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Pulmonary Hemosiderosis, Idiopathic  (Lane-Hamilton Syndrome)

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Pulmonary Hemosiderosis. Courtesy Quizlet.com

Lung from a Patient who Had Pulmonary Hemosiderosis. Courtesy Quizlet.com

What Is Idiopathic Pulmonary Hemosiderosis?

Pulmonary hemosiderosis (IPH) is a rare and severe pulmonary syndrome characterized by a triad of recurrent episodes of alveolar hemorrhage (bleeding into air sacs), hemoptysis (coughing blood), and iron deficiency anemia.

Q: What is the prognosis for pulmonary hemosiderosis?

A: Untreated pulmonary hemosiderosis ends in death. Idiopathic is a term that means the cause of a condition is not known.

What Is Pulmonary Hemosiderosis In Celiac Disease and/or Gluten Sensitivity?

  • Relationship between pulmonary hemosiderosis and celiac disease. Pulmonary hemosiderosis in celiac disease is an associated pulmonary disorder. The combination of idiopathic pulmonary hemosiderosis and celiac disease is extremely rare, although both diseases may have a common pathogenetic link. This combination of hemosiderosis and celiac disease is called Lane-Hamilton syndrome, named after the two doctors who first described it.1
  • Relationship between pulmonary hemosiderosis and diagnosis. Celiac disease should be looked for in patients with idiopathic pulmonary hemosiderosis, especially in those in whom the severity of anemia is disproportionate to radiologic findings (x-ray) even in the absence of gastrointestinal symptoms, since both diseases may benefit from a gluten free diet.2,3
  • Relationship between pulmonary hemosiderosis and treatment. If celiac disease is present, the incorporation of a gluten-free diet helps to control the symptoms of hemosiderosis, allows reducing the immunosuppressive treatment and improves the clinical course of both conditions.4

How Prevalent Is Pulmonary Hemosiderosis In Celiac Disease and/or Gluten Sensitivity?

The association of pulmonary hemosiderosis in celiac disease is well documented in case reports of affected patients.5,6

A retrospective study of children diagnosed with non-diarrheal celiac disease observed a prevalence of 2.1% with idiopathic pulmonary hemosiderosis.7

What Are The Symptoms Of Pulmonary Hemosiderosis?

Pulmonary hemosiderosis is marked by these symptoms:

  • Bloody sputum.
  • Pulmonary hemorrhage.
  • Anemia causing fatigue, weakness, shortness of breath on exertion.
  • Leg cramps on climbing stairs.
  • Inattention.
  • Skin pallor (pale).
  • Cold intolerance.
  • Reduced resistance to infection.
  • Some patients report ice craving.

How Does Pulmonary Hemosiderosis Develop In Celiac Disease and/or Gluten Sensitivity?

  • Pulmonary hemosiderosis results from iron deficiency caused by malabsorption in celiac disease.

Does Pulmonary Hemosiderosis Respond To Gluten-Free Diet?

Yes. Pulmonary hemosiderosis responds to gluten free diet containing adequate iron and with iron supplementation.2

6 Steps To Improve Pulmonary Hemosiderosis In Celiac Disease and/or Gluten Sensitivity:

Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both lung and gut health.

  • Gut health is the foundation to restore ALL health. Restored health will enable you to maintain a strict gluten free diet, just as other life tasks will be easier.
  • A strict gluten free diet means removing 100% of wheat, barley, rye and oats from the diet.
  • Cutting out bread and other obvious sources of gluten is not good enough for recovery. Even 1/8th teaspoon of flour or bread crumb is enough to sustain the inflammation that is damaging your small intestine, causing increased permeability (leaky gut) and allowing undigested gluten to enter your body where it can damage structures and function, and instigate immune inflammatory responses.

Correct Your Individual Nutritional Needs.

Recovery. You should begin to feel better within a week and notice more energy as inflammation subsides and the  absorbing cells that make up the surface lining of your small intestine are better able to function.

  • Intestinal lining cells are replaced every 5 days. The healing process is like sunburn where the damaged surface layer of skin sloughs off and is replaced with new normal cells.
  • Leaky gut normally resolves in two month after starting a gluten free diet and brings about a big improvement in health. Improvement in intestinal permeability precedes morphometric recovery (cell appearance and structure) of the small intestine in celiac disease.8
  • The intestinal lining may take up to a year to heal.
  • 2 Reduce Inflammation. Foods to Eat and Foods Not to Eat:

Because gluten is inflammatory, eliminate OTHER inflammatory foods from your diet to reduce an additive effect to gluten. At the same time, try to eat foods that reduce inflammation (anti-inflammatory).

Here Are Major Inflammatory Food Types That Reduce Healing:

  • Damaging Foods. In susceptible persons, includes corn, dairy (cow), and soy. Lactose, the sugar in any animal milk disrupts intestinal permeability causing leaky gut.9
  • Allergenic Foods. Includes foods that trigger the immune sytem to produce IgE antibodies. Allergy testing is the usual way to discover these offending foods.
  • Shelf Stable Processed Foods. Includes any that contain additives and preservatives. Look for them on the nutrition label of the box or package. Additives and preservatives also disrupt intestinal permeability causing leaky gut.9
  • Fats. Limit deep fried foods, trans-fats, saturated fats (animal fat/butter), and EXCESSIVE omega-6 fatty acid oils like corn oil. Rancid fats, sodium caprate (a medium chain fat), and sucrose monester fatty acid (a food grade surfactant) induce significant disruption of the intestinal barrier that causes leaky gut.9.
  • Excessive Refined White Flours (bran layer removed)Includes products made from them such as cookies, bread, cakes, pies. Bran contains the vitamins and minerals that metabolize grains and slows the otherwise rapid entry of sugar from their digestion into the bloodstream. Also disrupt intestinal permeability causing leaky gut.9
  • Refined Sugars.  Includes white sugar, corn fructose and high fructose corn syrup.
  • Certain Spices. Includes paprika and cayenne pepper which disrupt intestinal permeability causing leaky gut.9
  • Alcohol and Caffeine. Disrupt intestinal permeability causing leaky gut.10
Here Are Important Anti-Inflammatory Food Types to Promote Health:

  • Fruits. Contain ample amounts of vitamins, minerals and phytochemicals which are naturally occuring components in plants that detoxify toxins, carcinogens (reducing the risk by 50%) and mutagens.
  • Non-Starchy Vegetables. Support intestinal integrity and provide ample amounts of vitamins, minerals and phytochemicals. Includes lettuce, kale, onion, broccoli, garlic, and others.
  • High Quality Complex Carbohydrates. Provide vitamins, minerals, phytochemicals, and fiber while boosting serotonin levels to help you relax and feel calm. Includes whole grains, legumes, and root vegetables such as carrots, parsnips, sweet potatoes, turnips, red beets, and others.
  • Antioxidants. Protect the body from inflammatory oxidant molecules that continually occur and help us handle stress and reduce irritability. Includes vitamin C-containing foods such as lemon, grapefruit, apricot, Brussels sprouts and strawberries, and others. Also, includes vitamin E-containing foods such as nuts, seeds, avocado, olive oil, and others. Cocoa is good, too.
  • Omega-3 Fatty Acids. Balance opposing omega-6 fatty acids and bad fats. Fish sources include tuna, salmon, cod, and others. Plant sources include flax, chia seeds, canola oil, and others.
  • Probiotics. Supply normal microbes needed for colon health and health of the body such as these fermented foods: yogurt, kefir, and unpasteurized apple cider vinegar.
  • Prebiotics/ High Fiber Foods.  Food with fiber keeps our population of colonic microbes healthy.
  • Protective Herbs and Spices.  See below #6 for examples such as ginger.
  • 3 Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

  • 4 Manage Your Medications Safely: 

Certain prescription drugs can deplete iron causing pulmonary hemosiderosis. Ask your doctor or pharmacist about this possible adverse effect of these drugs if you have been prescribed. Do not stop prescribed medications without supervision.

 This is not a complete listing.

CHOLESTEROL DRUGS

  • Colestid® and Questran® deplete Iron and others.

ANTI-INFLAMMATORIES  disrupt intestinal permeability.

  • Aspirin and Salicylates deplete Iron and others.

ANTACIDS / ULCER MEDICATIONS

  • Pepcid®, Tagamet®, Zantac® deplete Iron and others.
  • Magnesium and Aluminum Antacid preparations (Gaviscon®, Maalox®, Mylanta®) deplete Iron and others.
  • 5Nutritional Supplements To Help Correct Deficiencies:

The type and quantity of nutritional supplements that may be needed depend on which nutrients are deficient.

  • Multi-vitamin/mineral supplement once a day giving 100% (not thousands).
  • Ferrous fumarate as prescribed by doctor based on blood studies to determine iron needs. Do not take with other supplements because of interactions.  Always check with your doctor when taking supplements to avoid interactions with medications.

Storage Note for Supplements: Store container tightly sealed, away from heat, moisture and direct light to avoid loss of potency. That is, in a safe kitchen cabinet  – not in the bathroom or on the kitchen table.

  • 6Manage Natural Remedies:
Hydration:

  • Eight glasses of water are recommended per day unless there is a contraindication such as kidney or heart disease. The Institute of Medicine recommends approximately 2.7 liters (91 ounces) of total water, from all beverages and foods, each day for women and 3.7 liters (125 ounces) daily of total water for men.
  • If you are thirsty, drink water. Add fresh, squeezed lemon to water. Lemon is anti-inflammatory, alkalizing and provides vitamin C.
  • Hydration Test: Urine should be pale yellow. Fingertips should be plump, without pruning but this may not be reliable when fingers are swollen with edema. Lips should be plump, without puckering. The feeling of thirst can be unreliable.
  • What is wrong with soda, coffee, tea, and alcohol? These drinks are dehydrating, increase acid, and deplete nutrients.

Carminatives are plant sources that tone muscle and improve peristalsis, and thus aid in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort. Puree any foods that cannot be thoroughly chewed. Cook meats well or make them into soups and stews for ease of digestion.

Carminative Food Remedies:

  • Raspberry.
  • Carrot is also a cleansing digestive tonic.
  • Grape is also bile stimulating and a cleansing remedy for sluggish digestion and laxative.
  • Redbeets stimulate and improve digestion and are easily digested.
  • Cabbage stimulates and improves digestion and is also a liver decongestant.
  • Lettuce stimulates and improves digestion and is also an alterative, meaning it improves the function of organs involved with the digestion and excretion of waste products to bring about a gradual change.
  • Potatoes are antispasmodic (due to atropine like properties) and a liver remedy.

Carminative Herb Remedies:

  • Sage is also a digestive, astringent, bile stimulant and energy tonic that heals the mucosa. Drink as tea or use in cooking.
  • Chamomile (as a tea) also promotes healing and help relieve nervous tension. Drink as a tea.
  • Parsley relieves colic, gas and indigestion.
  • Rosemary as a tea and in cooking also is a nervous system tonic for stress and fatigue, bile stimulant, and can relieve headaches and indigestion.
  • Thyme is a soothing remedy useful for stimulating digestion of rich, fatty foods.

Carminative Spice Remedies:

  • Cloves are also antispasmodic.
  • Nutmeg is also useful for indigestion.
  • Ginger.
Exercise Helps:

Exercise improves circulation and rids the body of toxins.

Note: Exercise is important, but the amount and type of exercise undertaken depends on your health. Your first priority is to heal.

What Do Medical Research Studies Tell About Pulmonary Hemosiderosis In Celiac Disease and/or Gluten Sensitivity?

RESEARCH STUDY SUMMARIES

“Celiac disease presentation in a tertiary referral centre in India: current scenario.” This facility-based retrospective observational study compared the clinical spectrum of nondiarrheal celiac disease  (NDCD) with that of diarrheal/classical celiac disease (CCD) included consecutive patients diagnosed with celiac disease (CD) (as per modified ESPGHAN criteria) from October 2009 to August 2011. A total of 381 patients were diagnosed with CD during the study period. NDCD was present in 192 (51.8 %). NDCD had higher mean age at presentation (5.8 ± 2.8 years vs. 6.9 ± 2.9 years respectively) and longer duration of symptoms prior to diagnosis (2.9 ± 1.7 years vs. 3.6 ± 2.2 years) as compared to CCD.

In the NDCD group, the most frequent gastrointestinal (GI) symptoms were recurrent abdominal pain [122 (63.5 %)] and abdominal distension [102 (53.1 %)] followed by constipation [48 (25 %)], vomiting [76 (39.6 %)] and recurrent oral ulcers [89 (46.4 %)]. Vomiting and constipation were more frequently seen in NDCD as compared to CCD. Common extraintestinal manifestations in NDCD included failure to thrive [109 (56.8 %)], isolated short stature [36 (18.8 %)], persistent anemia [83 (43.2 %)] and hepatomegaly/splenomegaly or both [56 (29.2 %)].

Associated comorbidities included autoimmune thyroiditis [11 (5.7 %)], type 1 diabetes mellitus [8 (4.2 %)], bronchial asthma [23 (11.9 %)], idiopathic pulmonary hemosiderosis [4 (2.1 %)], Down’s syndrome [3 (1.6 %)], alopecia areata [6 (3.1 %)], polyarthritis [2 (1.0 %)], dermatitis herpetiformis [6 (3.1 %)] and chronic liver disease [6 (3.1 %)].7

CASE REPORT SUMMARIES

“Celiac disease with pulmonary hemosiderosis and cardiomyopathy.” This case report describes celiac disease presenting in a patient with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vitamin A deficiency was detected. This case suggests that celiac disease should be considered in patients with cardiomyopathy and/or pulmonary haemosiderosis regardless of the intestinal symptoms of celiac disease.3

“Pulmonary hemorrhage associated with celiac disease.” This case report describes diagnosing celiac disease in two patients of 13 years who consulted for hemoptysis and severe anemia that had not responded to immunosuppressive treatment with pulses of methyl prednisolone, oral meprednisone and hydroxychloroquine. Although both children highlight the absence of gastrointestinal symptoms at the time of consultation, the dosage of anti-endomysial and anti-transglutaminase antibodies was positive and biopsy confirmed the presence of intestinal enteropathy. It is emphasized that in patients with diffuse alveolar hemorrhage, even in the absence of gastrointestinal symptoms, the concomitant presence of celiac disease should be evaluated. If celiac disease is present, the incorporation of a gluten-free diet helps to control the symptoms, allows reducing the immunosuppressive treatment and improves the clinical course of both entities.4

“Lane-Hamilton syndrome: case report and review of the literature.” This case report describes diagnosing celiac disease in a three-and-a-half-year-old boy, who presented with poor general condition, stunted growth, had the presence of nail clubbing, persistent cough and frequent diarrhea. Persistent iron deficiency anemia without signs of hemolysis suggested Lane-Hamilton syndrome (LHS) which is an extremely rare combination of idiopathic pulmonary hemosiderosis (IPH) and celiac disease (CD), although both diseases are immunologically mediated and the pathogenetic link between them is not clear.

Three years of follow-up on gluten-free diet (GFD) resulted in a gradual recovery of the abnormal laboratory results in combination with an improving growth. Clinically, he is asymptomatic without any additional treatment. This case illustrates that CD should be specifically looked for in patients with IPH, especially those in whom the severity of anaemia is disproportionate to the IPH symptoms. Both diseases may benefit from a GFD.1

“Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in association with celiac disease.” This case report describes the beneficial effect of removing gluten from the diet in Lane-Hamilton syndrome (the uncommon co-occurrence of idiopathic pulmonary hemosiderosis and celiac disease) in three children aged between 7 and 14 years with idiopathic pulmonary hemosiderosis who were detected to have co-existing non-diarrheal celiac disease.

Institution of gluten-free diet in each of the three children resulted in amelioration of the pulmonary symptoms along with improvement of anthropometric parameters and hemoglobin over a short-term follow-up period of 8-17 months. Inhaled/oral steroids and immunosuppressants could be weaned off after dietary exclusion therapy in each of the three children. The reporting physicians state, ” Gluten free diet should be instituted in all patients diagnosed with Lane-Hamilton syndrome. It ameliorates both the pulmonary as well as the intestinal symptoms although the precise mechanism of the pulmonary response is as yet unclear.”11

“Celiac disease as a cause of unusually severe anemia in a young man with idiopathic pulmonary haemosiderosis.”  This case report describes celiac disease presenting in a patient with severe anemia. The combination of idiopathic pulmonary hemosiderosis (IPH) and celiac disease  is extremely rare though both diseases may have a common pathogenetic link. As illustrated by this case celiac disease should be specifically looked for in patients with IPH, especially those in whom the severity of anemia is disproportionate to radiologic findings even in the absence of gastrointestinal symptoms since both diseases may benefit from a gluten-free diet.2

  1. Hendrickx GF, Somers K, Vandenplas Y. Lane-Hamilton syndrome: case report and review of the literature. Eur J Pediatr. 2011 Dec;170(12):1597-602. doi: 10.1007/s00431-011-1568-5. [] []
  2. Malhotra P, Aggarawal R, Aggarwal AN, Jindal SK, Awasthi A, Radotra BD. Coeliac disease as a cause of unusually severe anaemia in a young man with idiopathic pulmonary haemosiderosis. Respitory Medicine. Apr 2005;99(4):451-3. [] [] []
  3. Işikay S, Yilmaz K, Kilinç M. Celiac disease with pulmonary haemosiderosis and cardiomyopathy. BMJ Case Rep. 2012 Nov 20;2012. pii: bcr2012007262. doi: 10.1136/bcr-2012-007262. [] []
  4. Testa ME1, Maffey A, Colom A, Agüero L, Rogé I, Andrewartha MS, Teper A. Pulmonary hemorrhage associated with celiac disease. Arch Argent Pediatr. 2012 Aug;110(4):e72-6. doi: 10.1590/S0325-00752012000400016. [] []
  5. Malhotra P, Aggarawal R, Aggarwal AN, Jindal SK, Awasthi A, Radotra BD. Coeliac disease as a cause of unusually severe anaemia in a young man with idiopathic pulmonary haemosiderosis. Respitory Medicine. Apr 2005;99(4):451-3. []
  6. Işikay S, Yilmaz K, Kilinç M. Celiac disease with pulmonary haemosiderosis and cardiomyopathy. BMJ Case Rep. 2012 Nov 20;2012. pii: bcr2012007262. doi: 10.1136/bcr-2012-007262. []
  7. Bhattacharya M, Kapoor S, Dubey AP. Celiac disease presentation in a tertiary referral centre in India: current scenario. Indian J Gastroenterol. 2013 Mar;32(2):98-102. doi: 10.1007/s12664-012-0240-y. Epub 2012 Aug 19. [] []
  8. Cummins AG, Thompson FM, Butler RN, et al. Improvement in intestinal permeability precedes morphometric recovery of the small intestine in coeliac disease. Clinical Science. Apr 2001;100(4):379-86. []
  9. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. [] [] [] [] []
  10. Farhadi A, Banan A, Fields J, Keshavarzian A. Intestinal barrier: an interface between health and disease. Journal of Gastroenterology and Hepatology. 2003;18:479-91. []
  11. Sethi GR, Singhal KK, Puri AS, Mantan M. Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in association with celiac disease. Pediatr Pulmonol. 2011 Mar;46(3):302-5. doi: 10.1002/ppul.21357. []

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