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Behçet Syndrome

Stephen D. Kasteler, MD and Christopher M. Edens, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • Behçet syndrome (BS) represents a chronic, relapsing, multisystem, autoinflammatory disorder.

    • Precise etiology remains incompletely understood; strong but diverse genetic component

    • Cli...

DIAGNOSIS

HISTORY

  • Key symptom: Recurrent aphthous stomatitis (RAS) is the most frequent initial presenting symptom (65–70%); present in 97–100% during disease course, occurs in all three clinical subty...

TREATMENT

MEDICATION

  • Due to the complex pathogenesis and incomplete understanding of BS (including subtypes), there are no widely accepted treatment algorithms. Generally, treatment is centered on immu...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Depends on severity of system involvement and medication monitoring

  • Periodic ophthalmologic examinations

DIET

No special diet, although oral ulcers...

REFERENCES

Oǧuz AK, Yılmaz ST, Oygür ÇŞ, et al. Behçet's: a disease or a syndrome? Answer from an expression profiling study. PLoS One.  2016;11(2):e0149052.  [View Abstract]
Bulur I, Onder M. B...

ADDITIONAL READING

  • Caso F, Costa L, Rigante D, et al. Biological treatments in Behçet's disease: beyond anti-TNF therapy. Mediators Inflamm.  2014;2014:107421.

  • Chams-Davatchi C, Barikbin B, Shahra...

CODES

ICD10

  • M35.2 Behcet's disease

  • N77.0 Ulceration of vulva in diseases classified elsewhere

ICD9

  • 136.1 Behcet's syndrome

  • 616.51 Ulceration of vulva in diseases classified elsewhere

SNOMED

  • 310701003 Behcet'...

CLINICAL PEARLS

  • BS is a clinical diagnosis.

  • Consider BS in patients presenting with recurrent oral and genital ulcers.

  • Treatment depends on severity of disease and medication side effect profile.

  • Refer e...

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