ABSTRACT
BACKGROUND: Autoimmune disorders are reported as presenting signs in patients with immunoglobulin A (IgA) deficiency. Herein, we aim to evaluate serum IgA among patients with autoimmune polyendocrinopathy. METHODS: Patients with two or more autoimmune endocrinopathies were selected and the serum IgA levels were measured. Patients with an isolated low serum IgA (<7 mg/dL) after exclusion of other causes of hypogammaglobulinemia were considered as selective IgA deficiency (SIgAD), while partial IgA deficiency (PIgAD) was defined as IgA levels below lower limits of IgA normal range for age but higher than 7 mg/dL. RESULTS: Fifty-three patients (19 [35.8%] male and 34 [64.2%] female) with autoimmune polyendocrinopathy enrolled in the study. Parental consanguinity and positive family history of autoimmunity were reported in 38.0% and 52.9% of patients, respectively. Overall, IgA deficiency was observed in 5 (9.4%) patients including PIgAD in 3 (5.7%) and SIgAD in 2 (3.8%) patients. Among IgA deficient patients, the first autoimmune disorder was developed at earlier ages (p = .002), and the prevalence of infection (p = .002), lymphoproliferation (p = .021), and overlap between insulin-dependent diabetes mellitus and autoimmune thyroiditis (p = .032) were significantly higher than patients with normal IgA. Also, the number of autoimmune comorbidities was closely correlated with the occurrence of IgA deficiency (p = .008). CONCLUSION: The prevalence of IgA deficiency in patients with autoimmune polyendocrinopathy is higher than that in the general population. In these patients, immunologic workup may lead to early diagnosis of inborn error of immunity, which can positively impact the evolution of complications and even management of the autoimmune disorders.
Subject(s)
Autoimmune Diseases , IgA Deficiency , Polyendocrinopathies, Autoimmune , Autoimmune Diseases/diagnosis , Autoimmune Diseases/epidemiology , Female , Humans , IgA Deficiency/complications , IgA Deficiency/diagnosis , IgA Deficiency/epidemiology , Immunoglobulin A , Male , Polyendocrinopathies, Autoimmune/diagnosis , Polyendocrinopathies, Autoimmune/epidemiology , Polyendocrinopathies, Autoimmune/genetics , PrevalenceABSTRACT
Immunologically, End Stage renal Disease (ESRD) is associated with some disorders in both innate and adaptive immune system in such a form that there is a coexistence of both immune activation and immune suppression. Although these disorders are complex yet thoroughly unknown, there is a close relation between the progressively defective immune system with side effects as well as mortality causes including cardiovascular problems, infections, and malignancies. From the other point, chronic inflammation as a major determinant of "dialysis syndrome" (including malnutrition, cachexia, and vasculopathy) is considered as the main factor of inability and mortality in dialysis patients. Such inflammation is generally arisen from immune system response to uremia and individual's repetitive contact with dialysis instruments and, in the long term, leads to premature aging via intensifying tissue degeneration. Therefore, the immune system is known as one of the most important therapeutic targets to reduce morbidity and mortality in uremic and dialysis patients. This review addresses different aspects as well as mechanisms of immune system dysfunction and possible therapeutics in dialysis patients.
Subject(s)
Immune System Diseases/etiology , Immune System Diseases/therapy , Kidney Failure, Chronic/therapy , Renal Dialysis/adverse effects , Uremia/therapy , Humans , Immune System Diseases/physiopathology , Kidney Failure, Chronic/complications , Prognosis , Renal Dialysis/methods , Treatment Outcome , Uremia/etiologyABSTRACT
Herein, we report a rare case of primary lung lymphoma in a 61 year-old woman with a history of 6-month nonspecific symptoms like dry cough, fever, chills and weight loss. She was admitted to a hospital and received broad-spectrum antibiotics but discharged without full recovery. In her second hospital admission, a bronchoscopic evaluation and transbronchial biopsy were performed, which were not diagnostic. Finally, an open lung biopsy was done. Immunohistochemical (IHC) staining of the specimen suggested pulmonary Hodgkin lymphoma. Because of disease recurrence, a second bronchoscopy was performed and endobronchial biopsy revealed transformation to anaplastic lymphoma. In the second recurrence, we decided to reevaluate the last biopsy specimens in greater details. Finally, after conduction of several staining patterns, the diagnosis of primary composite lymphoma of the lung was made.
