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Bronchiectasis

Bronchiectasis Image. Courtesy Quizlet.com
Bronchiectasis Image. Courtesy Quizlet.com

What Is Bronchiectasis?

[dropcap]B[/dropcap]ronchiectasis is a pulmonary disorder characterized by chronic dilation of bronchi (main airways) and bronchioles (small airways) with chronic inflammation and swelling, increasing the risk of infection.

In bronchiectasis, mucus produced to trap foreign substances, such as dust and bacteria, builds-up because the ability of airways to move it out is impaired. Normally, hair-like cilia that line airways beat mucus out of the lungs. Loss of this action increases mucus in the airways which increases risk of pneumonia.

The anatomy of airways is likened to a branching tree. The trachea, or windpipe, like the trunk of a tree, branches into two primary bronchi that connect it to the lungs. One bronchus connects to the left lung and the other to the right lung. Each bronchus further divides into smaller bronchi that connect to the lobes. Lobes are large subdivisions of lung tissue: two of the left lung and three of the right lung.

Lobe bronchi divide into smaller bronchial tubes ending in small bronchioles. The ends of bronchioles are surrounded with and attached to tiny alveoli which are air sacs that look like clusters of grapes.

Q: What effect does inflammation have on the lungs?

A: Alveoli are only one cell thick, where oxygen is obtained by the bloodsteam from air breathed into them and carbon dioxide is released from the bloodstream to air that is breathed out of air sacs through the pulmonary tree. Two things happen:

  1. Inflammation  impairs exchange of gases in alveoli, resulting in lack of sufficient oxygen (O2) for body cell functions, called hypoxia, and build-up of carbon dioxide (CO2) in blood, called CO2 retention.
  2. Inflammation narrows passageways, which reduces the movement of air to and from the alveoli, and this puts stress on the right side of the heart.

Bronchiectasis can affect one or both lungs or one segment or multiple segments of a lung. Treatment is with antibiotics, chest physiotherapy and good nutrition.

What Is Bronchiectasis In Celiac Disease and/or Gluten Sensitivity?

Common Variable Immunodeficiency 

Depiction of an antibody. Courtesy NIH
Depiction of an antibody. Courtesy NIH

What Is Common Variable Immunodeficiency?

[dropcap]C[/dropcap]ommon variable immunodeficiency (CVID) is a primary antibody deficiency disease characterized by the onset of recurrent bacterial infections resulting from markedly decreased immunoglobulin antibody production and antibody levels.

Q: What causes common variable immunodeficiency?

A: Common variable immunodeficiency is caused by a defect in any critical stage of B cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. This is a set up for infection, autoimmune disease, and cancer.

One important histologic feature is the absence or paucity of plasma cells in the biopsy that occurs in common variable immunodeficiency. The diagnosis is initially made by measuring quantitative immunoglobulins and then specific immune testing on these cell subsets in the circulation by flow cytometry.1

Early diagnosis and treatment with IgG therapy (immunoglobulin G) can decrease illness and mortality.2 

Pulmonary damage is the most frequent complication and may result from recurrent infections and/or immune dysregulation. Other complications due to the underlying immune dysregulation include lymphoproliferative disease (granulomatous disease, lymphadenopathy and hepatosplenomegaly), autoimmune disease, gastrointestinal disease such as chronic inflammation and an increased risk of cancer.3

It is important to recognize that common variable immunodeficiency can occur at any age, but an early onset may be associated with an increased risk of gastric cancers and lymphoma, particularly of the intestine.3

Patients with common variable immunodeficiency should be managed by an immunologist with experience in primary immunodeficiency states, given the complications of these patients.4

What Is Common Variable Immunodeficiency In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Murray JA1, Rubio-Tapia A. Diarrhoea due to small bowel diseases. Best Pract Res Clin Gastroenterol. 2012 Oct;26(5):581-600. doi: 10.1016/j.bpg.2012.11.013. [↩]
  2. Maarschalk-Ellerbroek LJ, Hoepelman AI, van Montfrans JM, Ellerbroek PM. The spectrum of disease manifestations in patients with common variable immunodeficiency disorders and partial antibody deficiency in a university hospital. J Clin Immunol. 2012 Oct;32(5):907-21. doi: 10.1007/s10875-012-9671-6. [↩]
  3. Maarschalk-Ellerbroek LJ, Hoepelman AI, van Montfrans JM, Ellerbroek PM. The spectrum of disease manifestations in patients with common variable immunodeficiency disorders and partial antibody deficiency in a university hospital. J Clin Immunol. 2012 Oct;32(5):907-21. doi: 10.1007/s10875-012-9671-6. [↩] [↩]
  4. Murray JA1, Rubio-Tapia A. Diarrhoea due to small bowel diseases. Best Pract Res Clin Gastroenterol. 2012 Oct;26(5):581-600. doi: 10.1016/j.bpg.2012.11.013. [↩]

IgA Deficiency

IgA Molecule.
Depiction of the IgA Molecule

What Is IgA Deficiency?

[dropcap]I[/dropcap]gA deficiency (IgAD) is an immunodeficiency disease characterized by lack of immunoglobulin A type antibody production, called IgA antibody, with no detectable levels in blood or secretions.

Q: What is an IgA antibody?

A: IgA is an antibody of the immune system that is secreted by plasma cells (specialized white blood cells) through epithelial cell linings of mucosal surfaces into mucosa secretions to protect the lining from microbe invasion.

In fact, immunoglobulin class A is the main protein of the mucosal immune system. This includes mucosa of the eye surface, digestive tract, respiratory tract, urinary tract, and genital tract.

Both major histocompatibility complex (MHC) and non-MHC genes contribute to susceptibility to the disease. The former genes appear to be located in different parts of the MHC region depending on the HLA haplotype. The latter show a marked overlap with genes associated with a variety of autoimmune disorders including Graves’ disease, systemic lupus erythematosus, type 1 diabetes and celiac disease, suggesting common pathophysiological mechanisms. The involvement of genes associated with autoimmunity may suggest that IgAD in itself is an autoimmune disease.1

IgA deficiency may progress into a common variable immunodeficiency (CVID).2

What Is IgA Deficiency (IgAD) In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. Wang N, Hammarström L. IgA deficiency: what is new? Curr Opin Allergy Clin Immunol. 2012 Dec;12(6):602-8. doi: 10.1097/ACI.0b013e3283594219. [↩]
  2. Binek A, Jarosz-Chobot P.Selective immunoglobulin A deficiency. Pediatr Endocrinol Diabetes Metab. 2012;18(2):76-8. [↩]

Neutropenia 

neutrophilWhat Is Neutropenia?

[dropcap]N[/dropcap]eutropenia  is a blood disorder characterized by presence of an abnormally low number of neutrophils.

Neutrophils are white blood cells (leukocytes) that serves as the primary defense against infections by destroying bacteria in the blood. 

Specfically, neutrophils are a type of granulocyte that contain granules filled with potent chemicals to break down the microbes they ingest. Some of these chemicals, such as histamine, also contribute to inflammation and allergy.

The process of eating and digesting microbes is called phagocytosis. Neutrophils are phagocytes.1

Q: How do neutrophils eat microbes?

A: Segmented neutrophils  are the mature phagocytes that migrate through tissues to destroy microbes and respond to inflammatory stimuli. Segmented neutrophils comprise 40-75 % of the peripheral leukocytes. They are usually 9 to 16 µm in diameter. The nuclear lobes, normally numbering from 2 to 5, may be spread out so that the connecting filaments are clearly visible, or the lobes may overlap or twist. The chromatin pattern is coarse and clumped. The cytoplasm is abundant with a few nonspecific granules and a full complement of rose-violet specific granules.1

What Is Neutropenia In Celiac Disease and/or Gluten Sensitivity?

Sources:
  1. http://www.wadsworth.org/chemheme/heme/microscope/seg.htm [↩] [↩]

IgA Deficiency (IgAD): definition

An inherited immune deficiency disorder characterized by the marked absence of IgA (immunoglobulin A) type antibodies in blood in the presence of normal blood levels of IgG (immunoglobulin G) and IgM (immunoglobulin M) type antibodies.…