Behcets disease

Category: Illness or disabilities

Type

Involuntary

Introduction and description

Behçet's disease or Behçet disease or Silk Road disease, is a rare small-vessel systemic vasculitis.  Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. 

The latter name arose because Behçet's disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia.  However, this may be a red herring as this disease is not restricted to people from these regions. It has been found in Siberia for example.  Furthermore there are questions being asked about whether – by making it a separate disease -  an overall pattern of illness created by pathogens has been masked.  Similar symptoms have emerged in other areas due to certain strains of Streptococcus bacteria.

Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the visible symptoms of recurrent oral ulcers, genital ulcers, and uveitis. Uveitus is inflammation of the uvea, the middle layer of pigmented structures of the eye that includes the iris, ciliary body, and choroid.

This illness can be fatal due to ruptured vascular aneurysms or severe neurological complications.

Symptoms

Mouth ulcers - Nearly all patients present with some form of painful oral ulcers or non-scarring oral lesions.

Genital ulcers -  Painful genital ulcerations usually develop around the anus, vulva, or scrotum and cause scarring in 75% of the patients.

Skin inflammation - Additionally, patients may present with ‘erythema nodosum’ - inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that can usually be seen

Deep skin ulceration - deep ulcers that often occur on the legs. When they occur, they usually initially look like small bug bites or papules, and they progress to larger ulcers

Inflammatory eye disease – which  can develop early in the disease and lead to permanent vision loss in 20% of cases. Symptoms vary depending on where the infection has developed but may involve painful eyes, conjuctival redness, decreased visual acuity, and visual field floaters.

Loss of vision - Optic nerve involvement in Behçet's disease is rare, however, cases of acute optic neuropathy (specifically anterior ischemic optic neuropathy) have been reported to occur and lead to gradual loss of vision, which may affect either one or both eyes, reduced visual acuity, reduced colour vision, different sized pupils, central scotoma, swollen optic disc, macular edema, and as a consequence pain.

Bowels – gastrointestinal symptoms include abdominal pain, nausea, and diarrhoea with or without blood.  There may also be abdominal tenderness, bloating, and overall abdominal discomfort that ‘closely mimics irritable bowel syndrome’.

Lungs – the person may cough up blood, and have pleurisy - an inflammation of the pleura, the lining surrounding the lungs. It might be noted here that there are many possible causes of pleurisy but viral infections spreading from the lungs to pleural cavity are the most common, which should be an interesting clue to the cause of this disease overall.  It may even be one of the routes of entry.  Pleurisy usually causes considerable chest pain as the inflamed pleural layers rub against each other every time the lungs expand to breathe in air, but there are some cases of pleurisy that go unnoticed for some time because no pain is present.

Other types of lung problems include coughs and in severe cases ruptures of the pulmonary artery which can be life-threatening as this can result in severe vascular collapse and death from bleeding in the lungs.  Nodules, consolidations, cavities and ‘ground glass lesions’ are common in patients with pulmonary involvement.

Musculoskeletal system – joint pain is experienced by up to half of patients, primarily of the large joints of the lower extremities.

Brain - CNS involvement most often occurs as chronic meningitis and encephalitis. Lesions tend to occur in the brainstem, the basal ganglia and deep hemispheric white matter and may resemble those of MS. Brainstem atrophy is seen in chronic cases.  Symptoms include confusion, seizures, and memory loss. They often appear late in the progression of the disease but are associated with a poor prognosis.

Heart - Pericarditis is a frequent cardiac manifestation – that is inflammation of the pericardium, the fibrous sac surrounding the heart. A characteristic chest pain is often present.  “Chronic aortic regurgitation due to aortic root disease may also be seen”.

Causes

Diseases in general are caused by pathogens – toxins such as heavy metals, pesticides or insecticides, bacteria, fungi, radiation, parasites, and viruses, but Behcet’s disease is of interest because its primary cause is viruses as opposed to all the others. Not exclusively viruses as we shall see, but viruses seem to be a major cause.

Viruses

More than one virus is implicated in the development of Behcet’s.  So far the EBV, HIV, possibly the herpes family of viruses and the measles virus have been seen in patients when tested:

So the EBV is implicated.

Behcet’s can follow HIV infection:

And also appears to be linked to certain strains of Herpes

The following paper was written by people who assumed the person caught CMV after immunosuppressants were used, but the evidence seems to point that CMV was the cause of the disease and by using immunosuppressants, they allowed it to gain a much greater hold and fully emerge from its latent state

There are more viruses implicated, however, there are a number of papers on PubMed to which you can refer to get an up to date list. 

Vaccines

The following paper is key as in addition to implicating the EBV virus again, the researchers also found the measles virus, and the virus was obtained from a vaccine.  In effect the cause is vaccines.  Both EBV and the measles viruses are latent, as such whether you are vaccinated or not, if the virus is a live one then it can return.

 

Bacteria

There are serious links with bacteria and one main culprit is the Chlamydia strain of bacteria:

Another bacteria implicated is the Streptococcus  strain, for example

Parasiteso

The next culprit is the parasite, again more than one is implicated, but here we have an example of a particularly nasty one, one that may explain the lesions and nodules common in this disease

A final note

We may find in due course that other pathogens – toxins for example are implicated, but we wish to end this section on a note.  In a number of cases on PubMed, where case histories were described, the person was on some form of immunosuppressant.  Thus although the disease is caused by pathogens, we might say that the ultimate cause is the use of immunosuppressants of various sorts – including anti-histamines.

References and further reading

J Oral Microbiol. 2015 Jun 5;7:27156. doi: 10.3402/jom.v7.27156. eCollection 2015.  The seroprevalence and salivary shedding of herpesviruses in Behçet's syndrome and recurrent aphthous stomatitis.  Seoudi N1, Bergmeier LA1, Hagi-Pavli E1, Bibby D2, Fortune F3.

• 1Centre for Clinical and Diagnostic Oral Sciences, Institute of Dentistry, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom.
• 2Division of Virology, Barts Health Trust, London, United Kingdom.
• 3Centre for Clinical and Diagnostic Oral Sciences, Institute of Dentistry, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom; f.fortune@qmul.ac.uk

Autoimmun Rev. 2015 Jul;14(7):609-15. doi: 10.1016/j.autrev.2015.02.009. Epub 2015 Mar 11.  The role of infections in Behçet disease and neuro-Behçet syndrome.  Marta M1, Santos E2, Coutinho E3, Silva AM4, Correia J5, Vasconcelos C6, Giovannoni G7.

• 1Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University London, 4 Newark Street, London E1 2AT, UK. Electronic address: m.calado-marta@qmul.ac.uk.
• 2Neurology Department, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal; Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Electronic address: ernestina.santos@gmail.com.
• 3Neurology Department, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Electronic address: estercoutinho@gmail.com.
• 4Neurology Department, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal; Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal; Unidade Multidisciplinar de Investigação Biomédica, Instituto de Ciências Biomédicas de Abel Salazar, Rua de Jorge Viterbo Ferreira, 228, 4050-313 Porto, Portugal. Electronic address: anaadmsilva@gmail.com.
• 5Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Electronic address: joaoacorr@gmail.com.
• 6Unidade de Imunologia Clínica, Centro Hospitalar do Porto, Largo Prof. Abel Salazar, 4099-001 Porto, Portugal. Electronic address: cvcarlosvasconcelos@gmail.com.
• 7Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University London, 4 Newark Street, London E1 2AT, UK.

Int J Rheum Dis. 2011 Aug;14(3):298-310. doi: 10.1111/j.1756-185X.2011.01601.x. Epub 2011 Mar 14.  Investigation of bacterial and viral agents and immune status in Behcet's disease patients from Iran.  Irschick EU1, Philipp S, Shahram F, Schirmer M, Sedigh M, Ziaee N, Gassner C, Schennach H, Meyer M, Larcher C, Herold M, Schoenitzer D, Fuchs D, Schoenbauer M, Maass M, Huemer HP, Davatchi F.  1Department of Ophthalmology, Medical University of Innsbruck, Austria. eveline.schretter-irschick@i-med.ac.at

Related observations

Healing observations

• Dr Duke's list of Chemicals with Antibehcet's Activity 018890
• Dr Duke's list of Plants with AntiBehcet's Activity 018891
• The Healing Power of Sleep 026790

Hallucination

• Colchicine 016796
• Colcrys and Col-probenecid 018219