Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti-Jo-1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long-term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65-year-old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement.

Background Sjögren's syndrome (SS) is a chronic and autoimmune disease characterized by inflammation of the exocrine glands. Nervous system dysfunction is a common and important complication in Primary Sjögren's syndrome (pSS) and may be the cause of significant morbidity. A wide variety of peripheral (PNS) and central nervous system (CNS) complications are among the severe complications of pSS. Autonomic system involvements are also reported in pSS. Electroneuromyography has been proved to be an useful confirmatory diagnostic tool, because all the clinically symptomatic patients with peripheral neuropathy or polyneuropathy presented electromyographic and nerve conduction abnormalities. Sympathetic skin response (SSR) is a noninvasive method that can be used easily to evaluate autonomic dysfunction. It involves a recordable skin potential change following the application of an internal or external stimulus. Although it has been used in a variety of clinical settings to evaluate sudomotor sympathetic function, literature does not reveal any studies using SSR in pSS. Objectives The aim of this study was to show the possible autonomic involvement in patients with pSS. Methods Peripheral nerve electrophysiological studies and the SSR were studied in 29 patients with pSS (inactive disease period) and 31 normal controls. Motor and sensory nerve conduction studies were performed in the median, ulnar, tibial, peroneal and sural nerves using a standard method. Motor nerve conduction velocity, distal motor latency, and compound muscle action potential were recorded for the median, ulnar, peroneal and tibial nerves. Sensory conduction velocity and sensory nerve action potential were assessed for median, ulnar and sural nerves. For the palmar SSR, surface electrodes were used with the active recording electrode placed on the palm of the hand, and the reference electrode located at the wrist on the dorsum of the hand. SSR parameter included the latency in the onset of depolarization which was indicated by the first continuous deflection from the baseline. The amplitude was measured from peak to peak. Four successive SSRs were induced by unexpected acoustic stimuli, pinprick, touching and median nerve electrical stimulation at 30 mA stimulus intensity. The mean of these four latencies and amplitudes were taken as the valid value. Results The latencies of SSRs were delayed (p=0.024) and the amplitude of SSR were decreased (p=0.010) in patients with pSS when compared with the control subjects. When the peripheral motor and sensory nerves' conduction parameters of controls were compared with the patients' values, only median motor compound muscle action potentials were significantly lower in the patients (p=0.021). There were no statistically significant differences among the remaining electrophysiological results. Conclusions These findings reflect that sympathetic system is affected in pSS and this involvement is possibly seen before the peripheral nerve involvement. Our results suggest that SSR parameters may be used ...

BackgroundComorbidities are frequent in Psoriatic arthritis (PsA) and can have an impact on morbidity and mortality.ObjectivesWe aimed to investigate the frequency of different comorbidities in PsA, how comorbidities have an impact on PsA features and which factors were associated with the occurrence of comorbidities in a large number of patients, using PsART (Psoriatic Arthritis Registry of Turkey)registry.MethodsPsART is a national, web-based registry where consecutive patients with PsA are recruited and demographic, clinical and therapeutic information are collected as well as comorbidities. The following comorbidities are questioned: Hypertension (HT), hyperlipidemia, depression, diabetes mellitus (DM), hyperuricemia, coronary arterial disease, liver disease, cerebrovascular accident, cancer, and depression. BMI more than 30 is recorded as obesity. Patients with or without comorbidities were compared for their demographic features and outcome tools.ResultsOne thousand and sixty-nine patients whom comorbidity data were available were analyzed. Within these 693 (64.8%) were women. Mean (SD) age was 46.9 (12.8), with a median (range) PsA duration 45 (0–545) months. The number of patients with 1, 2 and 3 comorbidities were 642 (60.1%), 345 (32.3%), and was 191 (17.9%), respectively. Frequency of comorbidities was obesity in 327 (30.6%), HT in 256 (23.9%), hyperlipidemia in 199 (18.6%), depression in 188 (17.6%), DM in 161 (15.1%), hyperuricemia in 74 (6.9%), coronary artery disease in 39 (3.6%), liver disease 38 (3.6%), cerebrovascular event in 19 (1.8%), and cancer history 16 (1.5%). Patients with at least one comorbidity were older (51.1 (11.8) vs 40.9 (11.7), p<0.001) and had a higher disease activity (according to tender joint count (3.9 (5.2) vs 2.9 (4.2), p=0.001), pain (4.6 (2.6) vs 4.0 (2.6), p=0.006), patient global assessment (4.5 (2.4) vs 4.0 (2.5), p=0.014), fatigue (4.9 (2.5) vs 4.0 (2.6), p<0.001)). Education was found to contribute on comorbidities as patients with at least one comorbidity had less education in terms of school years (7.6 (4.4) vs 9.7 (4.4) years, p<0.001). This was also found separately for some of the comorbidities: Patients with DM (mean (SD) years of education 6.7 (4.2) vs 8.8 (4.5), p<0.001), HT (6.7 (4.3) vs 8.9 (4.5), p<0.001), cerebrovascular event (5.5 (2.9) vs 8.4 (4.5), p=0.012) and depression (7.5 (4.1) vs 8.6 (4.6), p=0.004) had less education compared to patients who don't have those comorbidities.ConclusionsOur registry supported that comorbidities are frequent in PsA increasing the complexity of the disease and have an impact on the disease severity. Patients with comorbidities are less educated showing that education and awareness are important to improve patient outcomes in PsA in long term.Disclosure of InterestNone declared

BackgroundPsART (PsA registry of Turkey) is a multicenter web-based registry.ObjectivesThe objective of this registry is to assess characteristics of PsA and compare the differences among genders in our population.MethodsPsART was established in 2014 including 32 rheumatology centers. Detailed data regarding demographics for the skin and joint disease, disease activity assessments and treatment choices were collected.ResultsOne thousand and eighty-one patients (64.7% women) with a median (range) PsA duration for 3.7 (0–45) years were enrolled. Median psoriasis duration was 13 (0–59) years. The most frequent type of PsA was polyarticular 437 (40.5%) followed by oligoarticular 407 (37.7%) and axial disease 372 (34.4%). Patients with a combination of axial and peripheral disease had a longer disease duration than patients with one type of joint disease (52 (0–545) vs 42 (0–486) months, p=0.008). Mean (SD) swollen and tender joint counts were 1.7 (3) and 3.6 (4.8). 38.6% of patients were on csDMARD monotherapy, 7.1% were anti-TNF monotherapy and 22.5% were using anti-TNF plus csDMARD combinations. Within items included in the minimal disease activity, only 17.6%>67% of patients had inactive disease state. The major differences among genders were women being older (48.3 (12.8) VS 44.4 (12.5), P<0.001), more fatigue and higher HAQ scores (Table). Women also had more frequent peripheral arthritis than men and less axial disease (only peripheral vs axial vs combined, n (%): women: 479 (68.5%), 62 (8.9%), 158 (22.6%) respectively; men: 222 (59.4%), 41 (11%), 111 (29.7%) respectively; p=0.011). Women had significantly more history of psoriasis in their family compared to men (34.8% vs 25.7%, p=0.003).All patients (n=1081)Female (n=702)Male (n=379)pInflammatory LBP n (%)275 (25.4)155 (22.3)120 (32.1)0.002Duration of morning stiffness37.9 (43.0)38.5 (43.8)36.7 (41.6)0.73Patient global assessment43.5 (24.9)44.3 (24.0)42.1 (26.6)0.22Fatigue VAS46.2 (26.2)48.5 (24.6)42.0 (28.6)0.002Pain VAS44.6 (27.1)45.5 (26.0)43.1 (28.9)0.21HAQ0.71 (0.65)0.74 (0.64)0.66 (0.67)0.014BASDAI39.4 (25.6)39.1 (24.5)39.9 (27.3)0.88BASFI29.0 (24.2)28.1 (23.7)30.7 (24.9)0.27PSI7.5 (7.2)7.4 (7.3)7.7 (7.1)0.57Physician global assessment35.6 (22.2)35.6 (21.1)35.8 (24.1)0.86Swollen joint counts1.7 (3.0)1.6 (3.1)1.8 (2.8)0.91Tender joint counts3.6 (4.8)3.7 (5.0)3.4 (4.3)0.93Body surface area6.1 (10.7)4.9 (8.0)8.4 (14.2)0.69Leeds enthesitis index0.2 (0.7)0.2 (0.8)0.2 (0.5)0.43ConclusionsPsART had similarities with the previously published registries, supporting its external validity. Women having more fatigue and worse functioning and as well as the high percentage of active disease state point out to the unmet need in treatment of PsA.Disclosure of InterestNone declared