Autoimmune Diseases

Background. Indirect immunofluorescence assay (IIFA) based on antineutrophil cytoplasmic antibody (ANCA) testing is a commonly employed test for diagnosing autoimmune vasculitis. Antinuclear antibody (ANA) can give rise to a false interpretation of perinuclear-ANCA (pANCA) in ethanol-fixed granulocyte substrates. Analytical interference could frequently occur in setups where ethanol-fixed substrates are used alone. Here, we intend to investigate this ANA interference in pANCA interpretation. Methods. In this retrospective study, we studied anti-MPO-negative but ANA-positive and pANCA (IIFA based) samples. We also correlated immunoblot results (where data were available) and checked the association between grades of blot positivity (an indicator of the concentration of ANA) and frequency of pANCA interpretation. Data were analyzed by appropriate statistical techniques (Chi-square and kappa statistics). Results. About 19.2% of ANA blot (ENA-blot) positive samples displayed a pANCA positive pattern in the ethanol-fixed substrate, while this positivity in ENA-blot negatives was 6.5%. In positive ANA-IIFA samples, about 14.7% yielded pANCA patterns (on ethanol fixed substrates). Out of this, nuclear homogenous pattern yielding samples gave the highest frequency pANCA, that is, in 31.5% followed by speckled (11.1%), DFS (10.3%), and centromere (6.7%).The association of the nuclear homogenous pattern was statistically significant. Conclusions. ANA-positive results may interfere with the interpretation of pANCA as observed in ANA-IIFA and ENA-blot positive samples. ANA-IIFA patterns like nuclear homogenous may strongly associate this pANCA interpretation. This can help laboratories perform ANCA testing more effectively, ruling out ANA interference in ANCA screening.

Background. Diabetes mellitus impairs the reproductive system by damaging the glands and changing their function and hormone secretions. Given the previous studies on medical properties of silk cocoon, the aim of this study was to investigate the effect of the hydroalcoholic extract of silk cocoon on pituitary-gonadal axis hormones and the testis changes in diabetic male rats. Methods. In this experimental study, 35 male rats were divided into 5 equal groups. Control (C), nontreated diabetic rats (DNT1), and experimental diabetic rats treated (DT1) with a silk cocoon extract at concentrations of 200, 400, and 800 mg/kg for 56 days. Diabetes was induced by an injection of streptozotocin. Blood sampling was performed by the tail and heart after fasting. Body weight, serum levels of glucose, prolactin, leptin, inhibin A, IGF-2, activin A, insulin, LH, testosterone, FSH, and GnRH were measured along with the testis weight and diameter as the outcome of the study. Data were analyzed by SPSS version 20. Results. Investigation of hormonal factors indicated that all diabetic groups had higher prolactin, inhibin A levels than those in C group and lower leptin, IGF-2, activin A, insulin, LH, testosterone, FSH, and GnRH levels than controls. Silk cocoon treatment significantly decreased prolactin and inhibin in comparison of DNT1 group. While there was a significant increase in leptin, IGF-2, activin A, insulin, LH, testosterone, FSH, and GnRH levels compared with DNT1 (). A significant decrease in both the testis weights and diameters was observed in diabetic male rats compared to controls (). While silk cocoon treatment improved gonadal weight, the diameter of tunica albuginea, and seminiferous tubules as long as increased in numbers of spermatocytes and Sertoli-Leydig cells. Spermatogonia, spermatocyte, spermatid, spermatozoid, Sertoli cells, and Leydig cell count were significantly lower in the DNT1 group in comparison with the control group, while all groups receiving the highest dose of SC800 mg/kg daily had a higher count of cells than the DNT1 group. Conclusion. It seems that silk cocoon treatment decreases the effects of diabetes on hypothalamic-pituitary–gonadal axis.

The association between infectious diseases and autoimmunity has long been reported. Specifically, during the coronavirus disease 2019 (COVID-19) pandemic, this relation was further emphasized. The interplay between the two disease processes remains interesting, yet incompletely defined. Herein, we report a case series of six patients presenting with autoimmune phenomena first developed or exacerbated following severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. We describe the disease course and discuss the possible mechanisms underlying the association between autoimmunity and COVID-19.

Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was performed from January 2012 to December 2021 in all pediatric patients (aged <18 years) who fulfilled the diagnostic criteria for BSLE to evaluate the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence patterns, serological abnormalities, internal organ involvement, treatments, and outcomes. Among 1,415 patients with SLE, five patients were validated for the diagnosis of BSLE, accounting for 0.35%. The mean age at diagnosis was 12.2 years (standard deviation, 1.92). The clinical features of BSLE in the study population were generalized tense bullae and large extensive vesicles on the lips and perioral and mucosal areas. Pediatric BSLE in the study population revealed high SLE disease activity with multiple organ involvement. Hematologic abnormalities, serositis, and renal involvement were found in all patients, while polyarthritis (40%) and neurological abnormalities (40%) were less frequently observed. Systemic corticosteroids, intravenous immunoglobulin, immunosuppressants, antimalarials, and dapsone were prescribed in the study population. The cutaneous lesions subsided in all patients with a median clearance duration of 14 days (range, 5–56 days). BSLE in the pediatric population has auxiliary manifestations with high disease activity. Multiple organ involvement, especially hematologic abnormalities, serositis, and renal involvement, was frequently found in the study population. Although cutaneous lesions in BSLE subsided in all patients, involvement of other organs, especially renal impairment, required aggressive treatment, and long-term follow-up.

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease in which pathogenic autoantibodies and immune complexes are formed and mediate multiple organ and tissue damage. Thrombosis is one of the most common causes of death in patients with SLE. Anticoagulant therapy blocks the vicious cycle between inflammation and thrombosis, which may greatly improve the long-term prognosis of patients with SLE. However, the etiology and pathogenesis of this disease are very complicated and have not yet been fully clarified. Therefore, in the present review, we will highlight the characteristics and mechanisms of thrombosis and focus on the anticoagulant drugs commonly used in clinical practice, thus, providing a theoretical basis for scientific and reasonable anticoagulant therapy in clinical practice.

IgG4-related disease is a multiorgan immunological fibroinflammatory disorder characterized by lymphoplasmacytic infiltration and fibrosis in multiple organs accompanied by high serum IgG4 levels. The salivary glands are the most common organs involved in this disease. Recently, chronic sclerosing sialadenitis affecting salivary glands, formerly known as Küttner’s tumor, and Mikulicz’s disease have been classified as a class of IgG4-related diseases. The etiopathobiology of IgG4-related disease is not fully understood. It has recently been hypothesized that the inflammatory and fibrotic process and the increased serum IgG4⁺ levels in IgG4-related disease are the result of an interaction between B cells and T helper cells, suggesting that T cells may play a key role in the pathogenesis of this disease. The aim of this review is to discuss the proposed roles of different T cell subsets in the pathogenesis of IgG4-related disease focusing on their roles in immunopathogenesis of IgG4-related sialadenitis.