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Abstract : Ankylosing spondylitis is a chronic inflammatory disorder characterized by inflammation of the axial skeleton and sacroiliac joints and to a lesser extent by peripheral arthritis and the involvement of some extra-articular organs. It is paramount for the provision of effective health care delivery to be familiar with the epidemiologic studies on prevalence, mortality, and disability. Furthermore, there is no systematic arrangement of studies related to the treatment of ankylosing spondylitis in Korea. In this review, we addressed Korean ankylosing spondylitis epidemiological studies related to prevalence, genetic factor especially human leucocyte antigen-B27, extra-articular manifestations, infections, mortality, radiologic progression, child-birth, and quality of life. Furthermore, we reviewed Korean ankylosing spondylitis treatment researches about treatment trend, patients’ registration program called The KOrean College of Rheumatology BIOlogics and targeted therapy (KOBIO) registry project, biologics and biosimiliars, complications especially infections, and issues about bony progression. There would be value to further studying the epidemiology and treatment of Korean ankylosing spondylitis.

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Abstract : Systemic sclerosis (SSc), a rare, chronic progressive systemic autoimmune disease of unknown etiology, is characterized by autoimmunity, tissue fibrosis, and obliterative vasculopathy. SSc can affect all major organs including the skin, blood vessels, lung, heart, kidneys, and gastrointestinal tract. Our understanding of its pathogenesis has increased over the past few decades, leading to improved diagnosis and treatment. However, the mortality rate of SSc remains considerable, mainly due to cardiopulmonary causes. A growing body of evidence suggests that geographical, regional, and ethnic differences could affect the epidemiology, clinical characteristics and prognosis of SSc. Although Korean data of this issue are lacking, a considerable amount of research has been published by many Korean researchers. To establish treatment strategies for Korean patients, extensive Korean research data are needed. This review summarizes the prevalence, incidence, mortality, and clinical and laboratory manifestations of Korean patients with SSc and discusses the current trends in evidence-based treatment and recommendations.

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Abstract : Objective: This study aimed to assess the prevalence of pain in patients with RA in clinical remission and analyze the demographic and clinical characteristics of those who experienced persistent pain despite remission status.
Methods: Data from 1,891 patients with RA registered on the Korean College of Rheumatology Biologics and Targeted Therapy registry were obtained. Remission was defined as a Disease Activity Score of 28 joints-erythrocyte sedimentation rate (ESR) <2.6. Pain intensity was classified as severe (pain visual analog scale [VAS] ≥7), moderate (4≤VAS<7), or mild (VAS <4).
Results: Our analysis showed that 52.6% of patients complained of severe pain at the start of or during switching biological disease- modifying anti-rheumatic drugs (bDMARDs) or targeted synthetic DMARDs (tsDMARDs). Despite having a 36.0% (n=680) remission rate after the use of bDMARDs or tsDMARDs at their 1-year follow-up, 21.5% (n=146) of these patients had moderateto- severe pain, higher frequency of foot erosions, and comorbidities, such as mental illness, endocrine, renal, and neurological disorders, than patients with a milder degree of pain. The multivariable regression analysis showed that presence of foot erosions, neurological disorders, and use of corticosteroids were independently associated with moderate-to-severe pain in patients with RA despite being in remission. The level of ESR and use of Janus kinase inhibitors were inversely associated with moderate-to-severe pain.
Conclusion: Persistent pain and discomfort continue to be a problem for patients with RA in clinical remission. Continued research on insistent pain in patients with RA is warranted to better alleviate distress and improve the quality of life in patients.

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Abstract : Objective: To identify the predictive factors for renal response in patients with lupus nephritis (LN).
Methods: Patients and data were extracted from a prospective systemic lupus erythematosus cohort in Korea, in which clinical data were collected at 0, 3, 6, and 12 months after induction therapy. Treatment response of LN were evaluated as a complete response (CR), partial response (PR), or non-response (NR) at 3, 6, and 12 months, respectively. Predictive factors for CR at 6 months were evaluated using multivariable Poisson regression analysis.
Results: A total of 75 patients with LN who underwent biopsy was enrolled. The mean age at diagnosis of LN was 28.9±9.7 years, and 68 (90.7%) were female. The frequencies of classes III, IV, III+V, IV+V, and V were 20.0%, 44.0%, 16.0%, 12.0%, and 8.0%, respectively. Compared to relapsed LN, new-onset LN showed a lower percentage of glomerulosclerosis (45.5% vs. 76.2%, p=0.013). The overall proportions of CR, PR, and NR at 6 and 12 months were 52.0%, 26.7%, 21.3% and 50.7%, 24.0%, 25.3%, respectively. In multivariate analysis, age at enrollment (odds ratio [OR]=1.02, p=0.022), relapsed LN (OR=0.71, p=0.037), anti-Ro antibody (OR=0.67, p=0.014), and class III LN (OR=1.48, p=0.001) were associated with CR at 6 months.
Conclusion: In our prospective cohort, class III LN was a good predictive factor for CR at 6 months in patients with LN, whereas younger age, relapsed LN, and anti-Ro antibody were poor predictive factors.

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Abstract : Objective: To investigate the histopathological characteristics of patients with lupus nephritis in the 2018 revised International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification and assess the prognostic factors.
Methods: This study enrolled 92 patients with lupus nephritis, who had conventional treatment and renal biopsy. Each renal tissue was evaluated according to 2018 ISN/RPS classification, and quantified apoptotic regulator protein, the B-cell lymphoma-2 protein (Bcl-2), expressions in selected lymphocyte subsets were measured using novel computational approaches using multicolor confocal images. Histopathological characteristics and prognostic factors of end-stage renal disease (ESRD) and chronic kidney disease (CKD) were compared. Follow-up data were obtained, and survival analysis was conducted.
Results: During follow-up period (average: 74.3 months), 16 and 18 patients progressed ESRD and CKD, respectively. Multivariable analysis of age, sex, disease activity and pathological features in ISN/RPS demonstrated the extent of interstitial inflammation (grade 0~3) was significantly associated with both ESRD and CKD. When interstitial inflammation was divided into mild (grade 0, 1) and severe (grade 2, 3), Cox regression analysis showed that patients with severe interstitial inflammation were significantly increased risk of both ESRD and CKD (hazard ratio: 4.67 and 3.8, respectively). Bcl-2 expression in CD4+ and CD20 cells was significantly higher in the severe interstitial inflammation group compared to in mild interstitial inflammation patients (p=0.006 and 0.010, respectively).
Conclusion: The extent of interstitial inflammation can predict clinical renal outcomes. Significantly elevated Bcl-2 expression in both CD4+ and CD20 cells was found in severe interstitial inflammation compared with mild interstitial inflammation.

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Abstract : Objective: To investigate the clinical features and associated underlying conditions of isolated tuberculous myositis (ITBM), a rare extrapulmonary tuberculosis (TB).
Methods: A systematic literature search and a multicenter survey were performed using a triangulation strategy. Data from the identified ITBM cases were extracted and analyzed to determine the underlying conditions, clinical presentations, treatments, and outcomes.
Results: Based on the systematic review, we identified 58 ITBM, including 9 pediatric, cases in the literature published from 1981 to 2021: 25 (43.1%) immunocompromised and 33 (56.9%) non-immunocompromised patients. Immunocompromised cases had a significant shorter symptom duration (median 30.0 vs. 75.0 days) and a higher prevalence of multilocular involvement (20.8% vs. 0%). Among 24 immunocompromised adult patients, dermatomyositis/polymyositis (DM/PM; n=10, 41.7%) were the most common underlying diseases in adults with ITBM identified in the systematic review. Over the past 20 years, 11 Korean adults with ITBM were identified in the multicenter survey. Of 7 immunocompromised cases, two (28.6%) were DM/PM patients. TB death rate of immunocompromised patients was 0.0% and 5/23 (21.7%) in the pediatric and adult ITBM cases identified in the systematic review, respectively, and 3/7 (42.9%) in survey-identified ITBM cases.
Conclusion: ITBM has a unique clinical presentation including fever, tenderness, local swelling, overlying erythema, abscess formation and was associated with a grave outcome, especially in immunocompromised hosts. DM/PM was a highly prevalent underlying disease in both systematic review-identified and survey-identified immunocompromised ITBM patients.

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Abstract : Deficiency of adenosine deaminase 2 (DADA2) is an autoinflammatory disease caused by pathogenic variants of the ADA2 gene and has similar clinical features to polyarteritis nodosa (PAN). We, herein, report a case of DADA2 in Korea that was diagnosed in a patient with childhood-onset PAN. The patient had a truncal ataxia and facial palsy caused by thalamic infarction at 34 months of age. Livedo reticularis with Raynaud phenomenon and abdominal pain with fever were followed. Radiologic examination showed multiple infarctions in brain and kidney. She was diagnosed with PAN using skin biopsy and angiography. She had severe hemorrhagic strokes despite medical treatments. Her disease activity was controlled after adding a tumor necrosis factor-α inhibitor. Molecular analysis revealed compound heterozygous pathogenic variants of ADA2 gene. This is the first case of DADA2 in Korea. Genetic analysis for ADA2 gene should be considered in patients with childhood-onset PAN.