Behcet's and Sarcoidosis

Region/Prevalence - Behcet’s Syndrome

Middle East, Mediterranean, Far East. High in Turkey (1 in 250 adults)

Region/Prevalence - Sarcoidosis

Worldwide. More common in African Americans (U.S.) and Nordic populations

Cause - Behcet’s Syndrome

Possible genetic factor, Innate and adaptive immune systems

Hyperreactive neutrophils

Cause - Sarcoidosis

Unknown; environmental trigger in genetically predisposed host. Leads to granuloma formation

Age/Gender - Behcet’s Syndrome

Teens to 50s. M = F, but males have more severe disease

Age/Gender - Sarcoidosis

Typically 30–40 years old. F > M under 40. M > F over 40 for eye involvement

Immune Pathology - Behcet’s Syndrome

Neutrophil hyperreactivity, immune system overactivation

Immune Pathology - Sarcoidosis

T-helper cell–driven granulomas (noncaseating)

Systemic Symptoms - Behcet’s Syndrome

Oral ulcers, genital ulcers, skin lesions, joint pain, vascular issues, GI & neuro involvement

Systemic Symptoms - Sarcoidosis

Cough, fatigue, weight loss, skin lesions, and possible involvement of lungs, eyes, heart, CNS, kidneys

Course - Behcet’s Syndrome

Often flares early and improves over time

Course - Sarcoidosis

50% recover in a few years. Others develop chronic disease (>5 years)

Ocular Frequency - Behcet’s Syndrome

Seen in ~50% of cases, especially in males

Ocular Frequency - Sarcoidosis

30% (U.S.); >70% (Japan)

Ocular Signs - Behcet’s Syndrome

Bilateral panuveitis, hypopyon, macular edema. Worse in males/posterior involvement

Ocular Signs - Sarcoidosis

Anterior uveitis, mutton-fat KPs, iris nodules, retinal periphlebitis ('candlewax drippings'), dry eye, glaucoma

Diagnosis - Behcet’s Syndrome

Clinical using ISG Criteria (oral ulcers + 2 others), Pathergy test, HLA-B51 may be positive

Diagnosis - Sarcoidosis

Biopsy showing noncaseating granulomas. Imaging (chest x-ray, CT), labs (ACE, calcium)

Key Test - Behcet’s Syndrome

Pathergy test

Key Test - Sarcoidosis

Chest X-ray (Scadding stages)

Mild Treatment - Behcet’s Syndrome

Topical steroids for ulcers, cycloplegics for anterior uveitis

Mild Treatment - Sarcoidosis

May resolve on its own. Monitor unless symptoms worsen

Moderate/Severe Treatment - Behcet’s Syndrome

Oral steroids, immunosuppressants (azathioprine, infliximab, adalimumab, cyclosporine)

Moderate/Severe Treatment - Sarcoidosis

Steroids (prednisone). Methotrexate for long-term management

Ocular Treatment - Behcet’s Syndrome

Anterior: Cyclopentolate/Atropine + Prednisolone. Posterior: Refer for systemic therapy

Ocular Treatment - Sarcoidosis

Anterior: Cycloplegics + steroids. CME: NSAID + steroid. Posterior: Refer

Referral - Behcet’s Syndrome

Refer to uveitis specialist or internal medicine/rheumatology for systemic disease

Referral - Sarcoidosis

Refer to uveitis specialist, pulmonologist, or internal medicine for systemic disease