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Recognize Behçet’s Disease

Diagnosis

According to a study, the average time to a Behçet’s Disease diagnosis is 5.3 years from the the onset of symptoms.1*

Diagnosis is complicated by the many differential diagnoses for symptoms of Behçet’s Disease2-5†

RHEUMATOLOGIC DISEASES

  • Reactive arthritis
  • Seronegative arthropathies

HEMATOLOGIC

  • Cyclical neutropenia  

 

GASTROINTESTINAL DISEASES

  • Inflammatory bowel disease
  • Celiac disease
  • Crohn's disease
  • Ulcerative colitis

AUTOINFLAMMATORY/
AUTOIMMUNE CONDITIONS

  • Familial mediterranean fever
  • Sarcoidosis
  • Multiple sclerosis
  • Systemic lupus erythematosus

INFECTIOUS DISEASES

  • Herpes simplex virus
  • Human immunodeficiency virus
  • Syphilis
  • Tuberculosis

DERMATOLOGICAL DISEASES

  • Bullous skin disorders
  • Recurrent aphthous stomatitis
  • Sweet's syndrome
  • Stevens-Johnson syndrome
  • Acne vulgaris

OTHER

  • Eales disease
  • Other systemic vasculitides
  • SAPHO syndrome

*Study of 661 patients at the Behçet’s Disease units of Akdeniz, Cukurova, Firat, Gazi, Inonu, and Mersin Universities.1
†Not inclusive of all possible differential diagnoses.

THE INTERNATIONAL STUDY GROUP (ISG) CRITERIA CAN AID IN DIAGNOSING BEHÇET’S DISEASE6

NOTE: A DIAGNOSIS CAN ONLY BE MADE BY A PHYSICIAN

RECURRENT ORAL ULCERATION

(aphthous or herpetiform) observed by the physician or patient, recurring at least 3 times in one 12-month period

Plus at least 2 of the following criteria

Recurrent
Genital Ulceration

Aphthous ulceration or scarring observed by physician or patient

Eye Lesions

Anterior uveitis, posterior uveitis, or cells in vitreous on slit-lamp examination

or

Retinal vasculitis observed by ophthalmologist

Skin Lesions

Erythema nodosum observed by physician or patient, pseudofolliculitis, or papulopustular lesions

or

Acneiform nodules observed by a physician in postadolescent patients not on corticosteroid treatment

Positive Result on
Pathergy Testing

Read by a physician at 24 to 48 hours post test

View images of Behçet’s Disease
manifestations HERE

Refer to a rheumatologist for diagnosis

I receive many referrals for patients suspected of having Behçet’s Disease; however, a careful clinical evaluation is needed to rule out other conditions that may mimic some of the features of Behçet’s Disease. In my experience, I have seen a range of misdiagnoses, including recurrent aphthous stomatitis, herpes simplex virus infection, and inflammatory bowel disease, including Crohn’s disease.

img

Amr H. Sawalha, MD

Professor of Internal Medicine
Marvin and Betty Danto Research Professor of Connective Tissue Research
Division of Rheumatology Department of Internal Medicine & Center for Computational Medicine and Bioinformatics
University of Michigan

We don’t know what causes Behçet’s. It’s definitely an inflammatory disease; multiple cytokines have been implicated. There seems to be a genetic component. Several environmental triggers have been proposed, but there isn’t strong evidence for any single specific trigger.

img1

Delfin Santos, MD

American Arthritis and
Rheumatology Associates

The patient journey with undiagnosed Behçet’s Syndrome is frustrating and potentially debilitating. Greater education will help facilitate timely referrals and ultimately a better patient experience.

img2

Andrew J. Sulich, MD

St. John Hospital and Medical Center
Beaumont Hospital – Grosse Pointe
Beaumont Hospital – Dearborn

Suspect Behçet’s Disease?
Refer to a rheumatologist to confirm a diagnosis

Help raise awareness of the
challenges of Behçet’s Disease and
reduce the delay in diagnosis

The most common manifestations of Behçet’s Disease, mucocutaneous lesions, frequently contribute to misdiagnoses.3,4 Sharing the below information via social media will help raise awareness of Behçet’s Disease and may even prevent a delay in its diagnosis.

Share to Raise Awareness

#BehcetsConnection

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Resources

References:

1. Alpsoy E, Donmez L, Onder M, et al. Clinical features and natural course of Behçet’s disease in 661 cases: a multicentre study. Br J Dermatol. 2007;157(5):901-906. 

2. Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol. 2013;9(2):79-89. 

3. Dalvi SR, Yildirim R, Yazici Y. Behcet’s syndrome. Drugs. 2012;72(17):2223-2241. 

4. Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14(2):107-119. 

5. Zeidan MJ, Saadoun D, Garrido M, Klatzmann D, Six A, Cacoub P. Behçet’s disease physiopathology: a contemporary review. Auto Immun Highlights. 2016:7(1):4. 

6. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet. 1990;335(8697):1078-1080. 

7. Senusi A, Higgins S, Fortune F. The influence of oral health and psycho-social well-being on clinical outcomes in Behçet’s disease. Rheumatol Int. 2018;38(10):1873-1883. 

8. Canpolat Ö, Yurtsever S. The quality of life in patients with Behçet’s Disease. Asian Nurs Res (Korean Soc Nurs Sci). 2011;5(4):229-235. 

9. Bernabé E, Marcenes W, Mather J, Phillips C, Fortune F. Impact of Behçet’s syndrome on health-related quality of life: influence of the type and number of symptoms. Rheumatology (Oxford). 2010;49(11):2165-2171. 

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