Sunday, January 20, 2013

Differential between Behçet's and ME/CFS is needed

Some of the symptoms of Behçet’s:
  • Unpredictability: impossible to predict onset or clearing-up of symptoms, onset of symptoms sudden, difficulties in keeping to arrangements, appointments, working, affairs, managing household, family, social activities, hobbies
  • Arthritis: reduced mobility or complete immobility, pain.
  • Oral ulcers: pain,diet restricted to soft or liquid food, inability to eat at all, inability to talk, dribbling, dehydration, malnutrition.
  • Genital ulcers: immobility, sex-life affected, embarrassment, suspicion, urinary retention, pain.
  • Visual level: fear of onset of blindness, handicapping according to level of impairment and ability to adapt, pain.
  • Skin: disfiguring and embarrassing skin lesions, easy bruising, poor slow healing, pain.
  • Gastro-intestinal: wind, diarrhoea (with blood and mucus) or constipation, severe abdominal pain. Mimics inflammatory bowel diseases, irritable bowel syndrome. Stomach ulcers, ulcers in gullet. Risk of perforated ulcer. Dehydration, malnutrition
  • Thrombophlebitis: immobility, pain.
  • Thrombosis: immobility, pain. Risk of embolism.
  • Ears: hearing loss, tinnitus, vertigo
  • Chest: wheezing, breathlessness, haemorrhages, haemoptysis, pleurisy, pain.
  • Cardio-vascular: breathlessness, haemorrhages, dysrhythmias, pericarditis, valve problems, pain. Risk of ruptured aneurysms.
  • Neurological: paralysis, strokes (CVAs), transient ischemic attacks (TIAs), memory & concentration impairment, seizures, migraine-type or meningitis-type headaches, double vision, incontinence, impotence, strange sensations, motor impairment ranging from mild clumsiness to vegetative states, personality changes, psychoses.
  • Fatigue: profound persistent exhaustion affecting all activity.
  • Feverishness: effects like having ‘flu, night sweats, bizarre sense of feeling cold or warm.
(Remember no patient has all symptoms! See below for the diagnostic criteria for Behçet’s.)

I marked those who I think are the most prominent overlap with ME/CFS. One can see that there is some kind of overlap (not including some of the neurological and gastro-intestinal symptoms that doubtlessly some of the ME/CFS patients have as well)

If some of primary symptoms of Behçet’s (oral ulcers, eye inflammation) are not pronounced (or in rare cases even oral ulcers can be absent), then it is easy to misdiagnose Behçet’s as ME/CFS.

Again, this shows how utterly important a proper differential diagnosis is in cases of suspected ME/CFS. There are many known diseases out there that can look to the untrained eye like another case of ME/CFS.

Of course there are newer and older diagnostic criteria. These are the newer (and probably better) 2006 ICBD criteria:
Add points for every symptom:
  • Oral aphthosis ("mouth ulcers"): 1 point
  • Skin lesions (e.g. acne): 1 point
  • Vascular lesions: 1 point
  • Positive pathergy test: 1 point
  • Genital aphthosis ("genital ulcers"): 2 points
  • Eye lesions: 2 point
3 points or more? It is most likely Behçet's.
And what are the older diagnostic guidelines for Behçet's?
1. International Study Group strict research level guidelines for diagnosis

Must have:
  • mouth ulcers (any shape,size or number at least 3 times in any 12 months)
Along with 2 out of the next 4 ‘hallmark’ symptoms:
  • genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
  • skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
  • eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
  • pathergy reaction (papule >2 mm diameter, 24-48 hrs or more after needle-prick)
2. Practical clinical guidelines for patients not included in research cohorts

Must have:
  • mouth ulcers
Along with 1 out of the 4 ‘hallmark’ symptoms above

Along with 2 of the following symptoms:
  • arthritis/arthralgia
  • nervous system symptoms
  • stomach and/or bowel inflammation
  • deep vein thrombosis
  • superficial thrombophlebitis
  • cardiovascular problems
  • inflammatory problems in chest and lungs
  • problems with hearing and/or balance
  • extreme exhaustion
  • changes of personality, psychoses
  • any other member of the family with a diagnosis of Behçet’s disease
3. 'Suspected' or 'possible' diagnosis

Usually given when someone does not have mouth ulcers or has mouth ulcers but does not have 1 of the 4 'hallmark' symptoms but has other symptoms and signs of inflammation and other causes for these have been ruled out.
Care must be taken for a differential between ME/CFS and Behçet's. As with all diagnostic criteria:
  • someone who meets some set of diagnostic criteria for a disease, does not necessarily have the disease – and could have another disease instead
  • and someone who barely meets the criteria may actually have the disease (see "suspected diagnosis" above)
regardless of whether it is Behçet's, ME/CFS or any other disease.

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