Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder that can impact any organ in the body. The pathophysiology of shrinking lung syndrome (SLS), a rare pulmonary complication of SLE, ...remains unknown. The objective of the current case series was to investigate the effects of inspiratory muscle training (IMT) on diaphragm thickness/mobility, respiratory muscle strength, peripheral muscle thickness/strength, and functional exercise capacity in patients with SLE and associated SLS. Three patients with SLE were included in the case series. Respiratory muscle strength, peripheral muscle strength, peripheral muscle thickness, diaphragm muscle thickness, diaphragm muscle mobility, functional exercise capacity, and pulmonary function test were assessed. A significant improvement has been determined in respiratory muscle strength, functional exercise capacity, peripheral muscle strength, peripheral muscle thickness, diaphragm muscle thickness, and diaphragm muscle mobility. This is the first case series showing the beneficial effects of IMT on respiratory muscle strength, diaphragm thickness/mobility, peripheral muscle thickness/strength, and exercise capacity in patients with SLE.
Full text
Available for:
DOBA, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
A 69-year-old woman with rheumatoid arthritis presented for the treatment of severe joint deformities. Examination of the hands, shown in a video, revealed telescoping fingers.
Up to 98% of rheumatoid arthritis (RA) patients experienced fatigue. It is an important physical and cognitive symptom which has overwhelming, uncontrollable, and unpredictable affects throughout ...their whole life. RA fatigue composes of complex and multi-dimensional components which are pain, stress, depression, inflammation, and disability. The acknowledgement of fatigue is important, and fatigue should be measured in all RA trials alongside the core set. The aim of this study was to determine reliability and validity of Turkish version of Bristol Rheumatoid Arthritis Fatigue Multi-Dimensional Questionnaire in RA patients. One hundred RA patients were evaluated in the study. Exclusion criteria were determined as patients with cognitive impairment, illiterate patients, unable to understand and speak Turkish, under the age of 18, and over the age of 75. To validate Turkish version of Bristol Rheumatoid Arthritis Fatigue Multi-Dimensional Questionnaire (BRAF-MDQ) (BRAF-MDQ-T), all participants answered BRAF-MDQ-T, Multidimensional Assessment of Fatigue (MAF) scale, and Short Form-36 vitality (SF-36 VT). BRAF-MDQ-T was applied again 7 days later for test–retest reliability. Validity, internal consistency, and test–retest results were based on a sample of 100 patients. Internal consistency reliability of BRAF-MDQ-T was Cronbach
α
= 0.95 which was excellent. The correlation between the total scores of the BRAF-MDQ-T scale and the total scores of MAF-T was statistically significant (
r
= 0.82,
p
< 0.001). The correlation between the total scores of the BRAF-MDQ-T scale and the subscale scores of SF–36 VT was statistically significant (
r
= − 0.64,
p
< 0.001). The BRAF-MDQ-T is a valid and reliable scale for the assessment of fatigue in Turkish rheumatoid arthritis patients.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
To evaluate the efficacy of on-demand use of anakinra in patients with crFMF. The Gazi FMF cohort was established in the year 2010, and from that date, 689 patients with FMF diagnosed according to ...the Tel Hashomer criteria were registered. Attack type, duration, severity, and their impact on life were collected either by disease diaries or a mobile phone application (FMF AIDD, AppStore, and Playstore). A retrospective cohort analysis was made from records of patients who have ever been treated with IL-1 inhibitors. A total of 78 patients were treated with IL-1 inhibitors in our cohort. Among these, 15 patients were identified who received on-demand anakinra. Rationale for on-demand use was prominent prodrome or trigger for attacks and patient’s personal claim. Six patients were switched from regular use and nine were directly started as on-demand use. All were using background colchicine in maximum tolerated doses. None of the patients had evidence of persistently elevated acute phase reactants or proteinuria. The median duration of on-demand anakinra use was 6 (3–36) months. Pre- and post- on-demand anakinra periods were compared. Patient reported attack severity (
p
= 0.002), duration (
p
= 0.001), frequency (
p
= 0.001), absenteeism (
p
= 0.002), and presenteeism (
p
= 0.002) were significantly improved. On-demand anakinra prevented progression of prodromes to full-blown attacks which was demonstrated by decrease in the rate of attack/prodrome ratio (
p
= 0.02). On-demand anakinra can be continued in ten subjects on long-term. On-demand anakinra significantly improved FMF attacks in certain patients which suggest this approach would be of benefit in daily practice in selected patients.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Background: Colchicine is the mainstay of the treatment of familial Mediterranean fever (FMF). However, 10% of FMF patients do not respond well to colchicine. Efficacy of interleukin (IL)-1 ...inhibitors in reducing attacks have been demonstrated in colchicine-resistant FMF (crFMF) patients recently. Colchicine is still the only approved drug for the prevention of amyloidosis in FMF and utility of IL-1 inhibitors in crFMF cases who already has amyloidosis remain to be elucidated. Herein, we evaluated efficacy and safety of IL-1 inhibitors in patients with crFMF-associated AA amyloidosis in a relatively large single center study.
Methods: Medical records of FMF patients complicated with AA amyloidosis in our dedicated FMF center were retrospectively reviewed and those patients who ever treated with IL-1 inhibitors were enrolled into the study. Patient global, physician global assessments (on 0-10 cm visual analog scale), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), serum creatinine and 24-h urinary protein excretion values for each visit were recruited from computer-based hospital records. Treatment response of patients were assessed with clinical symptoms, serum albumin, CRP and ESR values. Renal outcome parameters were analyzed on those not receiving renal replacement therapy.
Results: Seventeen patients were identified with crFMF-amyloidosis that ever treated with IL-1 inhibitors. Background colchicine therapy was continued in all patients in maximal-tolerated dose along with IL-1 inhibitors. All patients benefit from IL-1 antagonists assessed by patient and physician global assessments. Inflammatory markers, CRP and ESR, were significantly reduced in all and normalized in 12 out of 17 patients. More importantly, the amount of proteinuria was remarkably improved following IL-1 inhibitor therapy (1606 mg/day to 519 mg/day, p = .008). Both anakinra and canakinumab were well-tolerated without severe side effects. All patients were initially treated with anakinra but switched to canakinumab in seven patients (one leukopenia, four injection site reaction, two inefficacy).
Conclusion: We evaluated the clinical and laboratory responses to IL-1 inhibitors in crFMF-associated amyloidosis patients. We found significant decreases in CRP, ESR and proteinuria after IL-1 inhibitor therapy. This study confirmed that IL-1 inhibitors are effective for controlling attacks and inflammatory activity in FMF patients complicated with AA amyloidosis. Moreover, they reduce or stabilize amount of proteinuria and preserve renal function in short-term follow-up. Prolonged prospective clinical trials are warranted to assess their long-term efficacy in this particular patient group.
The Cochin 17-item Scleroderma Functional (CSF-17) Scale is a patient-reported outcome measure evaluating activities and participation in patients with systemic sclerosis (SSc).
The aim of the ...present study was to translate and cross-culturally adapt the CSF-17 into the Turkish language and investigate its convergent validity and reliability in Turkish-speaking patients with SSc.
The CSF-17 was cross-culturally adapted according to Beaton's guideline. Participants completed CSF-17 Scale, Scleroderma Health Assessment Questionnaire (SHAQ), Short Form-12 (SF-12) Health Survey and Hospital Anxiety and Depression Scale (HADS). Internal consistency and test-retest reliability were determined interpreting Cronbach's alpha and Intraclass Correlation Coefficient (ICC) values, respectively. Convergent validity was tested using Pearson's correlation coefficient.
Fifty-six patients with SSc were enrolled in the study. Cronbach's alpha and ICC values of the CSF-17 total score were found to be as 0.963 and 0.958, respectively, indicating excellent reliability. As for the convergent validity, it was determined that CSF-17 total score has a good correlation with SHAQ. Correlations of subscales of CSF-17 with subscales of SF-12 and HADS ranged from poor to moderate.
Turkish version of CSF-17 met the set criteria of reliability and convergent validity. According to the results of the analysis, it was concluded that the Turkish version of the CSF-17 is a reliable and valid tool for Turkish-speaking SSc patients.
Full text
Available for:
DOBA, IJS, IZUM, KILJ, NUK, OILJ, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK, VSZLJ
Familial Mediterranean fever (FMF) is characterized by recurrent short-lived/self-limiting inflammatory attacks. Besides these, a substantial number of patients with FMF present with a variety of ...other inflammatory diseases; however, this issue has not been systematically studied previously. Hence, we aimed to investigate the frequency of inflammatory comorbid diseases in a large FMF cohort. All patients were recruited from “FMF in Central Anatolia (FiCA) Cohort”, comprising 971 (mean age 35.3 ± 12 years, 61.5% female) adult subjects. All patients fulfilled Tel Hashomer criteria. Demographic data, FMF disease characteristics, MEFV gene mutations, and comorbid inflammatory diseases were meticulously questioned, and laboratory features and genotype data were retrieved from hospital records. There were comorbid inflammatory diseases in 205 (21.1%) patients. The most common inflammatory disease was spondyloarthritis (12.9%). Other remarkable inflammatory disorders were psoriasis, immunoglobulin A vasculitis/Henoch–Schönlein purpura, Behçet’s disease and inflammatory bowel diseases. Cryptogenic organizing pneumonia is a newly defined entity in our cohort which is seemed to be associated with FMF (0.3%). Number of patients with persistent inflammation was higher in those with comorbid diseases (
p
< 0.001). Our results suggest that FMF is commonly associated with other inflammatory diseases. Therefore, clinicians should be cautious about comorbid inflammatory diseases in FMF patients, particularly in those with persistent inflammation. Identification of pathogenic pathways linking FMF to these diseases warrants further investigations.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Familial Mediterranean fever is characterized by self-limited attacks of serositis and arthritis. However, substantial number of patients suffer from chronic complications of this disease, primarily ...involving musculoskeletal system. Treatment for these complications is challenging due to limited evidence. Interleukin-1 (IL-1) antagonists, tocilizumab and anti-tumor necrosis factor (anti-TNF) agents are off-label treatment options for the management of chronic manifestations of FMF, such as secondary (AA) amyloidosis, chronic arthritis and sacroiliitis. This paper presents a case series of four FMF patients who are refractory to IL-1 antagonists, anti-TNF agents and tocilizumab, who responded well to tofacitinib. The authors also conducted a comprehensive literature search for studies investigating tofacitinib use in FMF patients. Although still limited, current data suggest that tofacitinib could be a useful treatment option for FMF patients with associated inflammatory comorbid conditions and chronic manifestations of disease.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Objective
The objective of this study was to assess the impact of Familial Mediterranean Fever (FMF) attacks on daily physical activity and detect FMF attacks using a connected activity tracker.
...Methods
Patients with FMF according to the Tel-Hashomer criteria were included in this prospective observational study. Attack-related data were collected weekly via phone call to avoid memory bias. Median steps in attack and attack-free days were calculated and compared using the Wilcoxon rank test. Sensitivity and specificity threshold for capturing attacks was set to two thirds of median steps per day in the whole observation period.
Results
Twelve patients participated in the study. The median age of patients was 26 (18–32) years, and 7 (58.3%) of them were male. Patients with attacks (
n
= 10) walked a median of 6990 (4552–11,531) steps per day in attack-free days, whereas this number decreased to a median of 1841 (590–4783) steps in attack days (
p
= 0.005). The activity tracker captured 42 of 45 attack days and 312 of 361 attack-free days. The cutoff value had 93% sensitivity and 86% specificity for capturing attacks.
Conclusions
FMF attacks significantly impair the physical activity of patients. Activity tracking may be a reasonable method to document FMF attacks. This might prevent errors due to memory bias and help accurately identify and treat patients with FMF.
Full text
Available for:
EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OBVAL, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
PDF
