Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 12-year-old boy was hospitalized for resection of a bronchiectatic lesion. Investigation of an elevated cencentration of serum IgE led to a diagnosis of allergic bronchopulmonary aspergillosis. ABPA has rarely been described in the pediatric age group. This hypersensitivity lung disease is characterized by intermittent wheezing, fever, recurrent pulmonary infiltrates, eosinophilia, hyperimmunoglobulinemia E, and Type I (allergic) skin reactivity to aspergillus extract. Hyphae of aspergillus may also be found in expectorated brown mucus plugs. Type III (Arthus) skin test response and presence of precipitating antibody to this fungus may be demonstrated. Central bronchiectasis or pulmonary fibrosis may result from uncontrolled progression of this disease.
 ...
PMID:Hyperimmunoglobulinemia E in a child with allergic bronchopulmonary aspergillosis and bronchiectasis. 93 1

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease caused by bronchial colonization with Aspergillus fumigatus that affects approximately 10% of patients with cystic fibrosis (CF). The diagnosis in CF patients is difficult because the cardinal symptoms of ABPA occur frequently in CF, ie, pulmonary infiltrates and wheezing, as well as the frequent colonization with A fumigatus that leads to humoral reactivity. If left untreated, ABPA leads to bronchiectasis and pulmonary fibrosis. The pathogenesis of ABPA seems to be a prolonged asthmatic late-phase reaction orchestrated by CD4+ Th2-like T cells in response to persistent pulmonary A fumigatus allergen exposure. Thus, polyclonal and A fumigatus-specific IgE antibodies (and IgA and IgG) and blood pulmonary eosinophilia are stimulated by Th2-derived cytokines such as IL-4 and IL-5. In addition, IL-4 would also promote pulmonary transendothelial migration of eosinophils, basophils, and lymphocytes via induction of cell adhesion molecules and their ligands. IgE mast cell interactions would also contribute to the bronchial reactivity and inflammation. Recent advances have begun to identify immunodominant A fumigatus allergens. Evaluation of the quantity of IgE antibodies (and IgA and IgG) and T-cell cytokine responses to specific A fumigatus allergens should aid in the diagnosis and immunopathogenesis of ABPA, especially in CF patients.
 ...
PMID:Allergic bronchopulmonary mycosis complicating cystic fibrosis. 147 42

As elevated bronchoalveolar lavage (BAL) fluid histamine levels are noted in patients with pulmonary fibrosis (PF), we assayed BAL fluid from 16 patients with PF for the presence of a histamine releasing factor (HRF). HRF activity was assayed by measuring release of the preformed mast cell-derived mediators, histamine, or beta-hexosaminidase (beta-hex) from a purified population of IL-3 dependent mouse bone marrow derived mast cells (MBMMC) or human blood basophils. Mean BAL cell free histamine levels in the patients with PF was 1226 +/- 1349 pg/ml, whereas BAL histamine levels in a comparison group of six non-PF patients was 118 +/- 60 pg/ml. HRF was significantly elevated in BAL fluid of patients with PF (mean beta-hex release 24.5 +/- 12.9%; range 6.8 to 52.4%) compared to the non-PF group of patients (mean beta-hex release 7.9 +/- 7.7%; range 1.8 to 20.7%). The PF HRF not only degranulated MBMMC, but also induced the generation of the arachidonic acid metabolite leukotriene C4 from MBMMC (24.6 +/- 4.2 ng leukotriene C4/10(6) MBMMC). The PF HRF did not appear to be a cytokine previously identified in BAL fluid of patients with PF (i.e., platelet derived growth factor or insulin growth factor-1) or a human cytokine able to degranulate human basophils (i.e., IL-1, or granulocyte-macrophage-CSF) as these recombinant human cytokines did not induce MBMMC beta-hex release. Physicochemical characterization of the HRF revealed that it was relatively heat stable, pronase sensitive and on Sephadex G-75 and G-200 column chromatography had an apparent molecular mass of 30 to 50 kDa. The ability of PF BAL to induce beta-hex release from MBMMC was not dependent on IgE as unsensitized or lactic acid treated MBMMC release similar amounts of beta-hex compared to MBMMC sensitized with IgE. Thus, BAL fluid of patients with PF contains an HRF that induces beta-hex release from MBMMC via an IgE-independent mechanism. The presence of the HRF could explain elevated BAL histamine levels in patients with PF.
 ...
PMID:Mast cells and pulmonary fibrosis. Identification of a histamine releasing factor in bronchoalveolar lavage fluid. 169 11

Allergic bronchopulmonary aspergillosis (ABPA), an immunologic disorder, is characterized by bronchial colonization with Aspergillus species, resulting in chronic antigenic stimulation. Hallmarks of the disorder are asthma, cutaneous and serologic evidence of hypersensitivity to Aspergillus organisms, and fleeting pulmonary infiltrates. Diagnosis requires a high index of suspicion. Early diagnosis, aggressive therapy, and serial monitoring of serum IgE levels usually prevent progression of ABPA to bronchiectasis, pulmonary fibrosis, respiratory failure, and death.
 ...
PMID:Allergic bronchopulmonary aspergillosis. An increasingly common disorder among asthmatic patients. 220 99

Mast cells are critical for the expression of certain IgE-mediated responses, but the precise contributions of mast cells to biological processes not involving IgE are obscure. We have employed genetically mast cell-deficient WBB6F1-W/Wv and WCB6F1-S1/S1d mice to investigate the roles of mast cells in several different biological responses. This work strongly suggests that mast cells are not required for the elicitation of contact sensitivity (CS) responses, suppressor T cell-dependent tolerance to CS, reserpine-induced inhibition of CS responses, or bleomycin-induced pulmonary fibrosis. By contrast, mast cells appear to contribute to the acute gastric injury induced by ethanol and the acute inflammation of the skin induced by croton oil.
 ...
PMID:Analysis of mast cell function in biological responses not involving IgE. 357 Apr 98

Allergic bronchopulmonary aspergillosis (ABPA) occurs as a complication of asthma. It presents as an infiltrative pulmonary disorder with respiratory and systemic symptoms, eosinophilia, elevated total serum IgE, and skin sensitizing, as well as precipitating antibodies to Aspergillus fumigatus. Sputum cultures are not always positive for the organism. If unchecked, the disease may lead to bronchiectasis and ultimately pulmonary fibrosis. Therapy consists mainly of corticosteroids.
 ...
PMID:Allergic bronchopulmonary aspergillosis. 388 May 35

Mast cells are present in human lung tissue, pulmonary epithelium, and free in the bronchial lumen. By virtue of their location and their possession of specific receptors for IgE and complement fragments, these cells are sentinel cells in host defense. The preformed granular mediators and newly generated lipid mediators liberated upon activation of mast cells by a variety of secretagogues supply potent vasoactive-spasmogenic mediators, chemotactic factors, active enzymes, and proteoglycans to the local environment. These factors acting together induce an immediate response manifest as edema, smooth muscle constriction, mucus production, and cough. Later these mediators and those provided from plasma and leukocytes generate a tissue infiltrate of inflammatory cells and more prolonged vasoactive-bronchospastic responses. Acute and prolonged responses may be homeostatic and provide for defense of the host, but if excessive in degree or duration may provide a chronic inflammatory substrate upon which such disorders as asthma and pulmonary fibrosis may ensue.
 ...
PMID:The human lung mast cell. 642 78

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease characterized by Aspergillus fumigatus (Af) colonization, IgE and IgG anti-Af antibodies, pulmonary infiltrates, bronchiectasis, and pulmonary fibrosis. Little is known regarding T cell responses and their role in the pathogenesis of ABPA. To examine T cell reactivity to Af antigens, T cell clones (TCC) specific to the Asp f 1 antigen, an 18-kD protein of Af, were established from the peripheral blood of three ABPA patients. The majority of TCC isolated from ABPA patients, and specific for the Asp f 1 allergen of Af, are IL-4 producing CD4+ cells of the Th2 phenotype. Further analysis in this study revealed that the majority of TCC reacted to mainly two epitopes of Asp f 1, while the remaining TCC reacted to three additional "minor" epitopes. Blocking studies using monoclonal antibodies specific for class II HLA-D region gene products showed that most TCC, 19/21, were restricted by HLA-DR molecules, and the remaining two clones by HLA-DP molecules. The use of a panel of HLA-matched and mismatched EBV-transformed B cells as antigen presenting cells revealed that the HLA-DR restriction was mediated exclusively by either the HLA-DR2 or HLA-DR5 alleles. Genotyping of DRB1 gene products showed that class II presentation for most clones was not restricted to a single allele, representing DRB1 gene products of either HLA-DR2 or DR5. These studies offer insight into the cellular and molecular determinants which contribute to the immunopathophysiology of ABPA.
 ...
PMID:T cell subsets, epitope mapping, and HLA-restriction in patients with allergic bronchopulmonary aspergillosis. 863 13

Tropical pulmonary eosinophilia (TPE) usually affects people living in the tropics, especially those in Southeast Asia, India, and certain parts of China and Africa. However, owing to the rising frequency of world-wide travel and the migration between continents, this disease is increasingly seen in the West, where the diagnosis can be easily missed since it is rarely encountered and can mimic many other conditions. Cases of TPE have typically been reported to masquerade as acute or refractory bronchial asthma. TPE results from a hypersensitivity reaction to lymphatic filarial parasites found in endemic regions. There is evidence that it is more likely to occur in nonimmune individuals, ie, visitors to endemic regions, than in individuals of endemic populations who have developed immunity to filarial infections. Clinical features include paroxysmal cough, wheezing and dyspnea, and systemic manifestations such as fever and weight loss. A history of residence in a filarial endemic region and a finding of peripheral eosinophilia >3,000/mm3 should initiate a consideration of this disease. Other criteria for the diagnosis of TPE include absence of microfilariae in the blood, high titers of antifilarial antibodies, raised serum total IgE >1,000 U/mL, and a favorable response to the antifilarial, diethylcarbamazine, which is the recommended treatment. This disease, if left untreated or treated late, may lead to long-term sequelae of pulmonary fibrosis or chronic bronchitis with chronic respiratory failure. Herein lies the importance of early diagnosis and treatment of TPE.
 ...
PMID:Tropical pulmonary eosinophilia. 963 10

The occurrence of sarcoidosis in combination with common variable immunodeficiency (CVID) has been described in a small number of patients. In these patients, sarcoidosis consisted of lymphadenopathy, mild to moderate pulmonary involvement and hepatosplenomegaly. However, severe and rapidly progressive pulmonary fibrosis in combination with a severe defect of the cellular and humoral immune system has not been described yet. In our patient, defects of the T and B cell system resulted in severe immunodeficiency. The defect of the humoral immune system was characterized by the impairment of specific antibody production in vivo. In addition, hypogammaglobulinemia with missing IgA and IgE along with a marked defect in IgM and IgG production was noted. There was a progressively reduced lymphocyte proliferation in response to T cell mitogens, while proliferation after specific IL-2 stimulation was normal. A Th1 lymphocyte-subset-like profile might thus play a role in the pathogenesis and might form the connecting link between sarcoidosis and CVID. This is the report of a so far new and unique combination of severe immunodeficiency and sarcoidosis also associated with a congenital dysmorphia consisting of a palatal cleft. The findings of the 40 patients with CVID and sarcoidosis reported so far are discussed in order to point out the typical features of patients with this uncommon syndrome.
 ...
PMID:Sarcoidosis and common variable immunodeficiency. A case of a malignant course of sarcoidosis in conjunction with severe impairment of the cellular and humoral immune system. 1085 23


1 2 3 Next >>