beta agonists, and LAMA concomitantly, was prescribed for current smokers with asthma more frequently than for former smokers and never smokers with asthma (9.8% vs 4.0%, p = 0.01, 9.8% vs 3.3%, p < 0.01).

Conclusions

According to this survey, current smokers with asthma received more intensive therapy, including LAMA, than did former smokers with asthma.  相似文献   

18.

Objectives

To investigate the long-term effect of B-cell depletion therapy with Rituximab (RTX) alone as rescue therapy in primary antiphospholipid syndrome (PAPS) patients with severe thrombocytopenia.

Methods

We retrospectively retrieved data from patients who met the following inclusion criteria: (a) persistent antiphospholipid antibodies (aPL) positivity and fulfilled the Sydney criteria for PAPS (b) presented with severe thrombocytopenia (platelets <50,000/mm3) (c) were treated with RTX as a rescue therapy (d) had at least 1 year of follow-up after B-cells depletion therapy.

Results

This retrospective study included 6 consecutive female PAPS patients [median age 49.5 (range 38–66)] who presented with severe thrombocytopenia (platelets <50,000/mm3, mean value 31,000 ± 9000/mm3). We observed a full response (defined as >150,000 platelets/mm3) after treatment with RTX in 5 out of 6 patients (83.3%). Among responders, after a median follow-up of more than 4 years, we observed a median time free from relapse of 43 months (range 12–97). One patient did not respond to the B-cell depletion therapy and was treated with a splenectomy 1 month after RTX therapy and platelets levels normalized after 3 months. No adverse events were reported, no patients developed significant infections. Importantly, the patients required no further maintenance therapy for the thrombocytopenia.

Conclusion

In one of the longest-term observational (median 43 months) studies, sustained clinical remission of severe thrombocytopenia without immunosuppressive maintenance therapy was obtained by RTX alone in patients with PAPS and severe thrombocytopenia intolerant or refractory to conventional therapy.  相似文献   

19.
20.

Background

Spondyloarthritis (SpA), a chronic inflammatory, rheumatic disease, and hidradenitis suppurativa (HS), a chronic, debilitating, inflammatory skin disease, share several clinical and pathophysiological features, such as the association with inflammatory bowel disease and elevated cytokine levels IL-17 and TNF-α. Recently, SpA was reported to be more prevalent (2.3–28.2%) in patients with HS than in the general population. Conversely, the prevalence of HS in SpA is not exactly known.

Objective

To determine the prevalence of HS in patients with axial SpA, a subtype of SpA primarily of the axial skeleton. Secondly, to identify patient characteristics associated with the presence of HS in axial SpA.

Methods

In this cross-sectional study, a self-screening questionnaire based on validated diagnostic HS questions was sent to all participating axial SpA patients from the Groningen Leeuwarden Axial Spondyloarthritis (GLAS) cohort fulfilling the ASAS axial SpA criteria. Self-reported HS symptoms were confirmed by previous medical diagnosis or verification by phone using highly specific validated questions.

Results

In total, 75.6% (449/592) questionnaires were eligible for analyses. Included patients had a mean age of 50 ± 13 years, 64% was male, mean symptom duration was 23 ± 13 years, and 79% was HLA-B27 positive. HS diagnosis could be confirmed in 41 patients, resulting in an estimated prevalence of 9.1%. In comparison to patients without a positive history of HS, these patients were more often female (54% vs. 35%, P = 0.02), showed higher axial SpA disease activity (mean BASDAI 4.5 vs. 3.6, p = 0.01 and ASDASCRP 2.6 vs. 2.2, P = 0.003) and worse quality of life (QoL) (median ASQoL 9.0 vs. 4.0, P < 0.001). Also, a history of heel enthesitis and dactylitis was more prevalent (34% vs. 19%, P = 0.03 and 15% vs. 6%, P = 0.05, respectively). Multivariable analysis showed that a higher score on ASDAS was independently associated with HS (OR: 1.639, 95% CI: 1.176–2.284).

Conclusion

In our cohort of axial SpA patients, HS is more prevalent than in the general population (9.1% versus 0.053–4.1%). HS is associated with female gender, lower QoL, and especially higher axial SpA disease activity.  相似文献   

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