Sleep quality and its associations with disease activity and quality of life in older patients with rheumatoid arthritis.

PURPOSE: Sleep disturbance is among the most important geriatric syndromes, and its evaluation is part of the routine comprehensive geriatric assessment (CGA). Previous studies have demonstrated that older patients with rheumatoid arthritis (RA) have poorer sleep quality than younger control patients. However, there needs to be more data on the sleep quality of older patients with RA with age-matched controls. METHODS: Totally 100 participants, 50 older RA patients classified according to the ACR criteria, and 50 age- and gender-matched control patients without RA were included in the study. All patients underwent CGA, including assessing their functionality, depressive, cognitive, and nutritional status. In addition, sleep quality was assessed by the Pittsburg Sleep Quality Index (PSQI), and RA disease activity by a rheumatologist using the Disease Activity Score 28 (DAS28-CRP), and quality of life with the RA QoL questionnaire (RAQoL). RESULTS: The median age was 70 years (min-max: 65-86), and 62.5% were female. Co-morbidities and comprehensive geriatric assessment parameters were similar between the two groups. Median PSQI global score was higher in patients with RA than controls [9 (min-max: 1-20) vs. 5 (min-max: 1-13), p = 0.029). When the patients were categorized with respect to being 'poor sleepers' (PSQI score > 5), 62% of patients with RA and 38% of controls were poor sleepers (p = 0.016). The patients classified as poor sleepers were more likely to have a diagnosis of RA, higher DAS28-CRP and RAQoL scores, lower grip strength, and be a woman. PSQI global scores were significantly positively correlated with DAS28-CRP scores (r = 0.514, p < 0.001), RAQoL scores (r = 0.689, p < 0.001), number of medications used (r = 0.292, p = 0.003), and YDS scores (r = 0.407, p < 0.001), and significantly negatively correlated with handgrip strength (r = - 0.351, p = 0.001). CONCLUSION: The results suggest that older patients with RA might have poorer sleep quality compared to age- and gender-matched controls. Moreover, sleep quality correlated with RA disease activity and QoL in old age.

Parotid elastography: a potential alternative to replace labial biopsy in classification of patients with primary Sjögren's syndrome?

BACKGROUND: There has been a substantial improvement in classifying patients with primary Sjögren's syndrome (pSS), with the new 2016 ACR/EULAR classification criteria. It was aimed to investigate the potential role of parotid elastography in the classification of patients with pSS, as well as the clinical diagnosis of those who do not otherwise fulfil the criteria. METHOD: This is a cross-sectional analysis of patients with pSS followed up in tertiary out-patient rheumatology clinic. Patients' medical records were retrospectively investigated whether or not clinically diagnosed pSS patients fulfil 2016 ACR/EULAR criteria sets. Elastographic evaluation of parotid and submandibular glands bilaterally was performed when presented for follow-up. Strain ratio, shear wave velocity and Pascal values of the glands were obtained. RESULTS: Clinical data on 179 patients with Sjögren's syndrome were investigated. Ninety-six patients with pSS and 30 gender and age-matched healthy controls were included in the study. Eighty-six percent of the clinically diagnosed patients satisfied the 2016 ACR /EULAR criteria and were considered 'criteria patients', and the remaining were considered 'non-criteria patients'. Both criteria and non-criteria patients had significantly higher parotid strain ratio and submandibular velocity compared with healthy controls (p < 0.001 and p < 0.001 for parotid strain ratio and p < 0.001 and p = 0.016 for submandibular velocity, respectively). Replacing labial gland biopsy findings with parotid strain ratio in the new classification criteria resulted in similar sensitivity and lower specificity, 91.6% and 80%, respectively. CONCLUSION: Parotid shear elastography is an easy and noninvasive method and might be a useful tool for the classification of patients with pSS, especially when labial gland biopsy is not feasible. Key Points • Salivary gland elastography (SGE) is a useful tool for the classification of patients with pSS. • SGE could be performed instead of labial biopsy without changing the diagnostic power of classification criteria.

Frequency, characteristics, and clinical determinants of 'prodrome' in familial Mediterranean fever patients.

Objective: Prodrome is defined by manifestations that precede a familial Mediterranean fever (FMF) attack and predict its emergence. We aimed to determine the frequency, characteristics, and clinical determinants of prodrome in patients with FMF.Method: This cross-sectional study was conducted in a tertiary rheumatology clinic. During the clinical interview, all patients completed a standardized questionnaire about the pre-attack period. Prodrome was defined as the presence of any recurrent pre-attack manifestation occurring at least 4 h before an attack. Patients were classified according to whether they had prodrome of any kind of attack.Results: The study enrolled 401 patients aged 37.7 ± 11.0 years (mean ± sd). Male gender, M694V/M694V, homozygous MEFV mutation, peritonitis, pleuritis, and arthritis were more frequent in prodrome-positive patients. Altogether, 141 patients (35.2%) had prodrome. Male gender and ever having attack types of peritonitis or arthritis were independent clinical determinants of prodrome [relative risk (95% confidence interval): 1.72 (1.07-2.76), p = 0.02; 4.27 (1.80-10.1), p = 0.001; 1.77 (1.04-3.04), p = 0.04, respectively]. Age, MEFV mutations, pleuritis, and erysipelas-like erythema were not clinical determinants.Conclusions: All FMF patients, particularly males and patients who had peritonitis or arthritis at any time, should be questioned about prodrome. Prodrome should be analysed in terms of elucidating the pathogenesis of FMF and as an opportunity for a secondary prevention strategy for impending attacks. This study may shed light on prodrome for future cytokine or drug studies with the purpose of developing new cost-effective treatment protocols irrespective of colchicine resistance.

Validity and reliability study of the Turkish version of SACRAH (Score for Assessment and quantification of Chronic Rheumatic Affections of the Hands).

Rheumatic diseases cause deformities in the hands and affect daily living activities. Therefore, assessment of hand disabilities is important in rheumatic disease. The aim of this study was to test the validity and reliability of the Turkish version of the A Score For Assessment and Quantification of Chronic Rheumatic Affections of the Hands (SACRAH). A translation and back-translation of the SACRAH were performed, according to the Beaton guidelines. Patients who were between 18-65 years old, who were literate in Turkish, who had rheumatic disease diagnosis and whose hands were affected, were included in the study. Patients who were using a splint during daytime were excluded from the study. They completed the Turkish version of Disabilities of the Arm, Shoulder and Hand Questionnaire (DASH-T) once and the final version of the SACRAH Questionnaire twice with a 7 days' interval. The internal consistency (Cronbach's α) and reliability (test-retest reliability) of the questionnaire were assessed. Besides, correlations between SACRAH and DASH-T scores were analyzed using the Spearman correlation coefficient. One hundred and twenty patients participated in the study. The Turkish version of the SACRAH met set criteria of reliability and validity. Internal consistency was excellent (Cronbach's α=0.88) and test-retest reliability were very good (r=0.73). SACRAH showed a positive and statistically significant correlation with DASH-T scores (r=0.83, p<0.001). Our results show that the Turkish version of the SACRAH has excellent test-retest reliability and validity. As a result of this study we determined that SACRAH is a valid and reliable instrument for assessing functional status and subjective manual function in Turkish-speaking patients.

Chronic inflammation in adult familial Mediterranean fever patients: underlying causes and association with amyloidosis.

Background: Chronic inflammation, as determined by persistently elevated acute-phase reactants in attack-free periods, can occasionally be observed in patients with familial Mediterranean fever (FMF) and is suggested to be a risk factor for the development of amyloidosis. We aimed to investigate the underlying causes of chronic inflammation in FMF patients and its association with amyloidosis in long-term follow-up. Method: Electronic medical records of FMF patients who had regular follow-up for ≥ 5 years in our cohort were utilized. As part of routine evaluation, detailed history, physical examination, and pertinent laboratory and radiographic investigations were performed in all patients to determine potential causes of elevated C-reactive protein (CRP) levels. Results: The study included 146 FMF patients who had no evidence of amyloidosis at baseline and had regular follow-up for ≥ 5 years. Thirty-seven patients (25.3%) were found to have chronic inflammation in the disease course. Twenty-five (67.5%) of them had either very frequent attacks or chronic manifestations of disease. In the entire study group, amyloidosis developed in five patients (3.42%) during the 5 year follow-up, four in the FMF with chronic inflammation group (10.8%), and only one of the 109 patients without chronic inflammation (odds ratio 13.09, 95% confidence interval 1.41-121.2). Conclusions: The results suggest that persistently high CRP levels during the attack-free periods may be a strong risk factor for the development of amyloidosis in patients with FMF. The vast majority of FMF patients with chronic inflammation had active FMF.

Disease-specific quality of life in patients with lupus nephritis.

Background Patient-reported outcomes in lupus nephritis (LN) are not well studied. Studies with disease-targeted PRO tool in LN do not exist. Herein, we describe quality of life (QOL: HRQOL & non-HRQOL) among LN patients using LupusPRO. Methods International, cross-sectional data from 1259 patients with systemic lupus erythematosus (SLE) and LupusPRO were compared, stratified by (a) presence of LN (ACR classification criteria (ACR-LN)) at any time and, (b) active LN (on SLEDAI) at study visit. Damage was assessed by SLICC/ACR-SDI. Multivariate regression analyses for QOL against ACR-LN (active LN) after adjusting for age, gender, ethnicity and country of recruitment were performed. Results Mean (SD) age was 41.7 (13.5) yrs, 93% were women. Five hundred and thirty-nine of 1259 SLE patients had ACR-LN. ACR-LN group was younger, were more often on immunosuppressive medications, had worse QOL on lupus medications and procreation than non-ACR-LN patients. HRQOL and non-HRQOL scores were similar in both groups. One hundred and twenty-nine of 539 ACR-LN patients had active LN. Active LN group was younger, had greater disease activity and had worse HRQOL and non-HRQOL compared to patients without active LN. Specific domains adversely affected were lupus symptoms, lupus medications, procreation, emotional health, body image and desires-goals domains. Patients with ACR-LN and active LN fared significantly worse in lupus medications and procreation HRQOL domains, even after adjusting for age, ethnicity, gender and country of recruitment. Conclusions Lupus nephritis patients have poor QOL. Patients with active LN have worse HRQOL and non-HRQOL. Most domains affected are not included in the generic QOL tools used in SLE. LN patients must receive discussion on lupus medications and procreation issues. Patients with active LN need comprehensive assessments and addressal of QOL, along with treatment for active LN.

Granulomatous interstitial nephritis associated with Primary Sjögren's syndrome.

Primary Sjögren's syndrome (pSS) is an autoimmune disease characterized by lymphocytic and plasmacytic infiltration of the exocrine glands. Tubulointerstitial nephritis (TIN) is the most common type of renal involvement in pSS. However, clinically significant renal involvement is uncommon. Granulomatous interstitial nephritis (GIN) is a rare histopathological entity characterized by the presence of granulomas against a background of interstitial inflammation. GIN is not a typical and commonly seen form of TIN in pSS. Herein, we report on a patient who was concurrently diagnosed with pSS and GIN and was treated successfully with rituximab (RTX). pSS should be considered in the differential diagnosis of GIN, and RTX may be a good option in the treatment of this patient group.

Successful switch from inhaler iloprost to treatment in a patient who initially presented with pulmonary arterial hypertension and diagnosed as SLE.

BACKGROUND: SLE-induced Pulmonary Arterial Hypertension increases morbidity and mortality. Vasoconstruction, thrombosis, inflammation are the mostly proposed factors in PAH. Time until PAH develops is variable in patients with SLE. It has been shown that vasoactive agents improve symptoms and clinical findings in patients with SLE-induced-PAH. METHODOLOGY: The case note of the patient with with pulmonary arterial hypertension and SLE was retrieved and reviewed, and literature search was done. We reported a patient whose initially symptoms and clinical findings were consistent with idiopathic PAH and then had been diagnosed as SLE. RESULT: The patient tolerated the switch treatment from inhaler Iloprost from oral Bosentan well. Her symptoms, clinical findings and 6 minutes walking distance improved with treatment. CONCLUSION: Vasodilator treatment appears to be beneficial in patients with SLE-associated PAH and switch therapy also seems to be well tolerated. Additionally, in connective tissue diseases it must be kept in mind that PAH might be the presenting symptom.

The relationship between hip joint space width, center edge angle and acetabular depth.

OBJECTIVE: Radiographic parameters used to define acetabular dysplasia may be related to anthropological characteristics independent of dysplasia. The goal of the present study was to investigate the relationship between the minimal joint space width (JSW) of the hip and the parameters that define acetabular dysplasia, in clinically normal subjects. DESIGN: One hundred and eighteen patients who underwent supine abdominal radiography for non-rheumatological indications and had no hip pain or history of hip arthritis were evaluated. JSW was quantified manually using dial calipers, and center edge (CE) angle and acetabular depth were measured for each hip. RESULTS: CE angle, but not acetabular depth, correlated (inversely) with the minimal hip JSW (r=-0.26 and -0.20, P=0.005 and 0.038, R (right) and L (left) hips, respectively). CE angle inversely correlated with the pelvic width (r=-0.27 and 0.27, P=0.003 and 0.004, R and L hips, respectively) and acetabular depth correlated with subject's height (r=0.27 and 0.42, P=0.008 and <0.001 R and L hips, respectively) and leg length (r=0.27 and 0.45, P=0.008 and <0.001, R and L hips, respectively). Also, pelvic width correlated significantly with the JSW (r=0.27 and 0.20, P=0.003 and 0.033, for R and L hips, respectively). CONCLUSIONS: The radiographic parameters used to define acetabular dysplasia, CE angle and acetabular depth, are strongly associated with anthropological variables and CE angle is associated with minimal JSW of the hip. It is important to recognize that height and limb length variability may affect radiographic parameters of acetabular dysplasia, and thus may falsely suggest the presence of anatomic abnormalities in some patients.

Bone mineral density in the proximal tibia varies as a function of static alignment and knee adduction angular momentum in individuals with medial knee osteoarthritis.

Based on the premise that bone mass and bone geometry are related to load history and that subchondral bone may play a role in osteoarthritis (OA), we sought to determine if static and dynamic markers of knee joint loads explain variance in the medial-to-lateral ratio of proximal tibial bone mineral density (BMD) in subjects with mild and moderate medial knee OA. We utilized two surrogate markers of dynamic load, the peak knee adduction moment and the knee adduction angular momentum, the latter being the time integral of the frontal plane knee joint moment. BMD for medial and lateral regions of the proximal tibial plateau and one distal region in the tibial shaft was measured in 84 symptomatic subjects with Kellgren and Lawrence radiographic OA grades of 2 or 3. Utilizing gait analysis, the peak knee adduction moment (the external adduction moment of greatest magnitude) and the time integral of the frontal plane knee joint moment (the angular momentum) over the entire stance phase as well as for each of the four subdivisions of stance were calculated. The BMD ratio was not significantly different in grade 2 (1.32 +/- 0.27) and grade 3 knees (1.47 +/- 0.40) (P = 0.215). BMD of the tibial shaft was not correlated with any loading parameter or static alignment. Of all the surrogate gait markers of dynamic load, the knee adduction angular momentum in terminal stance explained the most variance (20%) in the medial-to-lateral BMD ratio (adjusted r(2) = 0.196, P < 0.001). The knee adduction angular momentum for the entire stance phase explained 18% of the variance in the BMD ratio (adjusted r(2) = 0.178, P < 0.001), 10% more variance than explained by the overall peak knee adduction moment (adjusted r(2) = 0.081, P < 0.001). 18% of the variance in the BMD ratio was also explained by the knee alignment angle (adjusted r(2) = 0.183, P < 0.001), and the total explanatory power was increased to 22% when the knee adduction angular momentum in terminal stance was added (change in r(2) = 0.041, P < 0.05, total adjusted r(2) = 0.215, P < 0.001). The BMD ratio and its relationship to dynamic and static markers of loading were independent of height, weight, and the body mass index, demonstrating that both dynamic markers of knee loading as well as knee alignment explained variance in the tibial BMD ratio independent of body size.

Serum interleukin 17 and interleukin 18 levels in familial Mediterranean fever.

OBJECTIVE: Familial Mediterranean fever (FMF) attacks are characterized by serosal inflammation rich in PMNL leukocytes and activation of a definite cytokine network. Moreover, there is sustained inflammation in attack-free FMF patients. Interleukin (IL)-17 and IL-18 are recently described proinflammatory cytokines, which can modulate certain neutrophil functions. In this study we measured serum levels of IL-17 and IL-18 in FMF patients. METHODS: The study groups comprised of 18 FMF patients in attack-free period (mean age: 30.2 +/- 9.5 years; male/female: 10/8), and 18 patients with an acute FMF attack (mean age: 25.4 +/- 4.9 years; male/female: 10/8). Twenty age-matched healthy subjects were included as a control group (male/female: 10/10). Levels of IL-17 and IL-18 were determined by commercial ELISA kits (Biosource International, USA). RESULTS: Serum IL-17 levels were 42.8 +/- 3.7, 42.7 +/- 3.2, and 39.9 +/- 2.3 pg/mL for FMF patients in attack-free period, FMF patients with acute attack, and healthy controls, respectively. Serum IL-18 levels were 878.8 +/- 315.0, 854.2 +/- 261.4, and 314.6 +/- 80.8 pg/mL for FMF patients in an attack-free period, FMF patients with acute attack, and healthy controls, respectively. Levels of both IL-17 and IL-18 were significantly higher in FMF patients with and without acute attack compared to control group (p < 0.05). Concentrations of those cytokines were comparable in FMF patients with acute attack and in attack-free period (p > 0.05). CONCLUSION: Our data suggest that IL-17 and IL-18 contribute to the cytokine network in the inflammatory cascade of FMF. However, their roles for the initiation of FMF attacks remain to be established.

The effects of rofecoxib on 24-h ambulatory blood pressure and heart rate monitoring in patients with hypertension and osteoarthritis.

The aim of the study was to investigate the effects of the cylooxygenase (COX)-2 specific inhibitor rofecoxib, on blood pressure (BP) and heart rate (HR) in patients with well-controlled hypertension and osteoarthritis via 24-h ambulatory monitoring. Thirty patients with well controlled hypertension were included. Fifteen patients had osteoarthritis and were recommended by their rheumatologists to take rofecoxib 12.5 mg/day (rofecoxib group). The control group consisted of 15 patients who had hypertension but no clinical osteoarthritis and did not receive any anti-inflammatory drugs. Twenty-four-hour ambulatory monitoring of BP and HR were performed on the day before initiation of rofecoxib therapy and on days 3 and 14 of COX-2 therapy. The control group underwent 24-h monitoring three times at similar intervals. Antihypertensive medications were continued. On day 3 of rofecoxib therapy, mean HR for both daytime and nighttime were lower than those at baseline. On day 14, the changes in mean HR did not differ from baseline values. Similarly, diastolic BP (daytime and nighttime) on day 3 appeared to be lower than at baseline. However this difference was not observed on day 14, and mean daytime and nighttime diastolic BP returned to baseline values. There was no statistically significant difference in the mean arterial pressure or systolic BP recordings on days 3 or 14 than at baseline. Rofecoxib 12.5 mg/day did not significantly increase BP during 24-h ambulatory BP monitoring in patients with well-controlled hypertension and osteoarthritis.

A case with multiple gynecological malignancies.

A patient with cervical non-Hodgkin lymphoma was treated with chemotherapy. Fourteen months after the diagnosis of the lymphoma, an endometrial adenocarcinoma was detected as a secondary malignant tumor. The patient was treated with surgery followed by radiotherapy. Approximately 7 years after the diagnosis of endometrial cancer, vaginal invasive squamous cell carcinoma was diagnosed as the third primary malignancy, and a second-line palliative radiotherapy was applied. Seven months after the last radiotherapy, postradiational sarcoma in the vagina was diagnosed. Congenital and acquired immune system disorders, viral oncogenes, and various human leukocyte antigen (HLA) types were investigated. Total blood count and lymphocyte subset analysis were performed, and CD4+ lymphopenia was detected. Serologic tests were carried out for human immunodeficiency virus, hepatitis B virus, human papillomavirus, Epstein-Barr virus, and herpes simplex virus infection. Epstein-Barr virus viral capsid antigen IgG was found positive. Low-risk human papillomavirus panel was detected by Hybrid Capture method in the cervical smear. The HLA investigation revealed HLA-A2, HLA-A3, HLA-B57, HLA-B35, HLA-B4, HLA-B6, HLA-DR3, HLA-DR1, HLA-DR51, HLA-DR52, HLA-DQ6(1), and HLA-DQ7(3). The patient died because of the disease.

The radiographic joint space width in clinically normal hips: effects of age, gender and physical parameters.

OBJECTIVE: The aim of the study was to assess the relationship of the radiographic joint space width (JSW) in the hip with age and a variety of physical parameters in a clinically non-arthritic population in order to identify potential age-related changes. DESIGN: One hundred and eighteen patients (58F/60M, age range 20-79 years) who underwent supine abdominal radiography for non-rheumatological indications and had no hip pain were evaluated. Height, weight and leg lengths were measured. JSW was quantified manually by a dial caliper, and femoral head diameters were determined for each hip. RESULTS: Overall, JSW was 3.61mm+/-0.58 (mean+/-SD) in the right (R) and 3.63mm+/-0.59 in the left (L) hip (range 2.34-6.1mm). There was no age-related decline in the JSW, either by decade (P=0.5 and 0.6, for R and L hips, respectively), or by individual age (Spearman's rho=-0.108 and 0.057, P=0.3 and 0.5 for R and L hips, respectively); similarly, no age-related changes were observed when each gender was analyzed separately. Women had significantly narrower hip JSW than men (P=0.001 and 0.01, R and L hips, respectively). However, gender was no longer significant after height was taken into account (P=0.26 and 0.45, for R and L hips, respectively). JSW correlated significantly with height, weight (which also correlated with height) (r=0.31/0.27 and 0.29/0.28 for height and weight R/L, respectively, P<0.004 for each), but not with body mass index (P=0.62 and 0.57, R and L, respectively). Hip JSW significantly correlated also with femoral head diameter and leg length (r=0.38/0.29 and 0.25/0.19 for femoral head and leg length, R/L, respectively). CONCLUSIONS: No effect of aging was detected on the radiographic JSW of the hip among normal individuals even at advanced ages. In contrast, height, femoral head diameter and leg length were directly related to JSW.

Localized Castleman's disease and nephrotic syndrome not responsive to resection plus colchicine therapy.

We describe one patient with localized Castleman's disease (CD) of the mixed hyaline vascular and plasma cell type located at the mesentery of the small bowel, associated with systemic amyloidosis and nephrotic syndrome. A true nephrotic syndrome has rarely been reported in patients with CD. In the literature, it has been suggested that clinical and laboratory manifestations generally improved after surgical resection of the tumor. However, in our case, clinical and laboratory findings did not regress after operation followed by colchicine therapy.

Radiographic osteoarthritis of the hip joint in Turkey.

The prevalence of hip osteoarthritis (OA) varies greatly across different geographic areas. Limited data exist about the prevalence of radiographic hip OA in the Turkish population. The aim of this study was to estimate the prevalence of radiographic hip OA in Turkey utilizing a random sample. That sample consisted of radiographs filed at the Department of Urology, Gazi University Hospital, Ankara, and included patients aged 25 years and older (range 25-97 years). Plain supine abdominal radiographs and intravenous pyelographies were evaluated using the Kellgren and Lawrence (K&L) grading system. Evaluations were made of 1,248 hips in 682 patients (205 women and 477 men). Overall, 8.8% of the hips evaluated had radiographic hip OA (K&L grade 2 or higher). Both hips were visible in 566 patients. Of those patients 11.7% had radiographic hip OA in either one or both hips (9.4% of women and 12.6% of men), and 51.5% of whom had bilateral findings. Prevalence increased with age and was 1.9%, 16.1%, and 21.5% for age ranges 25-39, 40-54, and 55 years and older, respectively. Patients aged 40 years and older had significantly more radiographic hip OA than those younger than 40 years (P<0.001). Men had a higher frequency than women after the age of 40 years, but this did not reach statistical significance. In most patients radiographic findings indicated only mild disease. Three patients had grade 3 disease and only one patient (aged 68 years) had grade 4 disease. Among patients aged 40 years or older. 1.1% had grade 3 or 4 disease. These findings suggest that K&L grade 2 OA changes emphasizing osteophytes at hip joints are not uncommon in the Turkish population, especially among those aged 40 years and older. However, frequency of moderate or severe radiographic hip OA may be low.