PubMed Health⌕ Search
Home/Archive/PubMed

PubMed · 8538083

[Diffuse panbronchioliltis associated with bullous pemphigoid].

K Oshikawa·Y Sugiyama·S Kitamura
View original source ↗Export metadata ↓

Abstract

A 64-year-old woman complained of multiple blisters in 1990. She had had a productive cough since 1975. Immunofluorescence study of a specimen obtained from a skin biopsy showed staining in a linear pattern for both IgG and C3 in the epithelial basement membrane zone (BMZ) of the dermal-epidermal junction, and a high titer of anti-BMZ antibody. These findings led to the diagnosis of bullous pemphigoid. Corticosteroid therapy was started, and resulted in some stabilization of the skin lesion. Exertional dyspnea and a productive cough developed gradually, and the patient was referred to our department in 1992. Chest X-ray film and CT findings revealed a diffuse granular shadow and bronchiectatic change, predominantly in the lower lung fields. Laboratory tests showed a high titer of cold hemagglutinin and a high level of serum immunoglobulin A. Pulmonary function tests showed a combined destructive-restrictive defect and hypoxemia (PaO2 = 58.5 Torr). From these findings diffuse panbronchiolitis was diagnosed. A low dose of erythromycin alleviated the patient's pulmonary symptoms and improved the chest radiographic findings.

Explore related subjects

AutoimmunityBronchiolitisFemaleHumansMiddle AgedPemphigoid, BullousCase ReportsEnglish AbstractJournal ArticleReview

Keep this discovery

Explore connections, maps & timelines

BibTeXRIS

K Oshikawa, Y Sugiyama, S Kitamura. 1995. [Diffuse panbronchioliltis associated with bullous pemphigoid].. https://pubmed.ncbi.nlm.nih.gov/8538083/

Cite the original work for its findings. Save a collection to share your selection of sources.

KEEP EXPLORING

Related citations

PubMed2026

Tracking GAD-specific T-cell expansions in Type 1 diabetes by intradermal GAD-Alum challenge.

Identifying and monitoring autoreactive T cells that drive beta cell destruction remains a major obstacle to developing effective immunotherapies for type 1 diabetes (T1D). These cells are extremely rare in peripheral blood and cannot be accessed directly from the pancreas. We used intradermal injection of Glutamic Acid Decarboxylase (GAD)-Alum to recruit GAD-specific T cells to accessible sites in the skin and skin-draining lymph nodes (LNs), sampled by skin suction blisters and ultrasound-guided LN aspiration. Peripheral blood samples obtained before GAD injection were restimulated with GAD in vitro to detect reactive CD4+ T cells. Single-cell RNA sequencing (scRNAseq) followed by re-expression of selected T cell receptors (TCRs) confirmed antigen specificity. Up to 70% of T cells at the skin injection site were clonally-expanded and 4 of 14 (28%) re-expressed TCRs were GAD-reactive. In LNs 1 of 14 (4%) clonally-expanded TCRs was GAD-reactive, representing ~0.08% of all T-cells. GAD-reactive cells across compartments displayed Th1 and Th17-associated transcription signatures. These results demonstrate the intradermal autoantigen challenge and scRNAseq, enable direct identification and molecular profiling of autoreactive T cells in vivo. This minimally invasive approach provides a powerful platform for tracking antigen-specific T cells to monitor disease activity and evaluate immune interventions in T1D.

Autoimmunity↗
PubMed2022

Immunodeficiency, autoimmunity, and increased risk of B cell malignancy in humans with TRAF3 mutations.

Tumor necrosis factor receptor-associated factor 3 (TRAF3) is a central regulator of immunity. TRAF3 is often somatically mutated in B cell malignancies, but its role in human immunity is not defined. Here, in five unrelated families, we describe an immune dysregulation syndrome of recurrent bacterial infections, autoimmunity, systemic inflammation, B cell lymphoproliferation, and hypergammaglobulinemia. Affected individuals each had monoallelic mutations in TRAF3 that reduced TRAF3 expression. Immunophenotyping showed that patients' B cells were dysregulated, exhibiting increased nuclear factor-κB 2 activation, elevated mitochondrial respiration, and heightened inflammatory responses. Patients had mild CD4+ T cell lymphopenia, with a reduced proportion of naïve T cells but increased regulatory T cells and circulating T follicular helper cells. Guided by this clinical phenotype, targeted analyses demonstrated that common genetic variants, which also reduce TRAF3 expression, are associated with an increased risk of B cell malignancies, systemic lupus erythematosus, higher immunoglobulin levels, and bacterial infections in the wider population. Reduced TRAF3 conveys disease risks by driving B cell hyperactivity via intrinsic activation of multiple intracellular proinflammatory pathways and increased mitochondrial respiration, with a likely contribution from dysregulated T cell help. Thus, we define monogenic TRAF3 haploinsufficiency syndrome and demonstrate how common TRAF3 variants affect a range of human diseases.

Autoimmunity↗
PubMed2007

A new expression of diabetes: double diabetes.

Diabetes is on the increase worldwide. The incidence of both type 1 and type 2 diabetes has shown a rise, in parallel with a notable increase in the incidence of a new expression of the disease in children and adolescents, with the characteristics of a mixture of the two types of diabetes, and referred to as 'double diabetes'. Insulin resistance and obesity, together with the presence of markers of pancreatic autoimmunity - namely, autoantibodies to islet cell antigens - typically define this condition. However, recognition of double diabetes can pose problems. In most cases, a reduction in the 'autoimmune load' and an increase in the 'metabolic load' are helpful for attaining a correct diagnosis in a diabetic child.

Autoimmunity↗