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Annals of Allergy, Asthma & Immunology... Jan 2021
Topics: Adenosine Monophosphate; Adult; Alanine; Antiviral Agents; Asthma; COVID-19; Common Variable Immunodeficiency; Fatal Outcome; Humans; IgG Deficiency; Immunization, Passive; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Methicillin-Resistant Staphylococcus aureus; Obesity, Morbid; SARS-CoV-2; Staphylococcal Infections; COVID-19 Serotherapy
PubMed: 32818593
DOI: 10.1016/j.anai.2020.08.017 -
Neurology(R) Neuroimmunology &... Nov 2020
Topics: Adult; Autoimmunity; Betacoronavirus; COVID-19; Coronavirus Infections; Female; Humans; IgA Deficiency; Pandemics; Plasma Exchange; Pneumonia, Viral; SARS-CoV-2
PubMed: 32817413
DOI: 10.1212/NXI.0000000000000881 -
Bone Marrow Transplantation Feb 2021CD19-CAR T-cell therapy (CART19) causes B-cell aplasia (BCA) and dysgammaglobulinemia but there is a lack of information about the degree of its secondary...
CD19-CAR T-cell therapy (CART19) causes B-cell aplasia (BCA) and dysgammaglobulinemia but there is a lack of information about the degree of its secondary immunodeficiency. We conducted a prospective study in children and young adults with acute lymphoblastic leukaemia treated with CART19, analysing the kinetics of BCA and dysgammaglobulinemia during therapy, as well as the B-cell reconstitution in those with CART19 loss. Thirty-four patients were included (14 female) with a median age at CART19 infusion of 8.7 years (2.9-24.9). Median follow-up after infusion was 7.1 months (0.5-42). BCA was observed 7 days after infusion (3-8), with persistence at 24 months in 60% of patients. All patients developed a progressive decrease in IgM and IgA: 71% had undetectable IgM levels at 71 days (41-99) and 13% undetectable IgA levels at 185 days (11-308). Three of 12 patients had protective levels of IgA in saliva. In two of three patients who lost CART19, persistent B-cell dysfunction was observed. No severe infections occurred. In conclusion, BCA occurs soon after CART19 infusion, with a progressive decrease in IgM and IgA, and with less impairment of IgA, suggesting the possibility of an immune reservoir. A persistent B-cell dysfunction might persist after CART19 loss in this population.
Topics: Antigens, CD19; Child; Female; Humans; Immunotherapy, Adoptive; Kinetics; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prospective Studies; Young Adult
PubMed: 32801317
DOI: 10.1038/s41409-020-01027-6 -
Medicine Jul 2020Lymphoid interstitial pneumonia is a rare benign pulmonary lymphoproliferative disorder usually presenting with a sub-acute or chronic condition and frequently...
RATIONALE
Lymphoid interstitial pneumonia is a rare benign pulmonary lymphoproliferative disorder usually presenting with a sub-acute or chronic condition and frequently associated with autoimmune disorders, dysgammaglobulinemia, or infections.
PATIENT CONCERNS
A 74-year-old woman with no past medical history presented with acute dyspnea, nonproductive cough, hypoxemia (room air PaO2: 48 mmHg) and bilateral alveolar infiltrates with pleural effusion. Antibiotics and diuretics treatments did not induce any improvement. No underlying condition including cardiac insufficiency, autoimmune diseases, immunodeficiency, or infections was found after an extensive evaluation. Bronchoalveolar lavage revealed a lymphocytosis (60%) with negative microbiological findings. High-dose intravenous corticosteroids induced a mild clinical improvement only, which led to perform a surgical lung biopsy revealing a lymphoid interstitial pneumonia with no sign of lymphoma or malignancies.
DIAGNOSES
Acute severe idiopathic lymphoid interstitial pneumonia.
INTERVENTIONS
Ten days after the surgical lung biopsy, the patient experienced a dramatic worsening leading to invasive mechanical ventilation. Antibiotics and a new course of high-dose intravenous corticosteroids did not induce any improvement, leading to the use of rituximab which was associated with a dramatic clinical and radiological improvement allowing weaning from mechanical ventilation after 10 days.
OUTCOMES
Despite the initial response to rituximab, the patient exhibited poor general state and subsequent progressive worsening of respiratory symptoms leading to consider symptomatic palliative treatments. The patient died 4 months after the diagnosis of lymphoid interstitial pneumonia.
LESSONS
Idiopathic lymphoid interstitial pneumonia may present as an acute severe respiratory insufficiency with a potential transient response to rituximab.
Topics: Aged; Antineoplastic Agents, Immunological; Fatal Outcome; Female; Humans; Lung; Lung Diseases, Interstitial; Rituximab; Tomography, X-Ray Computed
PubMed: 32791765
DOI: 10.1097/MD.0000000000021473 -
Case Reports in Medicine 2020X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency. Affected individuals usually present with the Epstein-Barr virus infection and have no...
X-linked lymphoproliferative disease (XLP) is a rare primary immunodeficiency. Affected individuals usually present with the Epstein-Barr virus infection and have no apparent disease prior to presentation. The most common clinical manifestations are fulminant infectious mononucleosis, dysgammaglobulinaemia, and lymphoma (usually of B-cell origin). XLP is caused by mutations in the gene which encodes the intracellular adaptor molecule SAP (signalling lymphocyte activation molecule- (SLAM-) associated protein). SAP is predominantly expressed in T cells and NK cells and functions to regulate signal transduction pathways downstream of the SLAM family of surface receptors to control CD4+ T cell (and by extension B-cell), CD8+ T cell and NK cell function, and development of NKT cells. Thus, SAP mutations cause dysregulation of the immune system, with defects in both cellular and humoral immunity. Here we report two clinical cases of three patients who presented with different manifestations of XLP, namely, fulminant infectious mononucleosis, Burkitt lymphoma and hypogammaglobulinaemia.
PubMed: 32774386
DOI: 10.1155/2020/7108657 -
European Review For Medical and... Aug 2020Mononeuritis multiplex (MM) is an unusual form of peripheral neuropathy involving at least two noncontiguous peripheral nerve trunks. The pure sensory form of MM occurs...
INTRODUCTION
Mononeuritis multiplex (MM) is an unusual form of peripheral neuropathy involving at least two noncontiguous peripheral nerve trunks. The pure sensory form of MM occurs rarely. Immunoglobulin (Ig)G subclass deficiency is a clinically and genetically heterogeneous disorder. Up to 50% of adults with selective subnormal IgG1 levels or selective IgG1 deficiency have a concomitant autoimmune disorder. Herein, we report the case of a patient with MM and selective IgG1 deficiency who showed remarkable clinical improvement after 2-year combination therapy with the DPP-4 inhibitor sitagliptin plus vitamin D3.
CASE REPORT
A 49-year-old man developed numbness in right hand and forearm. After 6 months, the patient developed left forefoot numbness. Approximately 8 years later, the patient started to develop numbness also in the right forefoot, along with symptoms of evening fatigue and occasional orthostatic hypotension. The patient also reported recurrent candidiasis in glans and intergluteal areas since adolescence. Electromyoneurography of lower and upper limbs revealed the presence of multiple mononeuropathies. Protein electrophoresis showed hypogammaglobulinemia and low serum IgG1 levels. Sural nerve biopsy showed the presence of perineuritis. The patient was diagnosed with MM due to perineuritis probably secondary to IgG1 deficiency. We, then, proposed combination therapy with sitagliptin and vitamin D3 in the attempt to achieve immunomodulation. At the last follow-up visit (2 years), the patient showed persistent clinical improvement, increase in IgG1 levels and normalization of protein electrophoresis.
CONCLUSIONS
To the best of our knowledge, this is the first case showing a remarkable clinical improvement of MM and selective IgG1 deficiency achieved through a combination therapy with sitagliptin and vitamin D3.
Topics: Cholecalciferol; Drug Therapy, Combination; Humans; IgG Deficiency; Male; Middle Aged; Mononeuropathies; Sitagliptin Phosphate
PubMed: 32767343
DOI: 10.26355/eurrev_202008_22502 -
International Journal of Molecular... Jul 2020In recent years, the incidence of immune-mediated gastrointestinal disorders, including celiac disease (CeD) and inflammatory bowel disease (IBD), is increasingly... (Review)
Review
In recent years, the incidence of immune-mediated gastrointestinal disorders, including celiac disease (CeD) and inflammatory bowel disease (IBD), is increasingly growing worldwide. This generates a need to elucidate the conditions that may compromise the diagnosis and treatment of such gastrointestinal disorders. It is well established that primary immunodeficiencies (PIDs) exhibit gastrointestinal manifestations and mimic other diseases, including CeD and IBD. PIDs are often considered pediatric ailments, whereas between 25 and 45% of PIDs are diagnosed in adults. The most common PIDs in adults are the selective immunoglobulin A deficiency (SIgAD) and the common variable immunodeficiency (CVID). A trend to autoimmunity occurs, while gastrointestinal disorders are common in both diseases. Besides, the occurrence of CeD and IBD in SIgAD/CVID patients is significantly higher than in the general population. However, some differences concerning diagnostics and management between enteropathy/colitis in PIDs, as compared to idiopathic forms of CeD/IBD, have been described. There is an ongoing discussion whether CeD and IBD in CVID patients should be considered a true CeD and IBD or just CeD-like and IBD-like diseases. This review addresses the current state of the art of the most common primary immunodeficiencies in adults and co-occurring CeD and IBD.
Topics: Adult; Celiac Disease; Child; Common Variable Immunodeficiency; Diagnosis, Differential; Gastrointestinal Tract; Humans; IgA Deficiency; Inflammatory Bowel Diseases
PubMed: 32718006
DOI: 10.3390/ijms21155223 -
Journal of Clinical Laboratory Analysis Oct 2020Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency disease and frequently reported in the Western countries. However,...
BACKGROUND
Selective immunoglobulin A deficiency (SIgAD) is the most common primary antibody deficiency disease and frequently reported in the Western countries. However, large-scale epidemiologic studies on SIgAD in China are still lacking.
METHODS
The clinical information of 555 180 subjects (age >4 years) including the outpatient, inpatient, and healthy subjects who had ordered serum immunoglobulin A, G, M in 9 hospitals of Zhejiang Province in China was collected. The SIgAD individuals were defined as IgA level <0.07 g/L with normal levels of serum IgG and IgM, whose age should be over 4 years, and any other secondary diseases causing SIgAD were also excluded. Then, the geographical and prevalence distribution of SIgAD individuals in Zhejiang Province and patients' clinical characteristics at the time of diagnosis were also reviewed.
RESULT
Among these 555 180 subjects who had ordered the immunoglobulin evaluation, the prevalence of SIgAD was 109/555180 (0.02%). The ratio of male to female of these SIgAD individuals was 1:1.37, which also included 87 adults (≥18 years) and 22 children (18 > age >4 years). For adults, the common clinical features were infections (43/87, 49.43%), autoimmune disorders (31/87, 35.63%), allergic cases (5/87, 5.75%), and tumor cases (4/87, 4.60%). Additionally, infectious diseases (20/22, 90.91%), autoimmune disorders (4/22, 18.18%), and allergic cases (1/22, 4.55%) were found in 22 children.
CONCLUSION
We first describe a large cohort of SIgAD individuals of Zhejiang Province in China. The incidence was 0.020%. The common clinical features were infection, autoimmune disorders, tumor, and allergy, and the infection rate was higher in children than the adults.
Topics: Adolescent; Adult; Child; Child, Preschool; China; Female; Geography; Hospitals; Humans; IgA Deficiency; Male; Middle Aged; Prevalence; Young Adult
PubMed: 32715518
DOI: 10.1002/jcla.23440 -
Revista Da Associacao Medica Brasileira... Jun 2020OBJECTIVE To study the profile of associated autoimmune diseases in a series of patients with systemic lupus erythematosus (SLE) and see if such associations are linked...
OBJECTIVE To study the profile of associated autoimmune diseases in a series of patients with systemic lupus erythematosus (SLE) and see if such associations are linked to IgA deficiency. METHODS Two hundred eighty-one patients with SLE were studied for Ig A levels by nephelometry. Levels equal to or under 0.05g/dL were considered as IgA deficiency. Epidemiological and clinical data, including the presence of associated autoimmune diseases, were extracted from the patient's charts. RESULTS Ig A deficiency was found in 6% of the patients. In 30.2% of SLE patients, there was at least one more autoimmune disease; Hashimoto thyroiditis and Sjögren's syndrome were the most common. No association between the occurrence of associated autoimmune disease with IgA deficiency was found. CONCLUSIONS There is a high prevalence of autoimmune diseases associated with SLE. IgA deficiency does not affect the presence of these associations.
Topics: Autoimmune Diseases; Humans; IgA Deficiency; Immunoglobulin A; Lupus Erythematosus, Systemic; Sjogren's Syndrome
PubMed: 32696881
DOI: 10.1590/1806-9282.66.6.752 -
Journal of Investigational Allergology... 2020
Topics: Adult; B-Lymphocytes; Biomarkers; Biopsy; Colonoscopy; Diagnosis, Differential; Disease Susceptibility; Germinal Center; Humans; Hyper-IgM Immunodeficiency Syndrome; Immunoglobulin Class Switching; Immunoglobulin M; Immunohistochemistry; Intestinal Polyposis; Male; Mutation; Peyer's Patches; Symptom Assessment
PubMed: 32694097
DOI: 10.18176/jiaci.0504