Lack of CTGF*-945C/G Dimorphism in Thai Patients with Systemic Sclerosis

An association between connective tissue growth factor (CTGF) gene dimorphism at –945 (CTGF*-945C/G) and systemic sclerosis (SSc) has been reported with inconclusive results. We performed this study to determine whether such an association exists among Thai patients with SSc. DNA samples were taken from 50 Thai SSc patients (diffuse SSc in 39 and limited SSc in 11) and 99 healthy controls for determination of CTGF*-945C/G dimorphism by polymerase chain reaction (PCR) using specific oligonucleotide primers. The associations between the genotype frequencies, clinical manifestations and auto-antibodies were determined as well. When compared with the controls, SSc patients had no significantly higher frequencies of the GG genotype (44.0% vs 39.4%, p = 0.60), G allele (63.0% vs 65.2%, p = 0.80) or G phenotype (82.0% vs 90.9%, p = 1.0). There was no association between the presence of the GG genotype and clinical manifestations (pulmonary fibrosis, sclerodactyly, digital pitting scars, telangiectasia and pulmonary arterial hypertension), or the presence of auto-antibodies (anti-Scl-70, anti-SSA/Ro, and anti-RNP). In conclusion, we found no association between CTGF*-945C/G dimorphism and Thai SSc patients

Frequency and predictors of the lupus low disease activity state in a multi-national and multi-ethnic cohort

published_or_final_versio

Physician Global Assessment International Standardisation COnsensus in Systemic Lupus Erythematosus: the PISCOS study

The Physician Global Assessment International Standardisation COnsensus in Systemic Lupus Erythematosus (PISCOS) study aimed to obtain an evidence-based and expert-based consensus standardisation of the Physician Global Assessment (PGA) scoring of disease activity in systemic lupus erythematosus (SLE). An international panel of 79 SLE experts participated in a three-round Delphi consensus process, in which 41 statements related to the PGA in SLE were rated, using a 0 (strongly disagree) to 10 (strongly agree) numerical rating scale. Statements with agreement of 75% or greater were selected and further validated by the expert panel. Consensus was reached on 27 statements, grouped in 14 recommendations, for the use of the PGA in SLE, design of the PGA scale, practical considerations for PGA scoring, and the relationship between PGA values and levels of disease activity. Among these recommendations, the expert panel agreed that the PGA should consist of a 0-3 visual analogue scale for measuring disease activity in patients with SLE in the preceding month. The PGA is intended to rate the overall disease activity, taking into account the severity of active manifestations and clinical laboratory results, but excluding organ damage, serology, and subjective findings unrelated to disease activity. The PGA scale ranges from "no disease activity" (0) to the "most severe disease activity" (3) and incorporates the values 1 and 2 as inner markers to categorise disease activity as mild (>= 0.5 to 1), moderate (>1 and <= 2) and severe (>2 to 3). Only experienced physicians can rate the PGA, and it should be preferably scored by the same rater at each visit. The PISCOS results will allow for increased homogeneity and reliability of PGA ratings in routine clinical practice, definitions of remission and low disease activity, and future SLE trials

Adult-Onset Still’s Disease-like Syndrome following COVID-19 Vaccination: A Case Report and Review of the Literature

Adult-onset Still’s disease (AOSD)-like syndrome has rarely been reported as a complication of COVID-19 vaccination. This study reports a 31-year-old female patient who presented with fever, myalgia, arthralgia, pleuropericarditis, leukocytosis, and transaminitis following ChAdOx1 vaccination, and met Yamaguchi’s criteria. A PubMed literature search, performed up until March 2022, identified 10 such cases. A total of 11 cases, including the one in this report, developed AOSD-like syndrome after administration of the viral vector (ChAdOx1) vaccine (six patients) and mRNA vaccine (five patients: BNT162b2 in four and mRNA-1273 in one). There were four male and seven female patients, with their median (Q1, Q3) age and the onset of symptoms after vaccination being 36 years (29, 45) and 10 days (6, 13), respectively. Fever (100%), arthralgia/arthritis (90.9%), skin rashes (81.8%), and sore throat (81.8%) were the main clinical findings. Pericarditis (45.5%), myocarditis/cardiac dysfunction (36.4%), pleuritis (54.6%), and pulmonary infiltrations (36.4%) were also common. One patient developed macrophage activation syndrome. One patient responded well to non-steroidal anti-inflammatory drugs, and the other six showed a good response to high-dose corticosteroids alone. Of the remaining four patients, who showed partial responses to high dose corticosteroids, showed good responses to biological agents. AOSD-like syndrome following COVID-19 vaccination shared many similar clinical features and treatment outcomes to those of idiopathic AOSD (but with a higher prevalence of cardiopulmonary involvement in the former). Physicians should be aware of this extremely rare complication to achieve early diagnosis and provide proper management

Comparison of clinical features, disease activity, treatment and outcomes between late-onset and early-onset patients with systemic lupus erythematosus. A sex- and year at diagnosis-matched controlled study

Abstract Background Several studies have compared the clinical features and outcomes of late- and early-onset systemic lupus erythematosus (SLE) patients. However, these previous studies were uncontrolled. The current study aimed to compare late- and early-onset SLE patients while controlling for sex and year at diagnosis (± 1 year). Methods The medical records of SLE patients in a lupus cohort from January 1994 to June 2020 were reviewed. Late-onset patients were identified as those with an age at diagnosis ≥ 50 years. The early-onset patients (age at diagnosis < 50 years) were matched by sex and year at diagnosis with the late-onset patients at a ratio of 2:1. Clinical manifestations, disease activity (mSLEDAI-2K), organ damage scores, treatment, and mortality were compared between the two groups. Results The study comprised 62 and 124 late- and early-onset patients, respectively, with a mean follow-up duration of 5 years. At disease onset, when comparing the early-onset patients with the late-onset patients, the latter group had a higher prevalence rate of serositis (37.0% vs. 14.5%, p < 0.001) and hemolytic anemia (50.0% vs. 33.9%, p = 0.034) but lower prevalence rate of malar rash (14.5% vs. 37.1%, p = 0.001), arthritis (41.9% vs. 62.1%, p = 0.009), leukopenia (32.3% vs. 50.0%, p = 0.022) and lymphopenia (50.0% vs. 66.1%, p = 0.034). The groups had similar SLE disease activity (7.41 vs. 7.50), but the late-onset group had higher organ damage scores (0.37 vs. 0.02, p < 0.001). The rates of treatment with corticosteroids, antimalarial drugs, or immunosuppressive drugs were not different. At their last visit, the late-onset patients still had the same pattern of clinically significant differences except for arthritis; additionally, the late-onset group had a lower rate of nephritis (53.2% vs. 74.2%, p = 0.008). They also had a lower level of disease activity (0.41 vs. 0.57, p = 0.006) and received fewer antimalarials (67.7% vs. 85.5%, p = 0.023) and immunosuppressive drugs (61.3% vs. 78.2%, p = 0.044), but they had higher organ damage scores (1.37 vs. 0.47, p < 0.001) and higher mortality rates/100-person year (3.2 vs. 1.1, p = 0.015). After adjusting for disease duration and baseline clinical variables, the late-onset patients only had lower rate of nephritis (p = 0.002), but still received fewer immunosuppressive drugs (p = 0.005) and had a higher mortality rate (p = 0.037). Conclusions In this sex- and year at diagnosis-matched controlled study, after adjusting for disease duration and baseline clinical variables, the late-onset SLE patients had less renal involvement and received less aggressive treatment, but had a higher mortality rate than the early-onset patients

3697/N

Abstract. Infection, particularly pneumonia, is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). This study was performed to assess the prevalence, causative organisms, and outcomes of community-acquired pneumonia (CAP) in Thai SLE patients, and determine the predicting factors for death. A retrospective chart review of adult SLE patients, age &gt;16 years, seen at the Division of Rheumatology, Chiang Mai University over an 18 year period was carried out. Cases diagnosed with CAP were selected for this study. Of 542 SLE patients, a total of 56 episodes of CAP occurred in 52 patients. Their mean age ± SD and duration of SLE were 37.98 ± 11.48 years and 34.99 ± 54.53 months, respectively. Thirty-three CAP cases (58.9%) occurred within the first year of diagnosis with SLE. The causative organisms identifiable in 40 patients (71.5%) were Mycobacterium tuberculosis in 12, Nocardia spp in 6, Aspergillus spp in 5, Staphylococcus aureus in 3, Pneumocystis carinii, Haemophilus influenzae, Escherichia coli, and Pseudomonas aeruginosa in 2 each, and Acinetobactor baumanii, Burkholderia pseudomallei, and Strongyloides stercoralis in 1 each. The remaining 3 patients had mixed bacterial infection. The overall mortality rate was 26.8%. Use of high dose prednisolone (≥15 mg/day), and ventilator support were significantly associated with death

HLA Association among Thai Patients with Diffuse and Limited Cutaneous Systemic Sclerosis

This study aimed to clarify the association of HLA Class I and II with dcSSc and lcSSc in Thais. HLA typing for 11 gene loci (Class I: HLA-A, B and C, and Class II [HLA-DR, DP and DQ]) was carried out using the Next Generation DNA Sequencing method (three fields) in 92 Thai patients with systemic sclerosis (55 dcSSc, 37 lcSSc) and 135 healthy controls (HCs). The distribution of HLA alleles in patients with dcSSc and lcSSc was compared. When compared with HCs, the AF of A*24:02:01, A*24:07:01, B*27:04:01 and B*27:06 showed an increasing trend in lcSSc patients without statistical significance. DRB1*15:02:01, DRB5*01:02:01, DQA1*01:01:01, DQB1*05:01:24, DPA1*02:01:01 and DPB1*13:01:01 increased significantly in dcSSc patients. DQB1*05:01:24 and DPB1*13:01:01 also increased significantly in lcSSc patients, but less significantly than in dcSSc patients. The association of DPB1*05:01:01 with lcSSc was significantly protective. HLA-A*24:02:01, B*27:06 and C*03:04:01 formed a three-locus haplotype that also constituted an eight-locus haplotype with DRB1*15:02:01, DQA1*01:01:01, DQB1*05:01:24, DPA1*02:01:01 and DPB1*13:01:01. There was a possibility that HLA Class I would play a role in the pathogenesis of lcSSc, while Class II played more of a role in the dcSSc in Thai patients

Identification of Emerging Human-Pathogenic Pythium insidiosum by Serological and Molecular Assay-Based Methods

Pythium insidiosum is a pathogen that causes disease in both animals and humans. Human infection is rare; however, when it does occur, most patients, especially those having underlying hemoglobinopathy syndromes, such as thalassemia, exhibit a severe form. We identified four isolates of P. insidiosum. Two were recovered from tissue biopsy specimens from thalassemic and leukemic patients, one was derived from brain tissue from a thalassemic patient, and another was isolated from a corneal ulcer from a fourth patient. Western blotting and an enzyme-linked immunosorbent assay (ELISA) were performed with a serum sample derived from one thalassemic patient. The methods used to identify the P. insidiosum isolates were based on morphology, nucleic acid sequencing, and a PCR assay. To confirm the identification, portions of the 18S rRNA genes of these four isolates were sequenced. The sequences were shown to be homologous to previously described P. insidiosum DNA sequences. In addition, PCR amplification of the internal transcribed spacer region specific for P. insidiosum was positive for all four isolates. The ELISA with the serum sample from the thalassemic patient gave a positive result from a serum dilution of 1:800. Finally, Western immunoblotting with this serum sample showed positive immunoglobulin G recognition for proteins of 110, 73, 56, 42 to 35, 30 to 28, 26, and 23 kDa. The results of this study show that both molecularly based diagnostic and serodiagnostic techniques are useful for the rapid identification of human pythiosis. The predominant antigens recognized by Western blotting may be useful in the development of a more sensitive and specific diagnostic tool for this disease