Behcet’s disease is a very rare disease characterised by vasculitis or inflamed blood vessels. Its main features are a complex of triads mainly, recurring oral aphthous ulcers, ulcers on the genital area and uveitis. There will be other systemic complains also but they differ from person to person. In most people, eye diseases are prominent with diseases of blood vessels due to active vasculitis. Vast majority cases of Behcet’s disease show skin manifestations. It can be mild or severe ranging from acne like lesions to nodules or erythema. Discomforts associated with gastro intestinal system may be so severe that it is impossible to get a differential diagnosis from inflammatory bowel disease clinically.
Major clinical presentations of Behcet’s disease include the following:
Oral ulcers – seen in almost all the cases
Genital ulcers – seen in 60-90 % of cases. They can be developed anywhere in the genitourinary tract. They may leave a scar.
Skin problems – papulonodular lesions, erythema nodosum, acneiform rashes, pseudofolliculitis, pyoderma gangenosum and rarely rashes similar to erythema multiforme.
Gastrointestinal discomforts – ulceration throughout the tract, mainly in the mouth, oesophagus, ileo-caecal junction and ascending colon. This condition is almost impossible to differentiate from IBD clinically.
Diarrhoea, bleeding, abdominal pain and fever are common. Complications like fistula and bowel perforation may occur.
Neurological – This is a rare compliation of Behçet’s disease affecting around 10% of patients. This can cause parenchymal and non-parenchymal disease. Parenchymal disease, affecting the cerebrum, brainstem, cerebellum and spinal cord, can result in intra-axial cranial neuropathy, pyramidal disease, focal and multifocal cerebral lesions, and various brainstem anomalies. The clinical presentation deends upon the part involved and the extent of neuraxial involvement. Non-parenchymal disease can cause cerebral venous sinus thrombosis, intracranial hypertension and recurrent meningitis.
Vascular BD – This is a unique form of the disease that affects arterial and venous system of all sizes. It is a major cause of morbidity and mortality.
Ocular – Eye diseases in BD if not treated properly can cause loss of vision.
Causes of Behcet’s disease
The exact cause is still unknown. It is believed that both genetic and environmental factors play a role in the development of this disease. About 60% of patients show the presence of the HLA-B51 genetic marker. HLA-B51 and HLA ERAP1 have been identified as BD susceptibility genes by Genome Wide Association Studies.
Pro-inflammatory cytokine cascade, inflammatory responses, tendency to relapse & remit and variable treatment responses to immunosuppressive medications in BD suggest that the disease has an autoinflammatory-autoimmune nature.
Treatment of Behcet’s disease
Many medications are administered through both oral and parenteral routes. Main drugs include:
Azathioprine
Mycophenolate mofetil
Methotrexate
Tacrolimus
Ciclosporin
Sulfasalazine
Dapsone
Thalidomide
Colchicine
Prednisolone
Cyclophosphamide
Anti-TNF α inhibitors
Infliximab
Adalimumab
Etanercept
Certolizumab
Rituximab
Interferon α
Alemtuzumab
Complications of medicine & treatment
Arterial aneurysm can develop during the course of treatment which may get enlarged in no time. It is a medical emergency that needs endovascular repair to prevent rupture or deranged pressure. In such cases, high mortality rate is seen in open surgeries. Formation of pseudoaneurysms can also lead to more complications. Any surgical procedure should be done with high immunosuppression measures.
Many medicines like thalidomide are contraindicated in pregnancy. Non-steroidal anti-inflammatory drugs should not be used in later stages of pregnancy because of the risk of premature closure of the patent ductus arteriosus.
Ayurvedic Concept of Behcet’s disease
Many disease conditions in Ayurveda involve presentations similar to conditions of vasculitis including Behcet’s disease. Diseases explained in Ayurveda in the names like Kushtha (deep rooted skin diseases), Vaatarakta (inflammatory joint & skin disease), Visarpa (inflammatory and fast spreading blisters like in herpes) share many stages of clinical manifestation of vasculitis. There is no directly named correlation for Behcet’s disease in Ayurveda. But considering the clinical presentation, it can be can be compared with Vaatarakta especially gambheera vaatarakta that involves deeper tissues & more systems. As the main affected part is blood and its channels, Rakta-Pitta vitiation is very evident. In most cases, the Vaata is also involved causing deranged movement & circulation leading to sthaanasamsraya of Pitta & Rakta in various parts of the body that manifests as clinical symptoms.
Ayurvedic Nidana of Behcet’s disease
- Food which is Vidaahi, viruddham & asrkpradooshanam(causing indigestion&acidity, opposite in potency, causing vitiation of Rakta)
- Unhealthy habits of sleep & waking up, sex
- Intolerant personality
- Sedentary lifestyle
- Injury
- Absence of Sodhanakriya(panchakarma)
- Causative factors for the vitiation of rakta along with usage of Vaata-vitiating and cold diet & regimen
Ayurvedic treatment for Behcet’s disease
As the vitiated Raktadhatu circulates all over the body with a fast pathogenesis, ayurvedic treatment for vasculitis starts with normalising the excess & vitiated Rakta by raktamoksha (bloodletting) in suitable patients. It is done either after using Sneha therapy or as an emergency procedure where the inflammation is severe. Raktamoksha should be done with letting out blood in small quantities, intermittently, considering the strength of the patient.
Other therapies are also performed according to the clinical presentation. Lepanam or external application of herbal pastes help reduce swelling and inflammation, thereby help relieve pain and improve mobility of the area, especially in joints. Dasamoolam, a combination of roots of ten herbal trees are used for this purpose. It is also used in the form of Parishekam or pouring warm decoction over the area for one hour or till the pain subsides. As vasculitis involves Raktadhatu, it needs alternate use of Heat and cold to get relief. Excess heat or cold will worsen the condition. Mild therapeutic sweating with various kinds of pindasweda(poultices), or upanaha (herbal paste with bandage covering) can help, especially in the early stages to reduce the thickening of vessel walls and to restore the blood flow. But when done too much, this can lead to haemorrhage.
Virechana or therapeutic purgation is the most advised Panchakarma procedure to balance Pitta, thereby Rakta here. It is done as an inhouse procedure under medical supervision with prescribed pre- and post-operative procedures. Also, daily intake of medicines for mild laxative effect like small doses of Avipattichoornam is beneficial. Basti procedures added with manjishtha, padma and ksheera are also done in many cases, considering the manifestations in joints and locomotor system. In patients with respiratory discomforts, vamana or therapeutic emesis will be helpful.
Commonly used internal medicines include Manjishthadi kashayam, mahamanjishthadi kashayam, guduchyadi kashayam, drakshadi kashayam, guduchisattwam, kokilakshakam kwatham and avipatti choornam. Proper diet, exercise and a healthy lifestyle are very important. Yoga or any other exercises are not advised during a painful flare up with severe inflammation. Regular stretching and mild cardio exercises are advised in the normal period. Yogacharya including naadisuddhi pranayama, sookshmasandhivyayama, suryanamaskaara is recommended to maintain balance.
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