The Discrepancy of ANA and Compartment Bead Patterns Suggestive of a Neuropsychiatry Systemic Lupus Erythematosus (NPSLE).

Neuropsychiatric systemic lupus erythematosus (NPSLE) exhibits neurological and psychiatric manifestations in systemic lupus erythematosus (SLE) patients, which NPSLE diagnosis can be challenging for rheumatologists. An Indonesian female, 44 years old, complained of two times seizures with 10-min duration, which during seizures were stiff, eyes rolled up, foaming at the mouth, wet the bed, and fainting afterward. The patient also has a history of SLE and received cyclophosphamide therapy 5 years ago. Her clinical condition showed facial and lingual palsy, with central type on the right. Antinuclear antibody indirect immunofluorescence (ANA IF) positive using cytobead ANA with a homogenous pattern and cytoplasmic speckled titer 1/80. Confirmation beads showed positive of dsDNA only. ANA profile showed positive antinucleosome, antihistone, and AMA-M2, and also increased anticardiolipin antibody that supports the diagnosis of NPSLE. The difference in the pattern of ANA IF with confirmation beads suggests the presence of other autoantibodies in NPSLE.

The Increasing Level of DKK-1 as a New Bone Formation Factor in Patients with Early Spondyloarthritis.

The role of dickkopf-related protein 1 (DKK-1) in radiographic development may become a robust marker for early spondyloarthritis (SpA) diagnosis. This study aimed at determining the serum DKK-1 profile in patients with SpA and investigating its relationship with SpA progression. Supported by analyzing the BMD data which aims to affirm the potential of DKK-1 as a biomarker for early diagnosis of SpA, this research may become the early study to produce a robust tool to diminish the fatal impacts in SpA. This cross-sectional study included patients with SpA using ASAS 2010 criteria from Dr. Soetomo General Hospital, Indonesia. Collected data included patients' general characteristics, disease duration, disease activity using ASDAS-CRP and ASDAS-ESR, serum DKK-1 levels, and BMD. The patients were classified as early SpA if the disease duration was ≤5 years and established SpA if the disease duration was >5 years, while the low BMD was indicated by Z score ≤ -2.00. The correlation was tested using the Spearman or Pearson test. The differences in patients' characteristics among early and established SpA and also between low and normal BMD were tested using the unpaired T-test or the Mann-Whitney test. The serum DKK-1 levels in early SpA (7365 ± 2067 pg/dL) were significantly higher than those in established SpA (5360 ± 1054 pg/dL). Serum DKK-1 levels were also associated with disease duration (r = -0.370, p = 0.040) and BMD at the total hip (r = 0.467, p = 0.028). The differences in all patients' clinical parameters were not found between patients with low BMD at any site and patients with normal BMD unless in the BMI (p = 0.019). Our findings found DKK-1 as a potential diagnostic marker for early SpA. Early diagnosis may lead to rapid treatment to delay disease progression and prevent future impairment.

An Indonesian female with severe cutaneous lupus erythematosus: A case report and literature review.

INTRODUCTION: Cutaneous lupus erythematosus (CLE) is one of the most common symptoms in systemic lupus erythematosus (SLE) cases. SLE manifestations in the skin area seem to significantly affect the patient's quality of life, which is this condition for unmarried females. CASE PRESENTATION: An Indonesian female, 23 years old, complained of skin peeling on her scalp, upper and lower extremities. The condition of the wound was severe in the head area. A biopsy was performed, revealing pustular psoriasis. She received immunosuppressant agent and wound care over the lesion. The patient showed good improvement after 2 weeks of this treatment. DISCUSSION: Diagnosis of CLE through history taking, skin examination and histopathological findings. Since immunosuppressant agent is the primary therapy of CLE, monitoring is required because immunosuppressive drugs increase the risks of infection. The outcome of CLE treatment is to minimize complications and improve the patient's quality of life. CONCLUSION: CLE primarily affects women, therefore early management, monitoring and collaboration with other departments will improve the patient's quality of life and increase their compliance with medication.

The Association of Complements, TGF-ß, and IL-6 with Disease Activity, Renal Damage, and Hematological Activity in Patients with Naïve SLE.

Several key player factors, such as cytokine and complement, play an important role in the pathogenesis of systemic lupus erythematosus (SLE). The purpose of this study was to reveal the association between complement 3 (C3), complement 4 (C4), interleukin-6 (IL-6), and transforming growth factor-ß (TGF-ß) with SLE disease activity, renal damage, and hematological activity in patients with naïve SLE. The Laboratory of Clinical Pathology Dr. Soetomo General Hospital in Surabaya performed all laboratory examinations on thirty women with naïve SLE. The SLE diagnosis is based on ACR criteria (1998 revised criteria) from Dr. Soetomo General Hospital Surabaya, Indonesia, and the systemic lupus activity measurement (SLAM) score is used to assess the disease activity. The correlation was statistically tested using the Spearman and Pearson tests. The differences in cytokine and complement levels are between SLE severity groups using the two-way Anova and Kruskal-Wallis. The unpaired T-test and Mann-Whitney test were used to determine the differences between the relatively normal and the more severe groups of organ damage and hematological activity. All tests were two-tailed, analyzed with GraphPad Prism 9 for windows, and a p value of less than 0.05 was considered statistically significant. This study found a significant decrease in C3 (20.2, 16.4-24.2 mg/dL) and C4 (7, 6-14.3 mg/dL) and an increase in IL-6 (35.60 ± 7.43 mg/dL) and TGF-ß (311.1 ± 290.8 mg/dL) in the group of severe patients with SLAM scores >30. Although there is no significant relationship between SLAM and renal impairment or hematologic activity, patients with higher SLAM had a significant decrease in complement; this complement decrease was also significant in patients with higher leukocyte counts. An insignificant increase in cytokines was also observed in patients with higher SLAM. Patients with high serum creatinine levels had a significant increase in TGF-ß, whereas those with a faster ESR had a significant increase in IL-6. In conjunction with complements evaluation, assessment of the cytokine profile may become a promising marker for reliable diagnosis and treatment of SLE in the future.

The roles of Single Nucleotide Polymorphism (SNP) Endoplasmic Reticulum Aminopeptidase 1 (ERAP 1) gene in axial spondyloarthritis Indonesian adults.

Background: Axial Spondyloarthritis (AxSpA) is chronic inflammatory arthritis involving the axial joint whose pathogenesis is related to the SNP ERAP1 gene, HLA B27, and cytokine proinflammatory (IL-17A and IL-23). Objective: Analyzed the role of SNP gene ERAP1 on disease activity and proinflammatory cytokines. Methods: This study comprised of two phases including a cross-sectional study and an in-vitro experiment in post-test with a control-group design. Participants underwent a PCR investigation searching for HLA-B27. Disease activities were measured by Ankylosing Spondylitis Disease Activity Score-Erythrocyte Sedimentation Rate (ASDAS-ESR) and modified Stokes Ankylosing Spondylitis Spinal Score (mSASSS). Subjects with HLA-B27 positive underwent PCR ERAP1 gene rs27434, genome-sequencing, and analysis. ELISA sandwich method was used to measure ERAP-1, IL-17, and IL-23 levels with lipopolysaccharide and IFN-γ induction. Analysis using independent t-test, Mann Whitney, and Pearson correlation test with p < 0.05. Results: The average ASDAS-ESR was 3.33 ± 0.89 and the average mSASSS was 26.53 ± 9.90. In HLA B27 positive group, SNP ERAP1 gene rs 27434 in which alleles A changed to G and A/G with genotypes AA to AG/GG was observed. SNPs of the ERAP1 gene had a correlation on mSASSS (r = 0.553; p < 0.05) and no correlation on ASDAS-ESR (r = 0.232; p = 0.235). There were significant differences observed in the SNP ERAP1 gene on ERAP1 and IL-17A levels in subjects with lipopolysaccharide and IFN-γ induction (p = 0.05) but no significant difference in IL-23 levels (p > 0.05). Conclusion: The SNP ERAP1 gene affects mSASSS value, ERAP1 levels, and IL-17A levels whereas ASDAS-ESR value and IL-23 level were not associated.

Th17/Treg Ratio and Disease Activity in Systemic Lupus Erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease that is characterized by T-cells imbalance. There are ongoing controversies about the role of specific T-helper cell subsets and their cytokines. The study aimed to confirm the disturbance of Th17/Treg ratio in SLE patients. METHODS: Subjects were SLE patients who met the American College of Rheumatology 1997 criteria. Disease activity assessment was measured by SLAM index. Th17 and Treg level was measured by flow cytometry. Th17 level was evaluated as CD4+L17 whilst Treg as CD4+Foxp3+. Final result is stated as Th17/Treg ratio. RESULTS: Thirty female subjects with active SLE had mean SLAM Score of 29.3±3.88, C3 level 25.2 (6-59.5), C4 level 15.25 (5-54.3), ESR 62.1±37.85, CRP 30.16±59.45, and anti-dsDNA 155.32±186.10. Higher Th17 level was found in SLE patients compared to healthy subjects (30.09 pg/ml vs 13.01pg/ml; 12.60% vs 0.91%). However, it did not correlate to disease activity (p>0.05; r=-0.28). Regarding Treg level, there was no significant difference between active SLE and healthy subjects (12.85 vs 11.05 pg/ml; 9.57% vs 2.05%). Treg level negatively correlated to SLE disease activity (p<0.01; r=-0.73). Th17/Treg ratio was 3.28±2.22% and it positively correlated to SLE disease activity (p<0.01; r=0.78). CONCLUSION: Th17/Treg ratio is positively correlated with disease activity. Th17 level is elevated but not correlated with disease activity. Decrease of Treg level is not significant though correlated with disease activity in SLE patients.