Recognize Behçet’s Disease

Diagnosis

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

Diagnosis is complicated by the many differential diagnoses for symptoms of Behçet’s Disease^1-3,†

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.
^†Not inclusive of all possible differential diagnoses.

THE INTERNATIONAL STUDY GROUP (ISG) CRITERIA CAN AID IN DIAGNOSING BEHÇET’S DISEASE⁴

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 posttest

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.

Amr H. Sawalha, MD

Professor of Internal Medicine
Marvin and Betty Danto Research Professor of Connective Tissue Research
Division of Rheumatology
University of Michigan

We don’t know what causes Behçet’s Disease. 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.

Delfin Santos, MD

Director of the Rochester Rheumatology Center
Rochester Hills, Michigan

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.

Andrew J. Sulich, MD

Rheumatologist
St. John Hospital and Medical Center
Beaumont Hospital – Grosse Pointe, Michigan

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 quality of life for a person with Behçet’s Disease can be impacted by a myriad of symptoms. Unfortunately, obtaining an accurate diagnosis can add to the challenge of living with this disease.^5,6 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

References:

1. Alpsoy E, Donmez L, Onder M, et al. Br J Dermatol. 2007;157(5):901-906.

2. Barnes CG. History and diagnosis. In: Yazici Y, Yazici H, eds. Behçet's Syndrome. Springer; 2010:7-33.

3. Ambrose NL, Haskard DO. Nat Rev Rheumatol. 2013;9(2):79-89.

4. International Study Group for Behçet's Disease. Lancet. 1990;335(8697):1078-1080.

5. Diagnosis of Behcet's Disease. American Behcet's Disease Association website. behcets.com. Accessed October 4, 2018.

Recognize Behçet’s Disease

Diagnosis

Diagnosis is complicated by the many differential diagnoses for symptoms of Behçet’s Disease1-3,†

RHEUMATOLOGIC DISEASES

HEMATOLOGIC

GASTROINTESTINAL DISEASES

AUTOINFLAMMATORY/ AUTOIMMUNE CONDITIONS

INFECTIOUS DISEASES

DERMATOLOGICAL DISEASES

OTHER

RECURRENT ORAL ULCERATION

Recurrent Genital Ulceration

Eye Lesions

Skin Lesions

Positive Result on Pathergy Testing

Refer to a Rheumatologist for Diagnosis

Amr H. Sawalha, MD

Delfin Santos, MD

Andrew J. Sulich, MD

References:

Diagnosis is complicated by the many differential diagnoses for symptoms of Behçet’s Disease^1-3,†

AUTOINFLAMMATORY/
AUTOIMMUNE CONDITIONS

Recurrent
Genital Ulceration

Positive Result on
Pathergy Testing