Category: Common variable immune deficiency

ABSTRACT

Behcet’s syndrome is an auto inflammatory vasculitis which affects arteries and veins of all the types, is also known as oculo orogenital syndrome which is remitting and relapsing disorder. Generally vasculitis is inflammation of the blood vessels which makes vessels weak, stretched and bigger. It affects people at any age and affects specific organs such as skin, eyes and brain. Vasculitis is generally tied up with the immune system, or person’s suffering from rheumatoid arthritis, lupus. It may be serious as blood vessels become weak and bulges out causing aneurysm. When the blood vessels get inflamed and narrowed the area of blood flow gets blocked and further the tissue will die. It affects different parts with different symptoms. If vasculitis affects the part of the brain it causes strokes, in the kidney it leads to kidney failure. There are different types of blood vessels such as large (in Takayasu’s arteritis), Medium (Buerger’s disease, polyarteritis nodosa), Small (in Behcet’s syndrome, cutaneous vasculitis). In this article we will study in detail on Behcet’s syndrome, its causes, treatment and its ayurvedic management.

INTRODUCTION

Behcet’s syndrome (silk road disease) is a rare disorder that affects blood vessels inflammation throughout the body, it causes issues in mouth and genital area that forms recurrent sores. There is associated inflammation in both vessels and eyes. This sores lasts for a few days to weeks, there may be inflammation of the brain’s spinal cord, sometimes blood clots, aneurysms. It is also known as malignant aphthosis, which was first described in 1937 from Istanbul by Hulusi Behcet. It uses auto inflammatory disease with mucocutaneous manifestations such as oral and genital ulcers and in the ocular part it causes relapsing uveitis and systemic vasculitis which involves arteries and veins of all sizes. Both the genders are equally affected. It is not passed from parent to child but has a genetic effect which passes and develops this condition. Painful mouth sores (aphthous sores) is the first sign of Behcet’s disease. Sometimes joint involvement is also common in this disease.

FACTS

• Behcet’s disease is a rare condition which is most commonly seen in turkey, and other countries throughout the world.
• It affects one person in every 170,000 in the United states.
• This condition is not contagiou but can spread from person to person.
• Behcet’s disease affects everyone differently and the symptoms may weaken over a period of time.
• The prevalence of Behcet’s disease in turkey is as high as 400 cases.

PATHOPHYSIOLOGY

Behcet’s disease is an auto inflammatory vasculitis which involves arteries and veins, the lesions in this disease is that they lack necrotizing vasculitis or there is giant cell formation. In Behcet’s disease they lack antibodies and cell mediated immunity plays an important role. Type 1 helper activates and leads to increased circulating levels of T lymphocytes. Increased macrophage activations, neutrophils chemotaxis and there is phagocytosis of lesions. Due to increased neutrophil activation (skin pustules, oral aphthae) which further leads to neutrophil vascular reaction which causes tissue injury. This immune complexes plays a role in causing neutrophilic vascular reactions.

CAUSES

As Behcet’s disease is an autoimmune disorder that affects the immune system of the body, genetic and environmental factors play an important role. Both HLA-B5 and HLA-B51 are responsible for Behcet’s disease. Heat Shock Proteins are present in some of the bacteria which serve as a danger signal to the immune system. The anti HSP60 and anti HSP65 produced by mycobacterium and streptococci leads to an immune response which is linked to uveitis. Sometimes it may be due to viral and bacterial infections which may trigger the condition.

SYMPTOMS

THE MAIN SYMPTOMS INCLUDES-

• Mouth sores/ Genital sores
• Skin and joint pain
• Inflammation in the eyes
• Areas that commonly affects the Behcet’s disease
• Mouth – Painful sores which look similar to canker sores are the most common signs. This can heal in some weeks but can reoccur.
• Skin – There are acne like sores on the whole body. Red colour nodules on the lower part of the leg.
• Genitals – Red sores which can be seen in the scrotum and vulva part, can further leave scars.
• Eyes – There is uveitis which causes redness, pain and blurred vision.
• Joints – There is swelling and pain in the joints, which can further involve ankles, elbows and wrist.
• Blood vessels – There may be inflammation and redness when there is formation of the blood clots. When there is inflammation in the arteries further causes blockage of the vessels.
• Digestive system – It includes abdominal pain, bleeding
• Brain – When there is inflammation in the brain it causes headache, fever, poor balance.
• In other organs such as lungs (pleuritis, hemoptysis) and kidneys involvement is rare but mainly include- A amyloidosis and glomerulonephritis.
• Heart – Pericarditis is a frequent cardiac manifestation, aortic regurgitation is seen and acute coronary thrombosis is reportedly seen.

DIAGNOSIS

1. Pathergy test
2. Blood tests-
• ESR (erythrocyte sedimentation rate)
• C- reactive protein (CRP)
• Kidney function tests
• Liver function tests
• Genetic Marker
3. Chest X Ray
4. Endoscopy
5. CT (Computerized tomography)
6. MRI (Magnetic resonance imaging)

DIFFERENTIAL DIAGNOSIS

• Inflammatory bowel disease
• Seronegative arthritis
• Systemic lupus erythematosus
• Sarcoidosis
• Multiple sclerosis
• Relapsing polychondritis

TREATMENT

There is no cure for Behcet’s disease, but certain medications can control the pain and inflammation of the flare.

• Skin creams, Gels, Ointments – These topical corticosteroids are applied to the skin and genital sores to reduce the inflammation.
• Mouth rinse – It may reduce the pain of the mouth sores.
• Eye Drops – They are anti inflammatory medicines which can relieve inflammation and make it mild.

SYSTEMIC TREATMENT

• Corticosteroids to reduce the inflammation
• Certain medications that suppresses immune system, certain drugs such as Azathioprine, cyclosporine
• Apremilast is an oral medication used to treat mouth ulcers.
• Colchicine helps to relieve mouth sores, genital sores.
• Surgical intervention is mainly indicated where there is vascular involvement.

COMPLICATIONS

• Behcet’s disease is associated with ocular and neurologic involvements. When patients have hypopyon related uveitis and retinal involvements they are at high risk of blindness.
• There may be coronary arterial aneurysmal rupture.
• Miscarriages due to vasculitis of the placenta.

AYURVEDIC MANAGEMENT

According to Ayurvedic principles this Behcet’s Syndrome is associated with aggravation of the pitta dosha. The main cause of most of the ulcers are due to provoking factors of vata and pitta doshas. Chronic digestive problems can easily trigger pitta dosha, when this dosha is imbalanced in the gut it causes gastric and duodenal ulcers, inflammatory bowel disease. Due to aggravated pitta dosha there is an excess amount of acidic juice which is produced by liver, pancreas. This is due to excess secretion of acid which overwhelms the mucous lining and causes hyperacidity and later results in ulceration.

TREATMENT

It can be treated with various herbs and panchkarma therapy for the cure of this disease-

• Vamana – The certain herbs are administered to induce vomiting, it removes the toxins from the stomach and helps in cleaning the stomach, eliminating the extra doshas from the body.
• Virechana – This herb helps to remove the excessive toxins and mucous from the GI tract and thus improves digestion.
• Basti – This is administered through anal canal and helps to clean the lower abdomen, especially the colon.
• Rasayana – It helps to heal the damaged muscle tissues and supports healing of the body.

Diet recommendations (AAHAR)

• Follow a pitta soothing diet, avoid hot and spicy foods.
• Include cereals like rice flakes, puffed wheat and rice.
• Avoid abrasive foods that cause irritation and pain.
• Hydrate adequately
• Have light sweet food
• Don’t skip food
• Include ghee, cucumber, cumin seeds in diet.
• Avoid sour food including curd and pickles.

Lifestyles Changes (VIHAR)-

• Practice good oral and personal hygiene
• Nasal karma can be beneficial
• Brush your teeth gently using soft brush
• Rinse your mouth
• Local applications of turmeric to heal the sores
• Kawal and gandusha is beneficial for specific period of time

HERBS USEFUL FOR BEHCET’S SYNDROME

1. SHALLAKI

Shallaki (Boswellia serrata) is helpful in the treatment of muscular pains and osteoarthritis. It has lagu (light to digest), rooksha (dryness) guna. It is helpful in balancing kapha and pitta doshas. Shallaki is very useful in treatment of ulcers, wounds and further cleanses wounds. It is useful in blood disorders such as in abscess, menorrhagia and in nasal bleeding. Helps to relieve pain, improves mobility and flexibility and is used in the treatment of rheumatoid arthritis.

2. DARUHALDI

Daruhaldi (Berberis aristata) is used to treat eye disorders, skin disorders, urinary tract disease. It has an alkaloid which is useful in treating diabetes and high cholesterol levels. Daruhaldi has antibacterial, antidiarrheal and anti cancer activity. It has tikta, kashaya taste. It balances kapha and pitta doshas. Helpful in treatment of pain and itching disorders related to eyes, ears and oral cavity.

3. KATUKI

Katuki (Picrorhiza kurroa) is a bitter root used in the treatment of chronic fever and in skin disorders. It has cooling potency so helps to pacify pitta doshas and kapha dosha without disturbing digestive fire. Katuki is a good carminative so used as a blood purifier, promotes the flow of bile from gallbladder to duodenum. Helps to relieve intestinal worm infestation, and restores liver function and controls high blood sugar level.

4. VACHA

Vacha (Acorus calamus) is known as a sweet flag, which helps to improve speech and intelligence. It has katu (pungent), tikta (bitter) taste, and helps to balance kapha and vata dosha. Vacha relieves ama dosha, improves digestion. It has antimicrobial properties, which helps to improve the voice tone and is used against psychological imbalances and disorders. Has antimicrobial properties and induces vomiting.

HERBAL REMEDIES BY PLANET AYURVEDA FOR BEHCET’S SYNDROME

Planet Ayurveda provides the best combination of herbal remedies which provides great relief to patients and the formulations prepared are made 100% pure without any adulterations. Ayurvedic medications prepared are under strict guidance of M.D experts and check the quality of medicines and there is no compromise with the health of the patients. For Behcet’s syndrome planet ayurveda provides many herbal remedies which are free from any side effects.

PRODUCTS LIST

1. BOSWELLIA CURCUMIN
2. YOGRAJ GUGGUL
3. NAVKARSHIK CHURNA
4. RUMOGIN 5 CAPSULES
5. KAISHORE GUGGUL
6. PITTA BALANCE

PRODUCTS DESCRIPTION

1. Boswellia Curcumin

Boswellia Curcumin are poly herbal capsules formulated by planet ayurveda using ingredients such as Shallaki (Boswellia serrata), Curcumin (Curcuma longa). These capsules have the property of anti-inflammatory so they provide relief to patients and it can be used as an arthritis supplement. It helps to reduce the morning stiffness and swelling in the joints. Haridra has liver cleansing property and helps in fighting any kind of arthritis which increases mobility and reduces stiffness.

Dosage: 1 capsule twice daily after meals with plain water.

2. Yograj Guggul

Yograj Guggul are herbal tablets formulated by planet ayurveda using ingredients such as Guggul (Commiphora mukul), Musta (Cyperus rotundus), Bibhitaki (Terminalia bellirica), Haritaki (Terminalia chebula), Piper longum, Cuminum cyminum and many more. Yograj Guggul is widely used in the treatment of Gout and rheumatoid arthritis. It helps to treat bloating and in case of worm infestation, and deals with splenomegaly and in abdominal tumors.

Dosage: 1-2 tablets twice daily with lukewarm water.

3. Navkarshik Churna

Navkarshik Churna is polyherbal powder which is formulated by planet ayurveda using ingredients such as Amla (Emblica officinalis), Bahera (Terminalia billerica), Neem (Azadirachta indica), Daruhaldi (Berberis aristata) and many more. This powder helps to reduce and treat the ailments like pain and causing tightness on the affected part. It is helpful in increasing the strength of the body. Helpful in detoxifying the blood and improves immunity and has immunomodulating action.

Dosage: 1 teaspoon twice daily after meals with plain water.

4. Rumogin 5 Capsules

Rumogin is poly herbal capsules formulated by planet Ayurveda using ingredients such as Haridra (Curcuma longa), Shallaki (Boswellia serrata), Pippali (Piper longum) and many more. These capsules detoxify the body and help to make joints healthy and movable. Helps to manage morning stiffness, swelling and pain and is useful in rheumatoid arthritis. Long use of these capsules help to maintain the synovial fluid and help in healing of fracture, and effectively balances vata and kapha disorders.

Dosage: 1-2 capsules twice daily.

5. Kaishore Guggul

Kaishore Guggul are herbal tablets formulated by planet ayurveda using ingredients such as Guggul (Commiphora mukul), Guduchi (Tinospora cordifolia), Ginger (Zingiber officinale), Red Physic Nut (Baliospermum montanum) and many more. These tablets help to eliminate the toxins from the joints, act as a blood purifier, and support healthy metabolism. Kaishore Guggul helps to relieve muscle cramps and inflammatory disease of muscle.

Dosage: 1-2 tablets twice daily with lukewarm water.

6. Pitta Balance

Pitta Balance are polyherbal capsules formulated by planet ayurveda using ingredients such as Jahar Mohra Pishti (Calcium Compound), Giloy Satva (Tinospora cordifolia), Mukta Pishti (Calcium Compound) and many more. This capsules help to balance pitta dosha and it helps to balance the imbalanced thermal energy, It helps to enhance the digestive power and helps to control anger, and is rich in calcium so helpful in menopausal women. Restores healthy skin and works as a blood cleanser.

Dosage: 1-2 capsules twice daily with plain water after meals.

CONCLUSION

Behcet’s syndrome is an auto inflammatory vasculitis which affects arteries and veins of all the types, is also known as oculo orogenital syndrome which is remitting and relapsing disorder. It is also known as malignant aphthosis, which was first described in 1937 from Istanbul by Hulusi Behcet. According to Ayurvedic principles this Behcet’s Syndrome is associated with aggravation of the pitta dosha. There are many herbal remedies which are described by Planet Ayurveda in detail and provide great relief to one’s being.