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Introduction: We aimed to evaluate the characteristics of the patients with a rheumatologic disease who underwent TPE., Method: A single-center, retrospective study was conducted between January 2016 and June 2023., Results: Twenty patients with a median age of 51 years received a median of 6 TPE sessions. Concurrently, immunosuppressive therapy was administered to 18 (90%) of them. During the follow-up period, 9 patients (45%) died. Creatinine (p = 0.001), C-reactive protein (p = 0.001), sedimentation rate (p = 0.002), leukocyte (p = 0.003), thrombocyte (p = 0.003), and neutrophil (p = 0.003) counts was decreased after TPE. Similarly, in the ROC analysis of post TPE laboratory parameters, urea, creatinine, CRP, hemoglobin, platelets, and lymphocytes predicted mortality with areas under the curve values ranging from 0.747 to 0.869. In the Cox regression analysis for mortality, creatinine was predictive for mortality (p = 0.030), HR 1.59 (95% CI: 1.05-2.41)., Conclusion: In rheumatologic conditions, TPE is beneficial to fill the gap until the effects of immunosuppressants become apparent., (© 2024 International Society for Apheresis and Japanese Society for Apheresis.)

Background: Idiopathic granulomatous mastitis (IGM) is a benign and rare chronic inflammatory disease of the breast. Although there are various treatment modalities, an ideal treatment algorithm has not been defined., Aims: We designed this study to evaluate the clinical status, duration of remission, remission rates, and treatment algorithm in patients with IGM during a 5-year follow-up period after immunosuppressive therapy., Methods: This study was planned retrospectively in the rheumatology outpatient clinic including 63 patients with biopsy-proven IGM. Demographic characteristics, clinical findings, treatment options, and drug-free remission periods after treatment were obtained from the patient's records., Results: The mean age of female patients with IGM was 36.4 ± 6.1 years. Remission was achieved in all patients receiving immunosuppressive treatment and the median remission period was 13.9 months. After 5 years of follow-up, the median remission time without medication was 46.1 months. There was a significant improvement in the laboratory parameters and clinical findings of the patients. The most preferred immunosuppressive agent in all patients was methotrexate, and the second was azathioprine., Conclusion: During the 5-year follow-up period, no recurrence after immunosuppressive therapy was detected in IGM patients. As seen in the treatment management chart, methotrexate provided remission in the majority of patients., Competing Interests: Declarations. Ethics approval: This study was granted approval by the Ethics Committee of Health Sciences University, Gulhane Training and Research Hospital within the framework of the Declaration of Helsinki (Date: 13.09.2023 and Decision number: 2023/201). Consent to participate: Informed consent (verbal due to nature of the study) has been obtained from all participants. Consent for publication: Not applicable. Conflict of interest: The authors declare no competing interests., (© 2024. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Aim: Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infection may have a more severe course in patients with underlying disease or who have had immunosuppression. In this study, it was aimed to determine the frequency of coronavirus disease 2019 (COVID-19) and the mortality rates related to COVID-19 among patients with rheumatic disease., Methods: The patients who were followed up with rheumatic disease in the rheumatology outpatient clinic in a tertiary hospital were retrospectively assessed if they had COVID-19 infection or not between March 2020 and January 2022., Results: A total of 10 682 patients were evaluated. There were 2928 (27.4%) COVID-19-positive and 7754 (72.6%) COVID-19-negative patients. The mean age of COVID-19-positive patients was 46.2 ± 14.6 years, and 65.8% were female. Forty-two (1.4%) patients died due to COVID-19. Among COVID-19-negative patients, 192 patients died. The most common rheumatic disease among patients with COVID-19 was spondyloarthritis (SpA) (30.4%). Corticosteroids were the most common treatment agent in COVID-19-positive patients regardless of mortality. Thirty-one (73.8%) patients were receiving corticosteroids, and 35 (83.3%) patients were receiving immunosuppressive agents among patients with mortality. According to the logistic regression analysis, older age, male gender, and receiving corticosteroid, hydroxychloroquine, mycophenolate mofetil, tofacitinib, rituximab, and cyclophosphamide were found to be related to increased mortality., Conclusion: COVID-19 is a serious infection and the current study emphasized that patients with rheumatic diseases had increased mortality rates, particularly in patients who were old, male, and on immunosuppressive treatments., (© 2024 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objectives: Behçet's disease (BD) is a multisystem disease and frequently occurs during the second-fourth decades of life, although disease onset may be seen at any age. This study aimed to analyze the influence of the age of onset on clinical manifestations of BD., Materials and Methods: This retrospective study analyzed two cohorts (paediatric and adult) to determine the association between the age of onset and clinical features in BD. Patients were classified into four groups to analyze the clinical characteristics according to the age of fulfilling the BD diagnostic criteria as follows: childhood onset (<12 years), adolescent onset (13-17 years), adult onset (18-39 years), and late onset (>40 years)., Results: The study included 801 patients with BD. Male predominance, pathergy test positivity, aphthosis (oral or genital), and skin and ocular involvements were higher among adult patients than paediatric patients. The presence of positive family history for BD, neuro-BD, and epididymitis were observed significantly common in the paediatric group., Conclusion: There may be differences in clinical manifestations with regard to the age of disease onset. Disease presentations may differ from adult patients, and clinicians should be aware of the high familial aggregation rate of BD, especially in countries where the disease is endemic., (© Japan College of Rheumatology 2023. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state which is characterized by seizures, headache, visual disturbances, paresis, and altered mental status. Golimumab is anti-tumor necrosis factor-α inhibitor (anti-TNF-α) that can be used in the treatment of rheumatologic diseases. Here, we present a patient who had developed PRES after golimumab treatment for ankylosing spondylitis (AS). A 45-year-old female patient was admitted to the emergency service with a newly onset severe headache, loss of vision in both eyes, and two generalized tonic-clonic seizures that lasted for 3 to 4 min. The patient had the diagnoses of AS for 12 years and hypertension for 3 years and receiving golimumab and carvedilol. The patient was diagnosed with PRES based on the current clinical and diffusion cranial magnetic resonance imaging (MRI) findings. On suspicion of being the trigger of this situation, golimumab was stopped. After starting anti-convulsant therapy and controlling blood pressure, the neurological findings recovered rapidly and no seizures were seen. Control MRI images, in the first month's visit, were normal. Although chemotherapeutic agents are well-known causes of PRES, there are few reported cases with anti-TNF-α agents in the literature. To our knowledge, this is the first case that developed PRES after golimumab. Demyelinating diseases are the most frightening neurologic complication of anti-TNF-α treatment; however, PRES should come to mind in patients presenting with neurological symptoms., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Background: Idiopathic granulomatous mastitis (IGM) is a rare inflammatory breast disease, in which there is no clear established treatment algorithm. Several physicians keep away from using immunosuppressive (IS) treatments in routine clinical practice., Aims: This study aimed to evaluate the rates of drug-free remission of the patients with IGM in a period of 3-year follow-up., Methods: This retrospective study conducted with 55 biopsy-proven IGM patients, who were followed up between February, 2011, and November, 2021, in rheumatology outpatient clinic of Gulhane Training and Research Hospital. The demographic and clinical characteristics of the patients were obtained from patients' files. The 3-year follow-up data were assessed for long-term outcome analyses., Results: There were 55 female patients with a mean age of 36.8 ± 6.3 years. Fifty-four (98.1%) patients were in drug-free remission at the end of 3 years. The median duration of drug-free remission in patients receiving methotrexate (MTX), only corticosteroid (CS), and azathioprine was 19.7, 32.9, and 14.7 months, respectively. The drug-free remission duration for the patient who received cyclosporine A as IS was 28.3 months. The median duration of IS treatment was 15.8 months, and the median duration of treatment with CS and other IS combination was 6.7 months. Recurrence was observed in 4 (80%) patients without IS therapy after surgery, of whom MTX was used in 3 (75%) patients and achieved remission., Conclusions: IS agents provide high rate of prolonged drug-free remission and should be considered a part of routine medical care of the patients with IGM., (© 2023. The Author(s), under exclusive licence to Royal Academy of Medicine in Ireland.)

Objectives: The study aimed to identify the interactions among treatment protocols and oral ulcer activity related factors in patients with Behçet's syndrome (BS) using the Classification and Regression Tree (CART) algorithm., Methods: In this cross-sectional study, 979 patients with BS were included from16 centres in Turkey, Jordan, Brazil and the United Kingdom. In the CART algorithm, activities of oral ulcer (active vs. inactive), genital ulcer (active vs. inactive), cutaneous involvement (active vs. inactive), musculoskeletal involvement (active vs. inactive), gender (male vs. female), disease severity (mucocutaneous and musculoskeletal involvement vs. major organ involvement), smoking habits (current smoker vs. non-smoker), tooth brushing habits (irregular vs. regular), were input variables. The treatment protocols regarding immunosuppressive (IS) or non-IS medications were the target variable used to split from parent nodes to purer child nodes in the study., Results: In mucocutaneous and musculoskeletal involvement (n=538), the ratio of IS use was higher in patients with irregular toothbrushing (ITB) habits (27.1%) than in patients with regular toothbrushing (RTB) habits (14.2%) in oral ulcer activity. In major organ involvement (n=441), male patients with ITB habits were more likely treated with IS medications compared to those with RTB habits (91.6% vs. 77.6%, respectively)., Conclusions: Male BS patients on IS who have major organ involvement and oral ulcer activity with mucocutaneous and musculoskeletal involvement have irregular toothbrushing habits. Improved oral hygiene practices should be considered to be an integral part for implementing patient empowerment strategies for BS.

Objectives: Smoking has a well-established detrimental effect on the prognosis and treatment success in patients with ankylosing spondylitis. It is important to encourage and convince patients to quit smoking. We aimed to evaluate the contribution of rheumatologists to smoking cessation in patients with ankylosing spondylitis., Methods: This single-center cross-sectional study was conducted in a tertiary research hospital between March 2022 and June 2022. The data related to demographics, smoking status, duration of smoking, average number of cigarettes smoked per day, reasons for quitting smoking, and methods of quitting smoking were obtained through face-to-face interviews., Results: This study was carried out on 308 patients. A total of 102 ankylosing spondylitis patients quit smoking. Of the patients who quit smoking, 39 (38.3%) patients quit smoking with a recommendation of a rheumatologist and 29 (28.4%) patients quit because of their concerns related to ankylosing spondylitis disease. The most commonly used methods for quitting smoking were herbal supplements in 40 (39.2%) patients and medication for smoking cessation in 40 (39.2%) patients., Conclusions: It has been shown that about one-fifth of ankylosing spondylitis patients are not questioned by a rheumatologist about smoking. On the other hand, it was seen that the factor with the greatest effect on those who quit smoking was the rheumatologist. Therefore, rheumatologists should question all ankylosing spondylitis patients about smoking and encourage smokers to quit in order to achieve better outcomes in the long term., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Kaya et al.)

Objectives: Rheumatoid arthritis associated interstitial lung disease (RA-ILD) is a major concern in RA. These patients have been included in clinical trials and in the post-marketing setting of RA patients using tofacitinib. We aimed to assess the real-life efficacy and safety of tofacitinib in patients with RA-ILD., Methods: RA patients with ILD diagnosis based on the HRCT images of the lungs from eight different centres recruited to study. As a control group, RA patients without ILD under tofacitinib were included. Demographic data, patients' characteristics, available pulmonary function tests regarding RA and RA-ILD at the visit in which tofacitinib was initiated and for the last follow-up visit under tofacitinib were recorded. Reasons for tofacitinib discontinuation were also recorded. Drug retention rates were compared by log-rank test. p-value <0.05 was considered statistically significant., Results: A total of 47(42.6% male) RA patients with RA-ILD and a control group of 387 (17.8% male) patients without RA-ILD were included in analysis. After the median of 12 (9-19) months follow-up, mean FEV1%; 82.1 vs. 82.8 (pre/post-treatment, respectively, p=0.08), mean FVC%; 79.8 vs. 82.8 (pre/post-treatment, respectively, p=0.014) were stable and worsening was observed in 2/18 (11.1%) patients. Retention rates were similar (p=0.21, log-rank). In RA-ILD group, most common cause of drug discontinuation was infections (6.3 vs. 2.4 per 100 patient-years)., Conclusions: Treatment strategy of RA-ILD patients is still based on small observational studies. A high rate of discontinuation due to infections was observed in RA-ILD patients under tofacitinib; however, RA-ILD patients were older than RA patients without ILD.

Objectives: The present study aims to evaluate the relationship between Behçet's uveitis and lymphangiogenesis by determining levels of Vascular endothelial growth factor-C (VEGF-C, its receptors sVEGFR-2, sVEGFR-3 and lymphangiogenesis markers podoplanin (PDPN) and lymphatic vessel endothelial hyaluronan receptor 1(LYVE-1), and C-type lectin domain family 1 member B (CLEC2)., Materials and Methods: 55 patients with BD uveitis and 31 healthy control subjects were enrolled in the study., Results: sVEGFR-2, sVEGFR-3, VEGF-C/sVEGFR-2 ratio, PDPN and LYVE-1 levels were higher in the patient group. A positive correlation was found between LYVE-1 and hsCRP levels. PDPN had a strong predictive value for progression with a cut-off value of 2 pg/mL, with 69% sensitivity and 68% specificity (p = 0.001)., Conclusion: LYVE-1 and PDPN can be good representatives of the ongoing inflammatory processes in BD uveitis and point out that the disease can be related to lymphangiogenesis.

Background/aim: Familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease that requires lifelong colchicine treatment. Colchicine is the mainstay of the treatment, which decreases the frequency and the severity of recurrent FMF attacks and prevents the development of amyloidosis. This study aimed to investigate the rates of colchicine treatment adherence in patients with FMF and the factors related to treatment non-adherence., Materials and Methods: This observational study was conducted with 179 patients with FMF between November 2018 and April 2019 in a tertiary rheumatology outpatient clinic. The sociodemographic and clinical data were recorded. Compliance Questionnaire on Rheumatology (CQR) was used to assess the treatment adherence and the Beliefs About Medicines Questionnaire (BMQ-T) was used to assess a patient’s beliefs about colchicine. The factors associated with adherence to the treatment were evaluated., Results: The study included 113 male (63.1%) and 66 (36.9%) female patients with a mean age of 30 (25–44) years. The rate of the patients who declared regular colchicine usage was 66.5%. The frequency of non-adherent patients was 83.8% according to CQR. Treatment adherence was better in patients with comorbid diseases than those without (41.4% vs. 22%, respectively, p = 0.028). The frequency of married patients in the adherent group (72.4%) was higher than the non-adherent group (47.3%) (p = 0.013). The colchicine dose used in the adherent group was 1.5 (1.3–1.8) mg/day, whereas it was 1.5 (1.0–1.5) mg/day in the non-adherent group (p = 0.033). The adherence rate was rising with increasing scores of BMQ-T Specific Necessity. As the scores of BMQ-T General Overuse and General Harm increased, non-adherence to colchicine increased., Conclusion: Evaluating adherence to colchicine treatment with objective methods is crucial to ensure sufficient treatment and prevent amyloidosis. Determining beliefs about colchicine may increase patients’ adherence to treatment., Competing Interests: None declared., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Purpose: Spondyloarthritis (SpA) is a group of diseases with overlapping skeletal and extra-articular features. Acute anterior uveitis (AAU) is the most common extra-articular manifestation of SpA. The relation between AAU and SpA is well defined in the current literature. Our study aims to analyze the frequency and factors associated with AAU in different forms of SpA in a large nationwide cohort of Turkish SpA patients., Design: Retrospective cohort study., Methods: The data were obtained from the TReasure database, which compiles data from records of the web-based Rheumatoid Arthritis (RA) and SpA patients treated with biological disease-modifying anti-rheumatismal drugs from different regions of Turkey. The clinical characteristics of SpA and uveitis are recorded., Results: Data of the 4,297 SpA patients were included in the study. Overall, 475 of 4,297 patients (11.0%) had experienced 1 or more episodes of uveitis. SpA patients with older age (P < .001), a smoking history (P = .004), delayed diagnosis (P = .001), longer disease duration (P < .001), arthritis (P < .001), positive HLA-B27 (P < .001), a family history of SpA (P < .001), and radiographic damage (presence of sacroiliitis, syndesmophytes, bamboo spine, hip involvement) (P < .001 for all) more commonly had uveitis. On the other hand, uveitis was less prevalent in patients with psoriasis and psoriatic arthritis (P < .001 for both)., Conclusion: Uveitis may be the key feature leading to SpA diagnosis. Patients with radiographic damage and long disease duration have an increased risk for uveitis in both male and female SpA patients. Patients with uveitis should be referred to a rheumatologist for a thorough evaluation of SpA., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to assess the demographic and clinical characteristics of patients with adult-onset Still's disease (AOSD) under biological treatment., Patients and Methods: This retrospective cohort study included a total of 19 AOSD patients (13 males, 6 females; median age: 37 years; range, 28 to 52 years) who received biological drugs due to refractory disease between January 2008 and January 2020. The data of the patients were obtained from the patient files. The response to the treatment was evaluated based on clinical and laboratory assessments at third and sixth follow-up visits., Results: Interleukin (IL)-1 inhibitor was prescribed for 13 (68.4%) patients and IL-6 inhibitor prescribed for six (31.6%) patients. Seventeen (89.5%) patients experienced clinical remission., Conclusion: Biological drugs seem to be effective for AOSD patients who are resistant to conventional therapies. Due to the administration methods and the high costs of these drugs, however, tapering the treatment should be considered, after remission is achieved., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2022, Turkish League Against Rheumatism.)

Objective: Some studies have shown that the use of complementary and alternative medicine (CAM) is common in patients with chronic painful conditions, such as ankylosing spondylitis (AS). This study aimed to determine the prevalence and types of CAM usage in patients with AS and to evaluate the impact of treatment adherence and beliefs about medicines on CAM usage., Methods: This study has a descriptive design. A total of 140 patients with AS were included. The treatment adherence of the patients was evaluated using the Morisky Green Levine Medication Adherence Scale. The Beliefs about Medicines Questionnaire (BMQ-T) was used to assess patients' beliefs about medicines., Results: Previous or current CAM usage was stated by 40% of the patients. It has been found that CAM usage was significantly high (p<0.05) in patients who were married, older, and diagnosed at older ages. The difference between patients' beliefs about medicines and CAM usage was not statistically significant (p>0.05). The BMQ-T scores were significantly different in terms of the patients' treatment adherence (p<0.05)., Conclusion: This study showed that approximately half of the patients with AS were using 1 CAM method. Furthermore, medication adherence and patients' beliefs about medicines did not have any impact on CAM usage, but the patients' beliefs about medicines affected treatment adherence.

The aim of the present study was to examine the effects of age on mucocutaneous activity by using moderation analysis in Behçet's syndrome (BS). In this cross-sectional study, 887 BS patients (female : male, 481:406; mean age, 38.4 ± 10.9 years) followed in 13 tertiary centers in Turkey were included. Mucocutaneous activity was evaluated by using the Mucocutaneous Index (MI) according to sex and disease course. Moderation analysis was performed to test the effect of age on mucocutaneous activity. A moderator variable is a third variable and affects the relationship between independent and outcome variables. Age was chosen as a potential moderator variable (interaction effect), MI score as the outcome variable and sex as an independent variable in the analysis. The moderation analysis tested the effects of age in three steps: whole BS patient group, patients without systemic involvement and those with systemic involvement. The moderation model was only significant in BS patients with systemic involvement (P = 0.0351), and a significant relationship was observed between female sex and MI score (P = 0.0156). In addition, the interaction plot showed that female patients had increased MI scores compared with male patients, especially in the 28-year-old age group (P = 0.0067). Moreover, major organ involvement was newly diagnosed in the majority of these young female BS patients. Our results suggest that the relationship between sex and mucocutaneous activity was moderated by age in the systemic involvement group. Also, increased mucocutaneous activity may be associated with new major organ involvement in young female BS patients with systemic involvement., (© 2020 Japanese Dermatological Association.)

Background/aim: Idiopathic granulomatous mastitis is a rare, benign inflammatory disease of breast. There is no general agreement on the appropriate treatment choice. The aim of the study was to investigate the immunosuppressive administer for idiopathic granulomatous mastitis and risk factors related with disease recurrence., Materials and Methods: The data of 53 patients with idiopathic granulomatous mastitis were evaluated for this cross-sectional retrospective study. Demographic features and clinical characteristics and course of the patients were obtained from file records., Results: The mean age of the patients was 37.2 ± 6.6 years. Fifty-one of 53 patients received immunosuppressive treatment with or without surgery. Forty-seven (88.6%) of the patients received only immunosuppressive treatment without surgery, while 4 (7.54%) patients received immunosuppressive treatment after surgery. Forty-one (77.3%) of 47 patients who had no surgical resection received methotrexate as immunosuppressive treatment. The other 6 (11.3%) patients received azathioprine or corticosteroid treatment. Complete or partial remission was observed in 50 (98%) of 51 patients who received immunosuppressive treatment, while only 1 (2%) patient did not reach remission. No factors were found related with recurrence of disease., Conclusion: Methotrexate seems to be efficient in the treatment of idiopathic granulomatous mastitis and provides drug-free remission., Competing Interests: The authors declare that there is no conflict of interest., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS)., Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year., Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration <5 years (74%) were higher in male patients with work-day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work-day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work-day loss compared to others (8.6%) (P = .059)., Conclusion: Work-day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work-day loss., (© 2019 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Objectives: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS)., Methods: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity., Results: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05)., Conclusions: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.

There is no established treatment protocol for amyloid-A (AA) amyloidosis secondary to Familial Mediterranean Fever (FMF). Recently, we reported the efficacy of tocilizumab in 11 amyloidosis cases associated with FMF. In 2 patients of 11, we discontinued the tocilizumab administeration owing to the normalization of amyloidosis-related symptoms, but proteinuria re-occurred eventually. Fortunately, the patients responded to tocilizumab re-treatment. This led us to conclude that physicians should not stop the treatment, even in patients with normalized proteinuria levels.

Exogenous ochronosis is characterized by hyperpigmented skin lesions that arise in association with local suppression of homogentisic acid oxidase enzyme. Although it generally develops in association with topical application of chemical agents, it can occasionally develop in association with antimalarial drugs. Here we present the case of a patient with rheumatoid arthritis who developed hyperpigmentation on the face and neck regions during hydroxychloroquine treatment. Hydroxychloroquine is being widely used in rheumatology practice, and cutaneous hyperpigmentation may develop as an adverse effect. In the present case, we emphasize the potential underlying mechanisms through which it may cause cutaneous hyperpigmentation and determine the clinical and histopathological findings of exogenous ochronosis.