Osteitis Fibrosa Cystica 

What Is Antiphospholipid Syndrome?

Antiphospholipid syndrome (APS) is an autoimmune disease and a blood clotting disorder characterized by these clinical and laboratory criteria:

Clinical criteria – recurrent vascular thrombosis (clots in veins/arteries) from hypercoagulability (abnormal excessive clotting) and/or recurrent complications of pregnancy that include loss of the fetus  (miscarriage) and pre-eclampsia or eclampsia.

Laboratory criteria – persistently elevated anticardiolipin, anti–beta-2 glycoprotein I, and/or lupus anti-coagulant antibodies in blood.

In antiphospholipid syndrome autoantibodies are produced by the body and directed against negatively charged phospholipids that are found in the outer layer of cell membranes and platelets. B2-glycoprotein-I (a protein in blood plasma) has been found as a major target antigen for antiphospholipid antibodies.

Q: Are phospholipids important in the body?

A: Yes.  Phospholipid molecules are an essential part of cell membranes. They form a barrier around cells that protect the cell, allow movement of oxygen in and carbon dioxide out of the cell, and regulate other small molecules through the cell wall. Because phospholipids are widespread in the body, this disorder can produce a large variety of symptoms and affect many organs.

One severe effect of APS is the development of a blood clot in a vein deep in the arm or leg, called deep vein thrombosis (DVT). DVT can cause pain, swelling, redness, or increased warmth in the affected limb. Deep vein clots can break off, travel to the lungs, and cause pulmonary embolism.¹ Pulmonary embolism is a medical emergency.

Treatment is with anticoagulant medications and blood monitoring.

What Is Antiphospholipid Syndrome In Celiac Disease and/or Gluten Sensitivity?

• Relationship between antiphospholipid syndrome and celiac disease. Antiphospholipid syndrome is an associated immune disorder in celiac disease. The association between celiac disease and other immune disorders may be due to the sharing of a common genetic background, such as HLA antigens. However, in a very large study, involving 909 patients with celiac disease, Ventura and his associates found that the development of immune disorders in celiac disease was clearly related to the duration of exposure to gluten.²
• Relationship between antiphospholipid syndrome and features. Celiac disease and antiphospholipid syndrome share common clinical features including hypercoagulability, thrombotic phenomena, and various neurological manifestations, as well as recurrent abortions and lower birth weight. Vasculitic skin lesions were significantly more common in EMA positive patients, ie. celiac disease, than controls ( 62.5 vs. 2%) and there was a higher prevalence of superficial cutaneous necrosis (37.5 vs. 2%).³
• Relationship between antiphospholipid syndrome and thrombosis risk. A study investigating clinical features and risk factors for thrombosis that may occur in adult celiac disease provides evidence that risk factors for thrombosis should be identified in patients in adult celiac disease in order to correct them and add a thromboembolic prophylaxis or prevention.⁴

How Prevalent Is Antiphospholipid Syndrome In Celiac Disease and/or Gluten Sensitivity?

• A retrospective study of 87 patients diagnosed with celiac disease showed a prevalence of 2.3% for antiphospholipid syndrome.⁵
• In the reverse, 14% of patients with antiphospholipid syndrome were found to have EMA (anti-endomysial) antibodies from celiac disease compared to 1.1% of controls.³

What Are The Symptoms Of Antiphospholipid Syndrome?

Clinical manifestations of antiphospholipid syndrome a wide variety of symptoms that can involve multiple organs at different times over the course of the disease, including the central nervous system. 

Antiphospholipid syndrome is marked by these symptoms:

• Superficial skin necrosis (tissue destruction).
• Ulcerations.
• Livedo reticularis (purplish mottling of skin).
• Blood clot in a vein deep in arm or leg that can cause pain, swelling, redness, or increased warmth in the affected limb.
• Digital gangrene (fingers and toes).
• Pregnancy losses after 10th week of gestation (miscarriage).
• Low birth weight of baby.
• Pre-eclampsia that may progess to eclampsia.
• Stroke and transient ischemic attacks (TIA) are the most common neurological manifestation of antiphospholipid syndrome that is an important cause of juvenile stroke. Of note, any brain region can be affected by vascular thrombosis in antiphospholipid syndrome.⁶
• Chorea is also the most commonly encountered movement disorder in primary antiphospholipid syndrome, with an estimated frequency of 1.3%.⁶
• Seizures are present in about 10% of patients and either can be secondary to vascular lesions or occur without evidence of previous stroke.⁶

How Does Antiphospholipid Syndrome Develop In Celiac Disease and/or Gluten Sensitivity?

• Antiphospholipid syndrome (APS) results from an autoimmune mechanism. The predominant antibodies in APS are directed against protein antigens that bind to anionic phospholipids, such as Beta 2-glycoprotein I (B2GPI) and prothrombin. 
• Evidence suggests that tTG (tissue transglutaminase) tissue expression may be enhanced during tissue injury in antiphospholipid syndrome, and in predisposed individuals, may lead to the manifestations of celiac disease antibodies and potentially to celiac disease.
• On the other hand, in patients with celiac disease, tTG may be involved in the pathogenesis of antiphospholipid syndrome.⁷

Does Antiphospholipid Syndrome Respond To Gluten-Free Diet?

Studies are inadequate to determine how gluten free diet could delay or mitigate the course of antiphospholipid syndrome.³

6 Steps To Improve Antiphospholipid  Syndrome In Celiac Disease and/or Gluten Sensitivity:

• [dropcap]1 Remove the Trigger. Maintain a Strict, Nutritious Gluten Free Diet:

[box type=”shadow” ]Treatment. This condition responds to the complete elimination of gluten, which is the required treatment that improves both antiphospholipid syndrome and gut health because it reduces inflammation.

• 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.

• Eat foods that can replenish missing nutrients. Find them under NUTRIENT DEFICIENCIES.
• Take nutritional supplements as needed. Find them under NUTRIENT DEFICIENCIES.

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.[/box]

• [dropcap]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).

[box type=”shadow” ]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.⁹[/box]

[box type=”shadow” ]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, 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 includes tuna, salmon, cod, and others. Plants 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 below for examples.[/box]

• [dropcap]3  Information Sheet You Can Take to Your Doctor or Other Health Professional:

Click here.

 

• [dropcap]4  Manage Your Medications Safely:

[box type=”shadow” ]

Certain prescription drugs used to treat antiphospholipid syndrome can cause nutritional deficiencies. Ask your doctor or pharmacist about this possible adverse effect. Do not stop prescribed medications without supervision.

This is not a complete listing.

ANTI-COAGULANTS

• Warfarin (Coumadin®) depletes this nutrient: Vitamin K.[/box]

• [dropcap]5 Nutritional Supplements To Help Correct Deficiencies:

[box type=”shadow” ]

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

• Multivitamin/mineral combination once a day is useful to improve overall nutrient levels. This is a safe dose, but always check with your doctor to avoid interactions with medications.
• Ferrous fumarate or gluconate as prescribed for low iron due to blood loss if present (requires blood monitoring).

Storage Note: 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.[/box]

• [dropcap]6 Manage Natural Remedies: 

[box type=”shadow” ]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.[/box]

[box type=”shadow” ]Carminatives. The following  anti-inflammatory plant sources called carminitives help heal the digestive tract. They also tone the digestive muscles which improves peristalsis, thus aiding in the expulsion of gas from the stomach and intestine to relieve digestive colic and gastric discomfort.

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 also stimulate and improve digestion and are easily digested.
• Cabbage also stimulates and improves digestion and is also a liver decongestant.
• Lettuce also 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, lemon balm, and fennel, (as a tea) also help relieve nervous tension.
• Parsley also relieves 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 also soothing remedy useful for stimulating digestion of rich, fatty foods.

Carminative Spice Remedies:

• Cloves are also antispasmodic.
• Nutmeg is also useful for indigestion.
• Ginger.[/box]

[box type=”shadow” ]Exercise Helps:

Gentle exercise improves circulation and rids the body of toxins. Exercise only as tolerated on the advice of your physician.

• Walking is aerobic exercise that reconditions the whole body to improve stamina. Read more about Exercise and Fitness.
• Weight training builds muscle. Read more about Exercise and Fitness.
• Stretching improves flexibilty. Read more about Exercise and Fitness.

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

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

RESEARCH STUDY SUMMARIES

“Adult celiac disease with thrombosis: a case series of seven patients. Role of thrombophilic factors.” This retrospective study investigating clinical features and risk factors for thrombosis that may occur in adult celiac disease provides evidence that risk factors for thrombosis should be identified in patients in adult celiac disease in order to correct them and add a thromboembolic prophylaxis or prevention.

 Of 87 patients with adult celiac disease  seven cases of thrombosis were identified. Researchers looked to see if risk factors for thrombosis were identified and tested retrospectively antiphospholipid antibodies on the serum.

The overall prevalence of thrombosis was 8%, and 5.7% for spontaneous thrombosis. Five patients had venous thrombosis, one had arterial thrombosis, and two had both. The seven patients consisted in six women and one man with a mean age of 44.8 years at time of thrombosis. Thrombotic events occurred before the diagnosis of celiac disease in four cases. In three cases, venous thrombosis was in unusual sites: 2 in the portal vein and one splenic vein thrombosis.

In six cases, risk factors for thrombosis were identified which could be linked to celiac disease: one case of hyperhomocysteinemia, 3 cases of protein C and S deficiency due to vitamin K deficiency, and 2 cases of antiphospholipid antibodies.⁴

“The prevalence of coeliac disease antibodies in patients with the antiphospholipid syndrome.” This study investigating the prevalence of celiac disease antibodies in patients with antiphospholipid syndrome demonstrated that the presence of EMA-ELISA antibodies is associated with high prevalence of antibodies recognizing certain B2-glycoprotein epitopes and with cutaneous manifestations of antiphospholipid syndrome.

Vasculitic skin lesions were significantly more common in EMA positive patients than controls ( 62.5 vs. 2%) and there was a higher prevalence of superficial cutaneous necrosis (37.5 vs. 2%). Celiac disease and antiphospholipid syndrome share common clinical features, but the relationship between skin manifestations of antiphospholipid syndrome and celiac disease needs to be explored.³

Sources:

1. http://www.nhlbi.nih.gov/health/health-topics/topics/ebc/signs.html [↩]
2. 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. [↩]
3. Shamir R, Shoenfeld Y, Blank M, et al. The prevalence of coeliac disease antibodies in patients with the antiphospholipid syndrome. Lupus. 2003;32:394-9. [↩] [↩] [↩] [↩]
4. Berthoux E, Fabien N, Chayvialle JA, Ninet J, Durieu I. Adult celiac disease with thrombosis: a case series of seven patients. Role of thrombophilic factors. Rev Med Interne. 2011 Oct;32(10):600-4. doi: 10.1016/j.revmed.2011.02.025. [↩] [↩]
5. Berthoux E, Fabien N, Chayvialle JA, Ninet J, Durieu I. Adult celiac disease with thrombosis: a case series of seven patients. Role of thrombophilic factors. Rev Med Interne. 2011 Oct;32(10):600-4. doi: 10.1016/j.revmed.2011.02.025. [↩]
6. Carecchio M, Cantello R, Comi C. Revisiting the molecular mechanism of neurological manifestations in antiphospholipid syndrome: beyond vascular damage. J Immunol Res. 2014;2014:239398. doi: 10.1155/2014/239398. Epub 2014 Mar 13. [↩] [↩] [↩]
7. Shamir R, Shoenfeld Y, Blank M, et al. The prevalence of coeliac disease antibodies in patients with the antiphospholipid syndrome. Lupus. 2003;32:394-9. [↩]
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. [↩] [↩] [↩] [↩] [↩] [↩]