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Sjögren’s Syndrome 

Hemochromatosis
Microscopic View of Hemochromatosis Stained Blue. Courtesy Wikimedia

What Is Hemochromatosis?

[dropcap]H emochromatosis, also called iron overload liver disease, is a common inherited disease in the Caucasian population that is characterized by increased iron deposition within the tissues (overload) associated with injury to them.

Hemochromatosis is an autosomal recessive disease, meaning a pair of abnormal genes are inherited from each parent. Parents are unaffected because they carry only one gene.

Q: Where is iron deposited?

A: In hemochromatosis, more iron is absorbed from the small intestine than is needed by the body. Subsequently, because the body has no satisfactory means to release iron overload, excess iron is deposited in various organs such as the liver causing cirrhosis, joints causing arthritis, and the pancreas causing diabetes mellitus.1

Consumption of alcoholic drinks with food should be avoided because alcohol increases leaky gut and greatly increases the absorption of iron. On the flip side, eggs and foods containing calcium such as milk and cheese are beneficial for hemochromatosis because they impair the absorption of iron. Another iron inhibitor is food that contains phytic acid which includes the bran and outer layer of grains, seeds, nuts, peas, beans, and lentils.

Iron overloading, as measured by a random (non-fasting) elevated transferrin saturation value, is estimated to occur in 1 to 6 people per 100 in the United States according to the CDC (Center for Disease Control and Prevention). Too much iron increases the risk for metabolic syndrome, type 2 diabetes mellitus, cancer, liver disease, and osteoporosis.

Medical treatment to remove excess iron from the body uses phlebotomy that draws blood through a vein and/or certain injectable drugs (deferoxamine and deferairox) that bind to iron.

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

Sources:
  1. www.irondisorders.org [↩]

Autoimmune Disorders In Celiac Disease

Lipase Enzyme. Courtesy johnsonmatthey.
Lipase Enzyme. Courtesy Johnson Matthey Catalysts.

What Is Macrolipasemia?

[dropcap]M acrolipasemia is a rare enzyme disorder characterized by altered molecules of lipase, a pancreatic enzyme needed to digest fats, that are abnormally bound with serum antibody proteins. These antibodies are commonly immunoglobulin G (IgG) and/or less likely immunoglobulin A (IgA).

Q: What happens when lipase is bound to immunoglobulins (IgA and/or IgG)?

A: The resulting molecule is too large to be filtered by the kidneys and excreted in the urine, consequently these abnormal molecules build up in the plasma causing sustained elevation of lipase levels called macrolipasemia.

Macrolipasemia occurs with or without macroamylasemia, which is the binding of immunoglobulin A and/or G to amylase, a pancreatic enzyme needed to digest starches.1

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

Sources:
  1. La Villa G, Pantaleo P, Tarquini R, Cirami L, Perfetto F, Mancuso F, Laffi G. Multiple immune disorders in unrecognized celiac disease: a case report. World J Gastroenterol. 2003;9(6):1377-1380, Available at: http://www.wjgnet.com/1007-9327/9/1377.asp. Accessed Jan 3, 2005. [↩]

IgA Nephropathy

X-ray of lungs in sacroidosis showing honeycoming. Courtesy Wikimedia.
X-ray of Lungs in Sacroidosis Showing Honeycoming. Courtesy Wikimedia.

What Is Sarcoidosis?

[dropcap]S arcoidosis is a multisystem granulomatous disease characterized by hard granulomas and inflammation of the alveoli in lungs that occurrs in 80% of patients.

Alveoli are the tiny one cell thick round structures that are clustered at the ends of air passages in the lungs where oxygen from air inhaled is exchanged for carbon dioxide from blood which is breathed out.

Q: What are granulomas?

A: Granulomas are collections of macrophages (white blood cells) that targeted and enclosed but failed to destroy a foreign substance that entered the body.  In turn, the macrophages become encased by other immune cells and fibroblasts and collagen. They can occur in any organ.

Presently, the etiology, or cause, of sarcoidosis is unknown, although it is thought the predisposition is genetic with a local immune mechanism. This disease has flares when active and remissions when symptoms subside.

Referral to a specialist is important for proper treatment and monitoring. The main treatment for severe active sarcoidosis is prednisone, a type of steroid that reduces inflammation in the body. In most people, prednisone relieves symptoms within a couple of months, although most people need to take prednisone for 12 months or longer. Long-term use of prednisone, especially at high doses, can cause serious side effects. Side effects include bone loss, elevated blood sugar, and high blood pressure.

When wheezing and coughing are a problem, inhaler devices are used to deliver medicine into the lungs in order to help open the airways.1

Ibuprofen is prescribed if sarcoidosis is causing joint pain. Steroid creams are prescribed for skin problems.

Sarcoidosis affects 10 to 20 person per 100,000.

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

Sources:
  1. National Heart, Lung, and Blood Institute [↩]

IgA Nephropathy: definition

A primary renal disease in which circulating IgA antigliadin antibodies (IgA-AGA) are often found and is characterized by recurrent hematuria (blood in urine), mild proteinuria (protein in urine), and changes to the kidney’s filtering apparatus…