Vascular Calcification in Takayasu Arteritis; Relationship with Metabolic Syndrome, Left Ventricular Mass Index and Intima-Media Thickness.

INTRODUCTION: ; The present study aims to determine the frequency of vascular calcification in Takayasu arteritis (TA) and the risk factors for it and to evaluate its relation with atherosclerotic predictors such as metabolic syndrome (MS), left ventricular mass index (LVMI) and carotid intima-media thickness (CIMT). METHOD: A cross-sectional study was conducted in patients with TA; MS was defined according to the US National Cholesterol Education Program/Adult Treatment Panel III (NCEP/ATP III) criteria. The study included 49 TA patients (22 with MS, 27 without MS) and 31 healthy controls (HCs). Non-contrast computed tomography measured calcification in coronary arteries, aorta, and branches. RESULTS: Forty-seven patients (95.9%) were female and mean age was 33.45±8.53 years. Total calcification score (mean±SD; 5223.9±18041.1 AU vs 35.87±72.70 AU (p=0.05)), CIMT and LVMI were found to be significantly higher in TA patients than HCs (p<0.05). While there was no significant difference between the total calcification score of MS (+) TA patients and MS (-) TA patients, in both patient groups, the total calcification score was found to be significantly higher than HCs. MS (+) and MS (-) groups were found to have significantly higher CIMT and LVMI values than the control group, in addition, MS (+) patients were found to have significantly higher LVMI and CIMT values than MS (-) group (p<0.05). CONCLUSION: ; Vascular calcification, CIMT, and LVMI are elevated in all TA patients, with greater impact in the presence of MS.

Assessment and comparison of the 2023 ACR/EULAR APS criteria with the revised Sapporo criteria.

OBJECTIVE: To analyze antiphospholipid antibody (aPL)-positive patients using the 2023 American College of Rheumatology/The European Alliance of Associations for Rheumatology (ACR/EULAR) antiphospholipid syndrome (APS) classification criteria and compare the revised Sapporo criteria and the 2023 ACR/EULAR criteria and evaluate whether the 2023 ACR/EULAR criteria provide added value over the revised Sapporo criteria. METHODS: In this descriptive study, 94 aPL-positive patients (with or without APS diagnosis) were identified from two hospital-based registries (Gazi and Hacettepe University). Patients were classified into four groups to compare both criteria sets. These four groups are as follows: (1) patients classified with only the revised Sapporo criteria; (2) patients classified with only the 2023 ACR/EULAR APS criteria; (3) patients classified with both two criteria sets; and (4) patients classified with neither two criteria set. RESULTS: Of the 94 patients, 11 were classified with only the revised Sapporo criteria; one with only the 2023 ACR/EULAR APS criteria; 52 with both criteria sets; and 30 with neither set of criteria. For these 94 patients, the operating characteristics of the 2023 ACR/EULAR APS criteria, using the revised Sapporo criteria as the gold standard, the 2023 ACR/EULAR APS entry criteria demonstrated 100% sensitivity, and the 2023 ACR/EULAR APS classification criteria demonstrated 98% specificity and 82.5% sensitivity. CONCLUSION: The study emphasizes the importance of recognizing differences in clinical manifestations, such as early pregnancy loss without severe preeclampsia (PEC) and/or severe placental insufficiency (PI) and calls for a nuanced discussion on anticardiolipin (aCL) and anti-beta 2-glycoprotein-I (anti-ß2GPI) immunoglobulin G (IgG) cutoff values.

Coronavirus Disease 2019 Outcomes in Amyloid A Protein Amyloidosis Secondary to Rheumatic Conditions and Signs of Post- Coronavirus Disease 2019 Proteinuria Progression.

BACKGROUND: We aimed to investigate coronavirus diease 2019 (COVID-19) outcomes in patients with amyloid A protein (AA) amyloidosis secondary to rheumatic diseases and discuss factors associated with disease course. METHODS: A retrospective cohort was formed from adult patients with a diagnosis of AA amyloidosis. In patients with a positive severe acute respiratory syndrome coronavirus 2 polymerase chain reaction (PCR) test, rates of hospitalization, intensive care unit admission and mortality due to COVID-19 were collected from medical records. Data regarding to demographics, comorbidities, laboratory tests, medical treatments, adherence to previous treatments during COVID-19 and treatment administered for COVID-19 were collected from hospital databases and patient reviews. RESULTS: In 96 patients with AA amyloidosis, 16 had COVID-19 with a positive PCR. Ten (62.5%) patients were hospitalized, 2 (12.5%) were admitted to ICU, 1 (6.25%) was died. Hospitalized patients tended to be older. Comorbidities seemed to be more frequent in hospitalized patients. None of the patients had rapid progression to end-stage renal disease post-COVID-19. Seven patients had pre-COVID-19 and post-COVID-19 proteinuria levels. Three had notable increase in proteinuria after COVID-19 in 2 of which amyloidosis treatment was revised accordingly. CONCLUSION: Despite high rates of hospitalization in AA amyloidosis patients, mortality was observed only in 1 patient. Progression of proteinuria requiring treatment adjustment may be an issue in these patients. Cite this article as: Güven SC, Erden A, Küçük H, et al. Coronavirus disease 2019 outcomes in amyloid A protein amyloidosis secondary to rheumatic conditions and signs of post-coronavirus disease 2019 proteinuria progression. Eur J Rheumatol. Published online April 4, 2024. DOI:10.5152/eurjrheum.2024.23050.

Calcineurin inhibitors experience in unplanned pregnancies with active lupus disease: A retrospective observational study.

OBJECTIVES: To evaluate the use of calcineurin inhibitors (CNIs), specifically tacrolimus, in unplanned pregnancies with active lupus disease among patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: The study includes data from pregnancies in women diagnosed with SLE at Gazi University Hospital in Ankara, Türkiye, between January 2010 and July 2022. The study categorized pregnancies into planned and unplanned groups based on lupus nephritis presence, emphasizing the need for inactive lupus disease for at least 6 months before attempting conception in planned pregnancies. The outcomes of pregnancies involving CNIs, particularly tacrolimus, were assessed. RESULTS: In our cohort comprising 632 SLE patients, 39 individuals reported 42 pregnancies. Among the 42 pregnancies, 14 have a history of lupus nephritis. We observed that 8 of 14 patients with a history of lupus nephritis had unplanned pregnancies. Three patients used cyclosporine and 2 used tacrolimus during their pregnancy; their pregnancies were completely healthy, and no lupus flare was observed during their pregnancies. The pregnancy of 2 patients who used azathioprine and 1 last patient who used no immunosuppressive treatment ended in abortion. CONCLUSION: This study reveals that tacrolimus can be effectively used in unplanned pregnancies with active lupus disease, providing favorable maternal and fetal outcomes. The findings emphasize the importance of considering CNIs, particularly tacrolimus, in the management of SLE pregnancies, even in cases of unplanned pregnancies with a history of lupus nephritis.

Unveiling cancer risk in ANCA-associated vasculitis: result from the Turkish Vasculitis Study Group (TRVaS).

To investigate cancer incidence in patients with ANCA-associated vasculitis (AAV), compare it with the age/sex-specific cancer risk of the Turkish population, and explore independent risk factors associated with cancer. This multicenter, incidence case-control study was conducted using the TRVaS registry. AAV patients without cancer history before AAV diagnosis were included. Demographic and AAV-related data of patients with and without an incident cancer were compared. Standardized cancer incidence rates were calculated using age-/sex-specific 2017 Turkish National Cancer Registry data for cancers (excluding non-melanoma skin cancers). Cox regression was performed to find factors related to incident cancers in AAV patients. Of 461 AAV patients (236 [51.2%] male), 19 had incident cancers after 2022.8 patient-years follow-up. Median (IQR) disease duration was 3.4 (5.5) years, and 58 (12.6%) patients died [7 with cancer and one without cancer (log-rank, p = 0.04)]. Cancer-diagnosed patients were older, mostly male, and more likely to have anti-PR3-ANCA positivity. The cumulative cyclophosphamide dose was similar in patients with and without cancer. Overall cancer risk in AAV was 2.1 (SIR) ((1.3-3.2), p = 0.004); lung and head-neck [primary target sites for AAV] cancers were the most common. In Cox regression, male sex and ≥ 60 years of age at AAV diagnosis were associated with increased cancer risk, while receiving rituximab was associated with decreased cancer risk. Cancer risk was 2.1 times higher in AAV patients than the age-/sex-specific cancer risk of the Turkish population population, despite a high rate of rituximab use and lower dose of cyclophosphamide doses. Vigilance in cancer screening for AAV patients covering lung, genitourinary, and head-neck regions, particularly in males and the elderly, is vital.

Colchicine-intolerant familial mediterranean fever patients: A comparative study between different colchicine doses and IL-1 inhibitor monotherapy.

OBJECTIVE: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Colchicine is the primary treatment for FMF, although some patients do not respond well or are unable to tolerate it. For these patients, the addition of interleukin-1 (IL-1) antagonists is the preferred option. However, the impact of colchicine treatment alongside the use of IL-1 antagonists remains unclear. METHODS: We recruited adult FMF patients who satisfied the Eurofever and Pediatric Rheumatology International Trials Organization classification criteria and were receiving IL-1 antagonist treatment from our FMF cohort. All the patients exhibited colchicine intolerance or resistance. As per the FMF cohort protocol, the patients were longitudinally followed up, including assessments of their C-reactive protein, erythrocyte sedimentation rate, autoinflammatory disease activity index (AIDAI), and autoinflammatory damage index (ADDI). RESULTS: Among the 125 patients (68 female and 57 male), 96 received a combination of IL-1 antagonists and the maximum tolerated dose of colchicine, whereas 29 were treated exclusively with IL-1 antagonists due to colchicine intolerance. The patients' inflammatory markers, AIDAI activity, and ADDI damage scores did not differ significantly between the two groups during the follow-up period. Notably, the drug retention rates were significantly higher in the patients treated solely with IL-1 antagonists. CONCLUSION: While the typical approach is to maintain colchicine treatment alongside the use of IL-1 antagonists, for patients who cannot tolerate effective colchicine doses, IL-1 antagonists alone may effectively control FMF disease activity.

Is compressed colchicine tablet superior to other colchicine preparations in patients with familial Mediterranean fever?

AIMS: The aim of our study is to evaluate the differences in effectiveness, dosage, and side effect profiles in the use of colchicine preparations and evaluate the superiority of compressed colchicine tablets in familial Mediterranean fever (FMF) patients with resistance or intolerance to coated colchicine tablets. MATERIALS AND METHODS: Patients who were diagnosed with FMF according to the Tel Hashomer criteria, aged 18 years and older, and switched from compressed colchicine to coated colchicine tablets in the rheumatology clinic of Gazi University were identified. The daily colchicine dose and FMF attack frequency before and after switching from coated colchicine tablets to compressed colchicine tablets were compared. RESULTS: The study included 43 female (72.9%) and 16 male patients (27.1%), and the mean age was 34.54 ± 8.3 years. The number of attacks per year was significantly reduced after switching to compressed colchicine tablets, and daily colchicine doses were lower after switching to compressed colchicine tablets (1.97 ± 0.23 vs 1.78 ± 0.39 mg, p < 0.001). CONCLUSION: Compressed colchicine tablets were shown to be superior to other colchicine preparations and compressed colchicine tablets to be a useful treatment option before initiating biological agents in patients who were unresponsive to coated colchicine.

Still's disease continuum from childhood to elderly: data from the international AIDA Network Still's disease registry.

OBJECTIVE: Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease. METHODS: Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease. RESULTS: A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments. CONCLUSIONS: Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages.

Could serum HMGB1 levels be a predictor associated with psoriatic arthritis?

Background/aim: Psoriasis is a chronic autoimmune disease that predominantly affects the skin and musculoskeletal system. We hypothesized that HMGB1, an inflammatory nuclear protein, may play a role in the musculoskeletal involvement of psoriasis. Methods: Forty patients with psoriasis and 45 with psoriatic arthritis were involved in the study; the results were compared with 22 healthy controls. Serum HMGB1 levels were evaluated from peripheral blood samples. Results: Serum HMGB1 levels were found to be significantly higher in patients with psoriasis regardless of joint involvement (p < 0.001). Also, HMGB1 levels were correlated with the extent of psoriasis. Conclusion: Serum HMGB1 levels may contribute to the progression of psoriasis to psoriatic arthritis and correlate with the severity of skin involvement.

Psoriasis is an autoimmune skin disease that may also affect the joints. Factors leading to the progression of psoriasis to psoriatic arthritis are still a mystery despite an increasing number of animal studies and real-life data. HMGB1 is a nuclear protein that leads to an increase in molecules that increase inflammation (TNF-α, IL-1 and IL-6) in the body. Until now, there was no report about the relationship between psoriatic arthritis and serum HMGB1 levels. Our study aimed to find any difference in HMGB1 levels between healthy and psoriatic patients. Psoriatic arthritis patients had higher levels of serum HMGB1 than patients with psoriasis. Also, HMGB1 levels were correlated with the severity of skin involvement. Our results showed that serum HMGB1 may indicate a high risk for developing psoriasis that involves the joints. Therefore the HMGB1 level in psoriasis patients can potentially serve as a predictor associated with disease severity and the risk of developing psoriatic arthritis.

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.

Diffuse generalized venulitis as the primary pathology of Behçet's disease: A comprehensive magnetic resonance venography study.

OBJECTIVE: Behçet's Disease (BD) is a chronic multisystem vasculitis that manifests with destructive inflammation affecting the eyes, central nervous system, and blood vessels. The pathology of vein involvement in BD is poorly characterized. Magnetic resonance (MR) venography gives more comprehensive information about deep veins and adjacent tissues. In this study, we aimed to characterize vein involvement and evaluate the diagnostic utility of MR venography in BD. METHODS: Sixty-five BD patients who fulfilled the International Study Group (ISG) criteria and 20 healthy control subjects were enrolled. Inferior vena cava (IVC), common iliac veins (CIV), external (EIV) and internal iliac veins (IVV), common femoral veins (CFV), femoral veins (FV), and greater saphenous veins (GSV) of BD patients and healthy controls were evaluated with MR venography and ultrasonography for the presence pathologic features, luminal thrombi, vessel wall changes, and perivascular abnormalities. RESULTS: 33 vascular and 32 non-vascular BD patients (mean age 39.3 ± 11.3 years and 48 [73.8%] male) were enrolled. MR venography revealed diffuse concentric thickening of the walls of IVC, CIV, EIV, IIV, CFV, FV, and GSV in BD (healthy controls vs. BD p<0.05 for all vein segments). MR venography provided additional information about veins and perivascular tissues like contrast enhancement, enlarged lymph nodes, and seminal vesicle vascularization, which were remarkably more frequent in vascular BD than non-vascular BD and healthy controls. CONCLUSION: The results of our study suggest that the involvement of the venous system is diffuse and generalized in BD, and demonstration of venulitis might help diagnose the disease.

Investigation of the value of hematological biomarkers in the clinical differential diagnosis of IgG4-RD.

BACKGROUND: IgG4- related disease (IgG4- RD) is a systemic fibroinflammatory disease whose pathogenesis has not been completely elucidated. Due to the novelty and complexity of the diagnostic criteria, it is difficult to distinguish from the diseases included in the differential diagnosis without tissue biopsy. This study aimed to discover new biomarkers that can help for disease diagnosis and its differential diagnosis by reviewing the relationships between neutrophil-lymphocyte ratio (NLR), platelet lymphocyte ratio (PLR), systemic immune-inflammation index (SII), and systemic inflammation response index (SIRI). METHODS: Thirty IgG4- RD, 38 granulomatous polyangiitis (GPA), and 46 sarcoidosis patients presenting to the Rheumatology Clinic meeting the criteria of 2019 American College of Rheumatology, 2012 International Chapel Hill and 1999 American Thoracic Society meeting, respectively, and 27 healthy control subjects were included. We collected data on complete blood count with automated differential values including NLR, PLR, SII, and SIRI. RESULTS: The SII and PLR values were significantly higher in patients with IgG4-RD compared to healthy controls, (SII median (minmax) 572 (102-5583) vs. 434 (172-897), PLR median (min-max) 130 (56.8-546) vs. 104 (57.5- 253) p < 0.001). SII value was found to have a significant positive correlation with CRP in IgG4-RD disease (r = 0.371; p = 0.043). While SII, SIRI, NLR, PLR parameters were not significant between the IgG4-RD and sarcoidosis groups, SII, SIRI, NLR, PLR were significantly higher in patients with GPA than in IgG4-RD patients (p < 0.001). DISCUSSION: This is the first study to review the SII, SIRI, NLR, and PLR in IgG4-RD. The obtained results suggest that the SII could beused as a new tool, for differential diagnosis and activity of the IgG4-RD.

The predictors of paradoxical reactions, especially psoriasis, to biologic therapy-findings from the TReasure database: a 5-year follow-up study.

OBJECTIVES: The objectives of this study were to assess the clinical characteristics, predictive factors, and practical algorithms of paradoxical reactions (PRs), specifically paradoxical psoriasis (PP). METHODS: The TReasure database is a web-based prospective observational cohort comprised of patients with RA and SpA from 17 centres around Turkey since 2017. A cohort study and a case-control study nestled within the cohort were identified. RESULTS: In total, 2867 RA and 5316 SpA patients were evaluated. The first biologic agent was found to have caused PRs in 60% of the 136 patients (1.66%) who developed the PRs. The median time interval between the PRs and biological onset was 12 months (range 1-132 months, mean 21 months). The most common types of PP, constituting 92.6% of PRs, were pustular (60.3%) and palmoplantar (30.9%). Adalimumab (30.9%), infliximab (19%) and etanercept (17.4%) were the most common agents causing the PP. In the treatment of most PP patients (73.2%), switching biologic agents was favoured, with TNF inhibitor (TNFi) chosen in 46.03% and non-TNFi in 26.9% of cases. The three most frequently selected drugs were etanercept (24.6%), secukinumab (9.5%) and adalimumab (8.7%). Only 5.17% of patients who switched to another TNFi showed progression. The odds ratios (s) for SSZ, HCQ, and LEF use were significantly higher in RA controls than in PP patients (P = 0.033, OR = 0.15; P = 0.012, OR = 0.15; and P = 0.015, OR = 0.13, respectively). In the PP group with SpA, the number of smokers was significantly higher (P = 0.003, OR: 2.0, 95% CI: 1.05, 3.81). CONCLUSION: Contrary to expectations based on earlier research suggesting that paradoxical reactions develop with the class effect of biological agents, the response of patients who were shifted to another TNFi was favourable.

Effects of interleukin-1 antagonists on de novo and pre-existing damage in patients with familial Mediterranean fever.

OBJECTIVES: Colchicine is the mainstay of familial Mediterranean fever treatment and interleukin (IL-1) antagonists are the treatment of choice in resistant patients. We aimed to investigate efficacy of IL-1 antagonists in the prevention of damage, as well as the causes of treatment failure. METHODS: A total of 111 patients fulfilling Euro fever and Tel-Hashomer criteria and treated with IL-1 antagonists were included in the study. Patients were grouped according to their recent damage status: no damage, pre-existing damage and de novo damage that developed under IL-1 antagonist treatment. The degree of damage was determined using the Auto Inflammatory Disease Damage Index (ADDI). Total damage score was calculated separately as its original definition and with excluding chronic musculoskeletal pain, creating the modified ADDI (mADDI). RESULTS: Forty-six patients (43,2 %) had damage according to the mADDI. Damage was commonly observed at musculoskeletal, renal and reproductive domains. Median duration of treatment was forty-five months. Two patients developed de novo damage: one musculoskeletal and one reproductive in this time-period. Five patients had a worsening of their damage while using IL-1 antagonists. De novo damage with IL-1 antagonist treatment was associated with acute phase protein levels. CONCLUSIONS: We evaluated change in damage accrual while using IL-1 antagonists in patients with FMF. Physicians should pay attention to controlling inflammation to prevent further damage, especially in those with pre-existing damage.

Lacrimal gland ultrasonography and elastography as a diagnostic and activity tool for primary Sjögren's syndrome.

OBJECTIVE: To investigate the effectiveness of 2-dimensional shear wave elastography (2D-SWE) in the assessment of lacrimal gland involvement in primary Sjögren's syndrome (pSS) and to determine the association between ultrasonographic findings and clinical activity measures. METHOD: Forty-six patients who fulfilled the 2016 American College of Rheumatology/European League Against Rheumatism (EULAR) classification criteria of pSS and 23 age and gender-matched healthy control subjects were enrolled. Clinical, laboratory and labial biopsy histopathologic characteristics of patients were recorded. Disease activity of pSS and severity of ocular dryness were evaluated with EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) and Ocular Surface Disease Index (OSDI), respectively. Parotid and lacrimal gland architectures were assessed by B-mode ultrasound and 2D-SWE techniques. RESULTS: Mean shear wave elastography measurements, reflecting loss of elasticity, were remarkably higher in pSS patients compared to healthy subjects both in the lacrimal and parotid glands (8.99 ± 3.45 vs 3.68 ± 1.76 in lacrimal glands and 14.14 ± 4.39 vs 7.83 ± 1.69 in parotid glands, all P < 0.001). Shear wave elasticity of lacrimal glands was correlated with OSDI and ESSPRI scores (r = 0.69; P = 0.001 and r = 0.58; P = 0.001, respectively). A cut-off value of 4.6 kPa in the lacrimal gland elasticity discriminated pSS patients from healthy subjects with a sensitivity of 94% and specificity of 87%. CONCLUSION: Results of our study suggest that lacrimal glands lose elasticity in patients with pSS and the assessment of elasticity with 2D-SWE might help to classify patients as having pSS. Further studies are needed to validate the diagnostic utility of lacrimal 2D-SWE by including diseases other than pSS.

Coexistence of 3q29 microdeletion syndrome and Takayasu arteritis: A case report.

Takayasu arteritis (TAK) is a rare type of large and medial vessel systemic vasculitis. A variety of factors are thought to play a role in the occurrence and development of TAK such as human leukocyte antigen-B52, autoimmunity, inflammation and environmental factors. 3q29 microdeletion syndrome is also a very rare inherited disease, which includes intellectual disability, growth retardation and neuropsychiatric disorders. Here, we present a case with concomitant TAK and 3q29 microdeletion syndrome. A 22-year-old woman presented to the emergency department with sudden bilateral vision loss and severe headache. During physical examination, the patient was noted to have a difference in blood pressure between extremities. Computed tomography angiography revealed vascular wall inflammation in the abdominal aorta. Based on clinical and radiographical findings, a diagnosis of TAK was made. Concurrently, the patient was found to have short stature and intellectual disability. A possible genetic etiology was sought out. Chromosome analysis showed a 1.5 Mb heterozygous deletion on chromosome 3 and a diagnosis of 3q29 microdeletion was made. Additional imaging also revealed a split cord in medulla spinalis along with hemivertebrae and fusion anomalies, neither of which were reported in TAK or 3q29 microdeletion cases in the literature.

Dual Biologic Therapy in Patients with Familial Mediterranean Fever and Spondyloarthritis: Case-Based Review.

Patients with familial Mediterranean fever and spondylitis often fail to respond to conventional and biologic therapies. Achieving remission in these patients usually requires conventional and biologic treatment combinations. Combination of biologic agents may be a promising option for patients with familial Mediterranean fever and spondylitis who have refractory disease. Until recently, limited evidence existed regarding the efficacy and safety of this treatment strategy. To address this, our report presented a case series of 4 patients with familial Mediterranean fever and spondylitis who were resistant to standard treatments and in whom remission is achieved only with dual biologic therapy. The authors also conducted a literature search for studies that reported dual biological therapy in inflammatory diseases.

COVID-19 in familial Mediterranean fever: Clinical course and complications related to primary disease.

OBJECTIVES: To evaluate the impact of familial Mediterranean fever (FMF) features on the clinical course and outcomes of coronavirus disease 2019 (COVID-19) and clinical course of FMF after COVID-19. METHODS: Consecutive FMF patients with COVID-19 were enrolled from three referral hospitals. Clinical features of FMF and detailed COVID-19 information were obtained from patient interviews and medical records. RESULTS: Seventy-three FMF patients were included in the study. 94.5% of patients had clinical symptoms of COVID-19. We found 24.7% hospitalization, 12.3% respiratory support, 4.1% intensive care unit admission, 6.8% complication, and 1.4% mortality rate in patients. The risk factors of hospitalization for respiratory support were male gender [OR: 7.167 (95% CI: 1.368-37.535)], greater age [OR: 1.067 (95% CI: 1.016-1.121)], and non-adherence to colchicine treatment before the infection [OR: 7.5 (95% CI: 1.348-41.722)]. One-third of patients had reported attacks after COVID-19. The patterns of triggered attacks were fever, peritonitis, pleuritis, transient arthritis, chronic knee mono-arthritis, and protracted febrile myalgia. CONCLUSIONS: FMF characteristics were not associated with worse outcomes of COVID-19. Colchicine non-adherence was the risk factor of hospitalization for oxygen support. The rate of FMF attacks after COVID-19 is prominently increased, with some of them being protracted and destructive.