Successful pregnancies in a patient with Takayasu arteritis and antiphospholipid syndrome, maintained on infliximab corticosteroid-free regimen: case-based review.

Takayasu arteritis (TA) is a large vessel vasculitis affecting predominantly females below the age of 40. Patients with TA seem to be at increased risk for adverse pregnancy outcomes, resulting in mother or child complications. Although few studies analyzed the presence of antiphospholipid antibodies (APLA) in TA patients, an association between antiphospholipid syndrome (APS) and TA is rarely reported in the literature, mainly in the form of case reports. In fact, very few data regarding pregnancy outcomes in patients with TA and APS are available. An active form of Crohn's disease (CD) might be another risk factor strongly affecting the fertility rate. Here, we would like to present a 33-year-old woman with TA, double-positive APS and Crohn's disease (CD). The report is followed by the literature review of the association of APLA and/or APS with TA, focusing on analyzing the pregnancy outcomes. To our knowledge, this is the first case describing two successful, naturally occurring pregnancies, in a patient suffering from TA, APS and CD, and maintained on infliximab, azathioprine, and a corticosteroid-free regimen.

Autoimmune/inflammatory syndrome induced by adjuvants in a woman with Hashimoto thyroiditis and familial autoimmunity-a case report and literature review.

Introduction: Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) consists of a wide spectrum of symptoms and immunological features that are believed to develop in predisposed individuals after exposure to an adjuvant, including a silicone breast implant (SBI). Different autoimmune diseases (AIDs) have been associated with ASIA, but ASIA development after SBI in women with Hashimoto thyroiditis (HT) and familial autoimmunity has rarely been described. Case report: A 37-year-old woman presented in 2019 with arthralgia, sicca symptoms, fatigue, + antinuclear antibody (ANA), + anti SSA, and + anticardiolipin Immunoglobulin G (IgG) antibodies. She was diagnosed with HT and vitamin D deficiency in 2012. The familial autoimmunity was present: the patient's mother had been diagnosed with systemic lupus erythematosus and secondary Sjogren's syndrome and her grandmother with cutaneous lupus and pernicious anemia. In 2017, the patient had a cosmetic SBI procedure that was complicated by repeated right breast capsulitis. After 2 years of irregular visits due to COVID-19, she presented with + ANA, + anticentromere antibodies both in sera and seroma, sicca syndrome, arthralgias, twinkling in extremities, abnormal capillaroscopic findings, and reduced diffusing capacity of the lungs for carbon monoxide. She was diagnosed with ASIA, and antimalarial and corticosteroid therapy were introduced. Conclusion: In patients with HT and familial autoimmunity, SBI should be carefully considered due to the possibility of ASIA development. Hashimoto thyroiditis, familial autoimmunity, and ASIA seem to be interconnected in the complex mosaic of autoimmunity in predisposed individuals.

Vitamin D Status in Patients with Systemic Lupus Erythematosus in Serbia: Correlation with Disease Activity and Clinical Manifestations.

BACKGROUND: Numerous studies indicate potential role of vitamin D as an important factor in the development of many autoimmune diseases including systemic lupus erythematosus (SLE). Patients with SLE are especially prone to the development of vitamin D deficiency due to the nature of their illness. AIM: The aims of our study were to determine the prevalence of vitamin D insufficiency and deficiency in patients with SLE in Serbia, to identify clinical variables associated with vitamin D status and to examine the impact of vitamin D status on disease activity and presence of specific lupus autoantibodies. MATERIAL AND METHODS: The study included 46 patients with SLE. Serum 25(OH)D concentration was measured by electrohemiluminiscent immunoassay. RESULTS: The mean serum concentration of 25(OH)D was 11.9 ± 7.3 ng/ml. The prevalence of insufficiency was 32.6%, while the prevalence of deficiency was 67.4%. There was no association between vitamin D status and photosensitivity, skin lesions, arthritis and lupus nephritis. Vitamin D status was not associated with the presence of specific autoantibodies. There was no correlation between disease activity assessed by SLEDAI scale with the concentration of 25(OH)D. Patients who used vitamin D supplements and calcium did not have a significantly higher concentration of 25(OH)D. CONCLUSION: In conclusion, vitamin D deficiency is common in patients with SLE.

CLINICAL CHARACTERISTICS AND TREATMENT OF MELKERSSON-ROSENTHAL SYNDROME--OVERVIEW OF SIX PATIENTS.

INTRODUCTION: Melkersson-Rosenthal syndrome is a rare disease of unknown etiology. Histopathologically, it presents as granulomatous cheilitis. From laboratory aspect, it is a nonspecific, differential diagnostically and therapeutically complex condition. CASE REPORT: This is a report of six cases treated at the Department of Allergology and Immunology of the Clinical Center of Serbia, who had presented with the referral diagnosis of recurring or persistent lip edema, and who were diagnosed with Melkersson-Rosenthal syndrome upon detailed evaluation. Three patients had complete triad of symptoms, two had the oligosymptomatic form and one manifested the monosymptomatic form of the disease. Histopathological findings of the oral mucosa specimens verified the presence of non-necrotic epithelioid granulomas in all patients. The patients were treated with the H1 and H2 antihistamines, corticosteroids, followed by anabolic drugs and antibiotics, resulting in transient and unfavorable effects. CONCLUSION: In differential diagnosis, Melkersson-Rosenthal syndrome diagnosis primarily refers to conditions of angioneurotic edema and hereditary angioedema, as well as granulomatous diseases such as sarcoidosis, tuberculosis and Chron's disease. It is necessary to follow-up these patients in view of monitoring the effects of the therapy and possible development of systemic granulomatous diseases.

Isolated hepatic sarcoidosis.

INTRODUCTION: Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Although hepatic granulomas occur in 50-65% of patients with systemic sarcoidosis, isolated liver sarcoidosis is rare. Clinical presentation varies from asymptomatic to manifest. The diagnosis is based on a characteristic histopathological finding of liver biopsy. CASE REPORT: We reported a 69-year old man was admitted due to abdominal swelling and abdominal pain. Laboratory studies detected: cholestasis, pancytopenia and elevaton of angiotensin-converting enzyme. Abdominal imaging techniques showed liver cirrhosis, splenomegaly and ascites. The diagnosis of the hepatic sarcoidosis was confirmed by histopathological examination of liver biopsy. The patient was treated with corticosteroids. After 18 months the patient was without any subjective symptoms, and with biochemical and clinical improvement. CONCLUSION: Isolated hepatic sarcoidosis should be considered in the differential diagnosis of asymptomatic or simptomatic patients with hepatosplenomegaly and changes in liver functional tests. Only the timely diagnosis and proper treatment can lead to subjective and objective improvement of patients.

Immunoreactivity to food antigens in patients with chronic urticaria.

The goal of study was better understanding of complex immune mechanisms that can help to evaluate patients with chronic urticaria (CU), especially those with unknown etiology. The study involved 55 patients with CU. Control group consisted of up to 90 healthy persons. The presence and intensity of serum IgG, IgA, IgM and IgE antibodies to common food antigens: cow's milk proteins (CMP), gliadin and phytohemagglutinin were determined by ELISA. Determination of subpopulations of immunocompetent cells was performed by flow cytometry. Significantly enhanced IgE, but also IgA immunity to CMP was found in patients with CU in comparison to healthy controls: (p < 0.000004) and (p < 0.002), respectively. Notably, in 40 out of 55 CU patients, the increased levels of some type of immunoglobulin reactivity to CMP were found. Regarding gliadin, only the levels of serum IgE anti-gliadin antibodies were significantly enhanced in patients with CU (p < 0.04). Significantly enhanced percentage of CD89+ cells accompanied with significantly lower percentage of lymphocytes and significantly higher mean fluorescence intensity of CD26 expression on lymphocytes were found in patients with CU in comparison to healthy controls (p < 0.04), (p < 0.02) and (p < 0.003), respectively. Results of this study may help in better understanding the complex immune disturbances in patients with CU.

[Laboratory and morphologic parameters in patients with lupus nephritis]. / Laboratorijski i morfoloski parametri kod bolesnika s lupusnim nefritisom.

INTRODUCTION: Lupus nephritis is an example of glomerulonephritis mediated by immune complexes. The information obtained by kidney biopsy corroborates diagnosis and evaluation of disease activity, specify of prognosis and mode of treatment. The object of our study was to determine the prevalence of particular pathohistological types of lupus nephritis in our group of patients, to establish if there was a correlation of laboratory and morphological parameters, and to present the use of specific therapeutical protocols. METHOD: The study included 58 patients with diagnosed systemic lupus erythematosus (SLE) and lupus nephritis, who had biopsy of kidneys. The indications for biopsy were the following: proteinuria level over 0.5 g/24 hrs, erythrocyturia and cylindruria. The patients were examined and treated at the Institute of Allergology and Immunology, Clinical Centre in Belgrade, over the period 1994-2001. Within the testing, besides standard laboratory tests, the immunological evaluation was also performed as follows: the level of standard serum immunoglobulins, C3 and C4 components of complement, antinuclear antibodies (ANA) and antibodies to double-stranded DNA (dsDNA), were determined. RESULTS: There was 84.48% of female patients in the studied group. The mean-age was 36.5 years, while the average duration of disease (SLE) to kidney biopsy was 28.3 months. Considering cytopenia, leukopenia was found in 26.79% of patients, lymphopenia was recorded in 62.26% of cases while anaemia was noted in 52.63% of patients. The values of serum creatinine were elevated in 25.86% of patients, while creatinine clearance rate was lower (below 80 ml/min) in 75% of cases. The values of proteinuria are illustrated in Graph I. Cylindruria was found in 20.69% of subjects, massive erythrocyturia in 44.83%, and 46.55% of patients had more than 5 red blood cells in urinary sediment. Regarding the pathohistological findings, according to WHO classification, the biopsy of kidneys revealed the following distribution: class I--3.45% of patients, class IIA--24.14%, class IIB--31.03%, class III--12.07%, class IV--24.14%, class V--3.35%, and class VI 1.72% of cases. DISCUSSION: Within the immunological evaluation, the increased serum immunoglobulin G (IgG) level was found in 26.79% of subjects, suggesting that the consumption of complements in formation of immune complexes was the basic pathogenetic mechanism of lupus nephritis. Positive finding of ANA was recorded in about 95% of subjects, what was typical for SLE, while antibodies to dsDNA were positive in no less than 72.72% of cases, arguing for the fact that they were one of major nephritogenic antibodies. Considering the correlation analysis, no correlation between pathohistological findings and serum creatinine level was found, but there was the correlation between pathohistological findings and decreased creatinine clearance rate. The correlation between pathohistological findings and proteinuria up to 0.5 g/24 hrs was verified. There was no correlation between the increased IgG level and kidney biopsy findings, but it was found that decreased level of C4 complement component correlated with the degree of kidney lesion. The value of diastolic pressure also correlated with pathohistological findings. Concerning the applied mode of treatment, 46.55% of patients were administered pulse doses of cyclophosphamide combined with pulse doses of methylprednisolone, 5.17% had pulse doses of cyclophosphamide and 32.76% pulse doses of methylprednisolone, while 12.07% received glucocorticoid drugs combined with azathioprine, and glucocorticoids only were given to 3.45% of them. The conclusion will be that the biopsy of kidneys is the imperative in the evaluation of lupus nephritis, because the complete insight into the degree and type of kidney lesion as well as search for an optimal mode of treatment may be achieved only by assessment of combined clinical, laboratory and morphological parameters.