Low relapse rate in patients with giant cell arteritis in a multi-centre retrospective Turkish Registry.

OBJECTIVES: Glucocorticoids (GC) are widely accepted as the standard first-line treatment for giant cell arteritis (GCA). However, relapse rates are reported up to 80% on GC-only protocol arms in controlled trials of tocilizumab and abatacept in 12-24 months. Herein, we aimed to assess the real-life relapse rates retrospectively in patients with GCA from Turkey. METHODS: We assembled a retrospective cohort of patients with GCA diagnosed according to ACR 1990 criteria from tertiary rheumatology centres in Turkey. All clinical data were abstracted from medical records. Relapse was defined as any new manifestation or increased acutephase response leading to the change of the GC dose or use of a new therapeutic agent by the treating physician. RESULTS: The study included 330 (F/M: 196/134) patients with GCA. The mean age at disease onset was 68.9±9 years. The most frequent symptom was headache. Polymyalgia rheumatica was also present in 81 (24.5%) patients. Elevation of acute phase reactants (ESR>50 mm/h or CRP>5 mg/l) was absent in 25 (7.6%) patients at diagnosis. Temporal artery biopsy was available in 241 (73%) patients, and 180 of them had positive histopathological findings for GCA. For remission induction, GC pulses (250-1000 methylprednisolone mg/3-7 days) were given to 69 (20.9%) patients, with further 0.5-1 mg/kg/day prednisolone continued in the whole group. Immunosuppressives as GC-sparing agents were used in 252 (76.4%) patients. During a follow-up of a median 26.5 (6-190) months, relapses occurred in 49 (18.8%) patients. No confounding factor was observed in relapse rates. GC treatment could be stopped in only 62 (23.8%) patients. Additionally, GC-related side effects developed in 64 (24.6%) patients, and 141 (66.2%) had at least one Vasculitis Damage Index (VDI) damage item present during follow-up. CONCLUSIONS: In this first multi-centre series of GCA from Turkey, we observed that only one-fifth of patients had relapses during a mean follow-up of 26 months, with 76.4% given a GC-sparing IS agent at diagnosis. At the end of follow-up, GC-related side effects developed in one-fourth of patients. Our results suggest that patients with GCA had a low relapse rate in real-life experience of a multi-centre retrospective Turkish registry, however with a significant presence of GC-associated side effects during follow-up.

Mortality in psoriatic arthritis patients, changes over time, and the impact of COVID-19: results from a multicenter Psoriatic Arthritis Registry (PsART-ID).

BACKGROUND: This study aimed to assess the mortality of PsA before and during the COVID-19 pandemic. METHODS: From the prospective, multicenter PsART-ID (Psoriatic Arthritis Registry-International Database), patients from Turkey were analyzed by linking the registry to the Turkish Cause of Death Registry. The outcome of interest was death from any cause, pre-pandemic (since the onset of registry-March 2014-March 2020), and during the pandemic (March 2020-May 2021). The crude mortality rate and standardized mortality ratio (SMR) were determined. RESULTS: There were 1216 PsA patients with a follow-up of 7500 patient-years. Overall, 46 deaths (26 males) were observed. In the pre-pandemic period, SMR for PsA vs the general population was 0.95 (0.61-1.49), being higher in males [1.56 (0.92-2.63)] than females [0.62 (0.33-1.17)]. The crude mortality rate in PsA doubled during the pandemic (pre-pandemic crude mortality rate: 5.07 vs 10.76 during the pandemic) with a higher increase in females (2.9 vs 8.72) than males (9.07 vs 14.73). CONCLUSION: The mortality in PsA was found similar to the general population in the pre-pandemic era. The mortality rates in PsA doubled during the pandemic. Whether PsA patients have more risk of mortality than the general population due to COVID-19 needs further studies. Key Points • Decrease in mortality in PsA might be expected with the more effective treatment options and better disease control. • A crude mortality rate is comparable to the general population and not increased until the pandemic. • Currently, there is a 2-fold increase in crude mortality rate possibly due to the COVID-19.

Evaluation of histopathological results of minor salivary gland biopsies in patients with the diagnosis of Sjögren's syndrome.

Objectives: This study aims to evaluate which of the histomorphological criteria defined in labial salivary gland biopsy are more valuable in diagnosing Sjögren's syndrome (SS) and to examine its correlation with clinical and laboratory findings. Patients and methods: Between January 2005 and January 2019, a total of 927 patients (104 males, 823 females; mean age: 51 years; range, 19 to 85 years) who underwent minor salivary gland biopsies with the suspicion of SS were retrospectively analyzed. The American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2016 classification criteria were used for the classification of SS. We evaluated salivary gland biopsies histomorphologically for the presence and number of lymphocytic focus, as well as chronicity findings (acinar atrophy, ductal dilatation, fibrosis), the presence of lymphocytic infiltration, distribution, localization, ectopic germinal center, and mast cell count. The presence of accompanying diseases, clinical and laboratory findings including age, sex, the presence of dry eye and mouth, and autoantibodies for discriminating SS were noted. Histomorphologically, salivary gland biopsy which fulfilled the adequacy criteria for glandular tissue were compared with the other criteria used to diagnose SS. Results: Strong chronicity and diffuse lymphocytic infiltration were significantly higher in the SS group compared to the non-SS group (p<0.001). Lymphocytic focus score >1 was significantly higher in the SS group compared to the non-SS group (p<0.001). Strong chronicity, acinar atrophy, andductal dilatation were significantly higher in the SS group compared to the non-SS group (p<0.001). Conclusion: More than one lymphocytic focus is the most valuable finding in diagnosing SS. However, it should be kept in mind that, in cases of SS, ductal dilatation, acinar atrophy, and chronicity may be present without lymphocytic infiltration.

PsART-ID inception cohort: clinical characteristics, treatment choices and outcomes of patients with psoriatic arthritis.

OBJECTIVES: Our aim is to understand clinical characteristics, real-life treatment strategies, outcomes of early PsA patients and determine the differences between the inception and established PsA cohorts. METHODS: PsArt-ID (Psoriatic Arthritis- International Database) is a multicentre registry. From that registry, patients with a diagnosis of PsA up to 6 months were classified as the inception cohort (n==388). Two periods were identified for the established cohort: Patients with PsA diagnosis within 5-10 years (n = 328), ≥10 years (n = 326). Demographic, clinical characteristics, treatment strategies, outcomes were determined for the inception cohort and compared with the established cohorts. RESULTS: The mean (s.d.) age of the inception cohort was 44.7 (13.3) and 167/388 (43.0%) of the patients were male. Polyarticular and mono-oligoarticular presentations were comparable in the inception and established cohorts. Axial involvement rate was higher in the cohort of patients with PsA ≥10 years compared with the inception cohort (34.8% vs 27.7%). As well as dactylitis and nail involvement (P = 0.004, P = 0.001 respectively). Both enthesitis, deformity rates were lower in the inception cohort. Overall, 13% of patients in the inception group had a deformity. MTX was the most commonly prescribed treatment for all cohorts with 10.7% of the early PsA patients were given anti-TNF agents after 16 months. CONCLUSION: The real-life experience in PsA patients showed no significant differences in the disease pattern rates except for the axial involvement. The dactylitis, nail involvement rates had increased significantly after 10 years from the diagnosis and the enthesitis, deformity had an increasing trend over time.

Disease characteristics of psoriatic arthritis patients may differ according to age at psoriasis onset: cross-sectional data from the Psoriatic Arthritis-International Database.

OBJECTIVES: To explore the impact of early versus late-onset psoriasis (PsO) on the disease characteristics of psoriatic arthritis (PsA) in a large-multicentre cohort. METHODS: The data from a multicentre psoriatic arthritis database was analysed. Patients were grouped according to age at psoriasis onset (early onset; <40 years of age, late-onset; >40 years of age) and disease characteristics of the groups were compared by adjusting for BMI and PsA duration, where necessary. RESULTS: At the time of analyses, 1634 patients were recruited [62.8% females; early onset 1108 (67.8%); late-onset, 526 (32.2%)]. The late-onset group was more over-weight [66.8% vs. 86.8%, p<0.001; adjusted for age - aOR 1.55 (1.11-2.20; 95% CI)]. The early onset group had more scalp psoriasis at onset (56.7% vs. 43.0%, p<0.001), whereas extremity lesions were more common in the late-onset group (63.8% vs. 74.2%, p<0.001). Axial disease in males and psoriatic disease family history in females were significantly higher in the early onset group [38.0% vs. 25.4%; p=0.005; adjusted for PsA duration - aOR 1.76 (1.19-2.62; 95% CI) / 39.5% vs. 30.1%; p=0.003; OR 1.51 (1.15-1.99; 95% CI), respectively]. Psoriatic disease activity parameters, patient-physician reported outcomes and HAQ-DI scores were similar in both groups. CONCLUSIONS: Clinical features of PsA may be affected by the age at onset of PsO. Different genetic backgrounds in early and late-onset PsO may be driving the differences in psoriasis and PsA phenotypes.

Large joint and lower extremity involvement have higher impact on disease outcomes in oligoarticular psoriatic arthritis.

OBJECTIVE: Joints with different sizes and anatomical locations can be affected in psoriatic arthritis (PsA). Our aim was to explore the effect of different joint patterns on patient-reported outcomes (PROs) in patients with mono-oligoarthritis. METHODS: Within PsArt-ID (Psoriatic Arthritis- International Database), 387/1670 patients who had mono-oligoarthritis (1-4 tender and swollen joints) were enrolled in cross-sectional assessment. The joints were categorized according to their size (small/large) and location (upper/lower extremity) and PROs, physician global assessment and C-reactive protein (CRP) were compared. Analysis was made by categorizing according to joint counts (1-2 joints/ 3-4 joints). RESULTS: The mean age (SD) was 46.9 (14.24) with a mean (SD) PsA duration of 3.93 (6.03) years. Within patients with 1-2 involved joints (n = 302), size of the joints only had an impact on CRP values with large joints having higher CRP (P = .005), similar to lower extremity involvement (P = .004). PROs were similar regardless of size or location if 1-2 joints were inflamed. Within patients with 3-4 involved joints (n = 85), patient global assessment (PGA), pain, fatigue and physician global assessment were higher in the group with large joints. Similarly, PGA, pain, and physician global assessment were higher in patients with lower extremity involvement as well as higher CRP values. CONCLUSION: For PsA patients with 3-4 joints involved, lower extremity and large joints are associated with poorer outcomes with worse PROs, physician global assessment, and higher CRP. The size and anatomical location of the joints are less important for patients with 1-2 joints in terms of the PROs.

Impact of Having Family History of Psoriasis or Psoriatic Arthritis on Psoriatic Disease.

OBJECTIVE: Psoriatic arthritis (PsA) has a genetic background. Approximately 40% of patients with psoriasis or PsA have a family history of psoriasis or PsA, which may affect disease features. The aim of this study was to assess the effects of family history of psoriasis and PsA on disease phenotypes. METHODS: Data from 1,393 patients recruited in the longitudinal, multicenter Psoriatic Arthritis International Database were analyzed. The effects of family history of psoriasis and/or PsA on characteristics of psoriasis and PsA were investigated using logistic regression. RESULTS: A total of 444 patients (31.9%) had a family history of psoriasis and/or PsA. These patients were more frequently women, had earlier onset of psoriasis, more frequent nail disease, enthesitis, and deformities, and less frequently achieved minimal disease activity. Among 444 patients, 335 only had psoriasis in their family, 74 had PsA, and 35 patients were not certain about having PsA and psoriasis in their family, so they were excluded from further analysis. In the multivariate analysis, family history of psoriasis was associated with younger age at onset of psoriasis (odds ratio [OR] 0.976) and presence of enthesitis (OR 1.931), whereas family history of PsA was associated with lower risk of plaque psoriasis (OR 0.417) and higher risk of deformities (OR 2.557). Family history of PsA versus psoriasis showed increased risk of deformities (OR 2.143) and lower risk of plaque psoriasis (OR 0.324). CONCLUSION: Family history of psoriasis and PsA impacts skin phenotypes, musculoskeletal features, and disease severity. The link between family history of psoriasis/PsA and pustular/plaque phenotypes may point to a different genetic background and pathogenic mechanisms in these subsets.

Current Smoking Is Increased in Axial Psoriatic Arthritis and Radiographic Sacroiliitis.

OBJECTIVE: The effect of smoking in psoriatic arthritis (PsA) is under debate. Our aim was to test whether smoking is increased in axial PsA (axPsA). METHODS: Included in the analysis were 1535 patients from PsArt-ID (PsA-International Database). The effect of smoking on axPsA (compared to other PsA phenotypes) and radiographic sacroiliitis were investigated. RESULTS: Current smoking was more common in axPsA (28.6% vs 18.9%, p < 0.001). It also was found as an independent predictor of axPsA (OR 1.4) and radiographic sacroiliitis (OR 6.6). CONCLUSION: Current smoking is significantly associated with both axPsA and radiographic sacroiliitis in patients with PsA.

What are the main barriers to achieve minimal disease activity in psoriatic arthritis in real life?

OBJECTIVES: Minimal disease activity (MDA) is an important target in patients with psoriatic arthritis (PsA), however it is also criticised for having a low threshold for patient reported outcomes (PRO).The aim of the study was to assess the prevalence of MDA and its components in patients with PsA and to evaluate disease characteristics and patterns in patients with or without MDA (MDA+ or MDA-). METHODS: PsArt-ID (Psoriatic Arthritis-International Database) is a prospective, multicentre web-based registry. PsA patients who had at least 1 year of disease duration and had full data for MDA were included for this analysis (n=317). Patients were considered in MDA+ when they met at least 5/7 of the MDA criteria. RESULTS: MDA was achieved in 46% patients. Within MDA- patients, body surface area (51.2%) and swollen joint count (53.5%) domains could still be achieved in the majority and 93.5% of them had no enthesitis using the Leeds enthesitis index. Of 170 patients with MDA-, 90 patients did not fulfill all 3 PROs of MDA. Mono-arthritis subtype (RR: 2.01), absence of enthesitis (RR: 1.570) and absence of distal interphalangeal (DIP) joint disease (RR: 1.1) were associated with higher probability of achieving MDA. CONCLUSIONS: The MDA criteria provide an objective target for treatment in trials and clinical practice; however, in real life PROs are the most significant barriers to achieve MDA. The presence of DIP joints disease makes it difficult to reach MDA due to active PROs.

Bone mineral density and serum osteocalcin levels in patients with gout.

Objective: The objective of the study is to evaluate the relation of gout with osteoporosis and serum osteocalcin (OC) levels. Material and methods: Seventy-five patients diagnosed with gout and 55 controls were included in the study. Comorbid conditions and drugs associated with osteoporosis were excluded. The T and Z scores from lumbar spine (L2-L4) and femur (neck, ward, trochanter, total) were determined by dual-energy X-ray absorptiometry (DXA). OC levels were measured by enzyme-linked immunosorbent assay. Results: Osteoporosis according to T scores of lumbar vertebrae L2-L4 was found to be significantly higher in patients with gout compared to the control group (p = 0.02). Lumbar spine T-score was -1.6 in gout group and -1.0 in controls. OC level was 7.9 ng/mL in the gout group and 18.9 ng/mL in the control group. There was a significant difference (p < 0.001). In addition, mean OC level was 12.4 ± 6.9 ng/mL in the patients diagnosed with osteoporosis and 17.2 ± 10.6 ng/mL in the patients that were classified as normal and a significant difference was established between the two groups (p = 0.03). A significant negative correlation was found between OC level and body mass index, age, and age at first attack. Similarly, femoral T-score established a negative correlation with parathyroid hormone, age, age at first attack, and allopurinol dose. Conclusion: Serum OC level can be a useful marker in the assessment of bone turnover and clinicians should keep osteoporosis in mind in gout patients.

Association of synaptosomal-associated protein 25 (SNAP-25) gene polymorphism with temperament and character traits in women with fibromyalgia syndrome

Abstract Background: Synaptosomal-associated protein 25 (SNAP-25) may be contribute to the pathogenesis of fibromyalgia Syndrome (FMS) by affecting the release of neurotransmitters. Objectives: We aimed to investigate the relationship between the SNAP-25 gen (DdeI = rs1051312 and MnlI = rs3746544) polymorphism and the temperament and character traits. Methods: A total of 85 female patients diagnosed with FMS and 70 age-matched healthy female subjects were enrolled into the study. The Temperament and Character Inventory (TCI) were performed on all the patients. SNAP-25 gene polymorphism was determined in the patients group and controls group. Results: No significant difference between groups was found regarding the distribution of SNAP-25 MnlI polymorphism (p > 0.05), but it was seen that the frequency of TC genotype for DdeI gene was higher in the patients group (p < 0.05). Increased hazard avoidance was found in the patients group (p < 0.05). When TCI scores were assessed in terms of SNAP-25 gene polymorphism, no statistically significant relationship was detected between the TT, TG, GG genotypes for MnlI gen and TCI scores (p > 0.05). However, increased hazard avoidance was detected in patients with TC genotype for DdeI gene compared to patients without such genotype. Discussion: SNAP-25 might be an etiological factor in FMS pathogenesis and might affect personality traits of FMS patients by mediating neurotransmitter release.

Axial psoriatic arthritis: the impact of underdiagnosed disease on outcomes in real life.

Psoriatic arthritis (PsA) may affect different joints, including the spine. The prevalence of spinal involvement is variable depending on the definition and a subset of patients have been identified in cohorts that do not have clinical features of axial disease and yet have imaging findings. Still, there is not a consensus on how and when to screen axial disease. In this study, we aimed to investigate factors associated with being underdiagnosed for axial psoriatic arthritis (axPsA) and its impacts on outcomes. Disease features and outcomes of axPsA according to the physician (n = 415) were compared with patients with imaging findings only (sacroiliitis fulfilling the modified New York criteria, n = 112), using data from a real-life PsA registry. Patients with imaging findings only were more frequently women (83/220 (37.7%) vs 29/122 (23.8%); p = 0.008). This group also had higher peripheral disease activity (imaging only vs clinical AxPsA: mean (SD) tender joint count 5.3 (6.1) vs 3.3 (4.7), swollen joint count 1.9 (2.9) vs 1.2 (2.4); p < 0.001 for both comparisons) and was less often treated using TNF inhibitors (16.1 vs 38.2%; p < 0.001) than patients who were classified as axPsA. Patient-reported outcomes were similar in both groups. PsA patients, especially women with more severe peripheral disease, have a higher risk of being underdiagnosed for axPsA. The severity of peripheral symptoms may be a risk factor to mask the spinal features of PsA.

A clinical threat in patients with granulomatosis polyangiitis in remission: Subglottic stenosis.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatous disease that involves small- and medium-sized arteries and affects the main respiratory tracts and kidneys. Upper respiratory tract involvement usually occurs in 90% of patients, who most frequently present with symptoms of chronic sinusitis. Subglottic stenosis (SS) is a rare and severe complication that is usually observed in approximately 15% of patients. Here we present a case of SS in a patient with limited form of GPA during remission.

Serum, salivary, and tissue levels of plasminogen in familial Mediterranean fever, amyloidosis, and chronic periodontitis.

BACKGROUND: There are no published studies regarding the role of the plasminogen (PLG) system in familial Mediterranean fever (FMF), FMF-associated secondary amyloidosis, or chronic periodontitis (CP), although recent limited data have focused on the association between FMF and chronic periodontitis. Therefore, the aim of this study was to evaluate the serum, salivary, and gingival tissue levels of PLG in patients with CP, FMF, and amyloidosis. METHODS: The study population included 122 patients with FMF (only FMF, and FMF and amyloidosis and 128 individuals who were systemically healthy controls. Blood and salivary samples were obtained from the cases and controls, and clinical periodontal parameters were recorded. Serum and salivary PLG levels were assessed. The gingival tissue samples of the case and control groups were analyzed histopathologically and immunohistochemically for amyloid deposition and PLG. RESULTS: The amyloidosis group had significantly more severe clinical periodontal parameters than those of the FMF and systemically healthy groups (P < 0.05). Salivary levels of PLG were significantly higher in the FMF and amyloidosis groups compared with those in the control group (P < 0.001). The FMF with periodontitis and amyloidosis with periodontitis groups had higher salivary PLG levels compared with those in the CP group. Serum and salivary PLG levels were significantly associated with the clinical periodontal parameters in the FMF group. The amyloidosis cases had hyperplasia, severe inflammation, and activation of the gingiva. CONCLUSION: The PLG system could play an important role in inflammatory diseases, such as chronic periodontitis, FMF, and FMF-associated secondary amyloidosis.

Nationwide Experience With Off-Label Use of Interleukin-1 Targeting Treatment in Familial Mediterranean Fever Patients.

OBJECTIVE: Approximately 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anti-IL-1) agents. Here we report our experience with the off-label use of anti-IL-1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study. The medical records of all FMF patients who had used anti-IL-1 treatment for at least 6 months were reviewed. RESULTS: In total, 172 FMF patients (83 [48%] female, mean age 36.2 years [range 18-68]) were included in the analysis; mean age at symptom onset was 12.6 years (range 1-48), and the mean colchicine dose was 1.7 mg/day (range 0.5-4.0). Of these patients, 151 were treated with anakinra and 21 with canakinumab. Anti-IL-1 treatment was used because of colchicine-resistant disease in 84% and amyloidosis in 12% of subjects. During the mean 19.6 months of treatment (range 6-98), the yearly attack frequency was significantly reduced (from 16.8 to 2.4; P < 0.001), and 42.1% of colchicine-resistant FMF patients were attack free. Serum levels of C-reactive protein, erythrocyte sedimentation rate, and 24-hour urinary protein excretion (5,458.7 mg/24 hours before and 3,557.3 mg/24 hours after) were significantly reduced. CONCLUSION: Anti-IL-1 treatment is an effective alternative for controlling attacks and decreasing proteinuria in colchicine-resistant FMF patients.

Effects of Vitamin D Therapy on Quality of Life in Patients with Fibromyalgia.

OBJECTIVE: The role of vitamin D in the etiopathogenesis of fibromyalgia and non-specific musculoskeletal pain is controversial. In our study, we aimed to investigate the effect of vitamin D therapy on quality of life in patients with fibromyalgia. MATERIALS AND METHODS: Seventy patients diagnosed with fibromyalgia and 65 age- and sex-matched controls were included in the study. Patients were grouped as deficient (<20 ng/mL), inadequate (20-30 ng/mL), and sufficient (>30 ng/mL) according to the levels of vitamin D. Vitamin D replacement was performed for patients with deficiencies and inadequacies. Before and after vitamin D therapy, patients filled in the assessment tools, fibromyalgia impact questionnaire (FIQ), Arizona sexual experience scale (ASEX), Beck depression inventory (BDI), visual analog scale (VAS), and short form-36 (SF-36). RESULTS: Vitamin D deficiencies and inadequacies were observed in 60% of the patients (n=42). Among patients with low and normal levels of vitamin D, no statistically significant difference was observed in their values. In scales examined after vitamin D replacement therapy, statistically significant differences were observed in the FIQ, BDI, VAS, and SF-36 compared with pre-treatment. CONCLUSION: Vitamin D deficiency seems to be linked to the pathogenesis of fibromyalgia. Vitamin D supplementation may improve the quality of life in patients with fibromyalgia.

Catastrophic antiphospholipid syndrome treated with rituximab: A case report.

Catastrophic antiphospholipid syndrome (CAPS) is a rare and fatal condition that is characterized by diffuse venous and/or arterial thromboembolism within a short period of time and histopathological confirmation of small-vessel occlusion in at least one organ or tissue in the presence of positive antiphospholipid antibodies. Here we report the case of a 19-year-old woman with CAPS. During the first week of her hospitalization, she was diagnosed with CAPS on the basis of skin necrosis, pulmonary artery thrombosis, cerebral venous sinus thrombosis, and positive lupus anticoagulant. She was treated with corticosteroids, intravenous immunoglobulins, plasmapheresis, and anticoagulants. Forty days after the onset of CAPS, cutaneous lesions were recurred during skin surgery. She required a high dose of corticosteroids, intravenous immunoglobulins, and rituximab. No further thrombotic events occurred. Rituximab may be an effective treatment option for patients with CAPS.

Chronic Non-Productive Cough in Patients With Primary Sjögren's Syndrome.

OBJECTIVES: This study aims to evaluate the frequency of chronic cough due to dryness in the trachea and the relationship between depression and chronic cough in patients with primary Sjögren's syndrome (pSS). PATIENTS AND METHODS: Eighty non-smoking patients (7 males, 73 females; mean age 47.6±9.2 years; range 18 to 70 years) with newly diagnosed pSS were included. All patients were evaluated clinically, radiologically, and physiologically. Patients with cough were assessed using the Leicester Cough Questionnaire and visual analog scale. Beck Depression Inventory was used to determine the risk of depression in patients with cough. Patients with asthma, rhinosinusitis, gastrooesophageal reflux, and drug use which cause cough and pulmonary involvement in pSS were excluded. RESULTS: Non-productive cough was detected in 30 patients (37.5%). Fourteen patients (17.5%) were admitted to the hospital with cough at least once before the diagnosis of pSS. The median time between onset of symptoms and firm diagnosis was 24 months (25-75 interquartile ranges: 3-60 months). A significant difference was observed in the Beck Depression Inventory scores between patients with and without cough. There was a significant negative relationship between Leicester Cough Questionnaire and Beck Depression Inventory scores. CONCLUSION: Patients with pSS often have symptoms for a long duration that mimic those of more commonly encountered non-specific pulmonary conditions. Xerotrachea should be considered in the differential diagnosis of chronic cough, especially in patients with normal pulmonary function tests and pulmonary imaging.

The Psoriatic Arthritis Registry of Turkey: results of a multicentre registry on 1081 patients.

OBJECTIVE: The aim was to assess the characteristics of PsA, find out how well the disease is controlled in real life, demonstrate the treatments and identify the unmet needs. METHODS: The PsA registry of Turkey is a multicentre Web-based registry established in 2014 and including 32 rheumatology centres. Detailed data regarding demographics for skin and joint disease, disease activity assessments and treatment choices were collected. RESULTS: One thousand and eighty-one patients (64.7% women) with a mean (sd) PsA duration of 5.8 (6.7) years were enrolled. The most frequent type of PsA was polyarticular [437 (40.5%)], followed by oligoarticular [407 (37.7%)] and axial disease [372 (34.4%)]. The mean (sd) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8), respectively. Of these patients, 38.6% were on conventional synthetic DMARD monotherapy, 7.1% were on anti-TNF monotherapy, and 22.5% were using anti-TNF plus conventional synthetic DMARD combinations. According to DAS28, 86 (12.4%) patients had high and 105 (15.2%) had moderate disease activity. Low disease activity was achieved in 317 (45.7%) patients, and 185 (26.7%) were in remission. Minimal disease activity data could be calculated in 247 patients, 105 of whom (42.5%) had minimal disease activity. The major differences among sexes were that women were older and had less frequent axial disease, more fatigue, higher HAQ scores and less remission. CONCLUSION: The PsA registry of Turkey had similarities with previously published registries, supporting its external validity. The finding that women had more fatigue and worse functioning as well as the high percentage of active disease state highlight the unmet need in treatment of PsA.

The significance of urinary beta-2 microglobulin level for differential diagnosis of familial Mediterranean fever and acute appendicitis.

The clinical and laboratory parameters widely used are not specific to discriminate the abdominal pain due to FMF attack from that of acute appendicitis. The present study aims to investigate the urinary beta-2 microglobulin (U-ß2M) level as a potential parameter to identify these two diseases mimicking each other. A total of 51 patients with established FMF diagnosis due to Tel Hashomer criteria on colchicine treatment (1-1.5 mg/day), 15 patients with acute appendicitis who had appropriate clinical picture and were also supported pathologically after the surgery, and 20 healthy controls were enrolled in the study. Of the 51 patients with FMF, 25 were at an attack period, while remaining 26 were not. For the diagnosis of acute attack, as well as physical examination, laboratory tests including white blood cell count, C-reactive protein, and erythrocyte sedimentation rate were performed. From urine specimens U-ß2M, microalbumin, and N-acetyl glucosaminidase (U-NAG) were measured. U-ß2M levels were significantly higher in acute appendicitis group compared to FMF attack, FMF non-attack, and control groups (p < 0.001, p < 0.001, and p < 0.001, respectively). U-NAG and microalbuminuria were significantly higher in acute appendicitis, FMF attack, and FMF non-attack groups compared to controls (U-NAG p < 0.001, p = 0.016, p = 0.004, microalbuminuria p < 0.001, p < 0.001, p < 0.001, respectively). Microalbuminuria was significantly higher in acute appendicitis group compared to the FMF attack group (p = 0.004). Determination of U-ß2M levels may be helpful for differential diagnosis of peritonitis attacks of FMF patients on colchicine treatment and acute appendicitis. However, this finding should be substantiated with other studies.