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Table 4 Clinical characteristics of juvenile Behçet’s disease patients treated with anti-tumor necrosis factor-alpha therapy and response of the treatment

From: Clinical manifestations and anti-TNF alpha therapy of juvenile Behçet’s disease in Taiwan

Case

Sex

Previous treatments

Duration of steroid use before aTNF (years)

Type of aTNF

Duration of aTNF (years)

Reasons for use of aTNF

Treatment responses

Side effects

#1

F

PD, AZA

7.0

ETN

3.2

Frequent fever, oral ulcer, enteritis. Frequent hospitalizations.

Fever subsided and ESR level returned to normal range within 1 year. Steroid was discontinued for 2.8 years.

One BD flare after tapering ETN.

Herpes zoster, pneumonia

#2

F

PD, 5-ASA, colchicine

3.3

ETN

2.4

Steroid-dependent disease status with oral ulcers and GI bleeding.

Hgb and CRP level returned to normal range within 4 months. Steroid was discontinued 6 months later. One BD flare after tapering ETN

None

#3

F

PD, AZA Colchicine

1.6

ADA

1.0

Recurrent uveitis, oral and genital ulceration.

Improved uveitis and vessel leakage.

VA remained stationary.

None

#4

M

PD, AZA

0.6

ETN

7.8

Refractory oral ulcer, gastrointestinal symptoms.

Improved clinical symptoms; CRP and ESR returned to normal range. PD and AZA were discontinued within 1 year.

Recurrent sinusitis

#5

M

PD, HCQ, CsA, AZA

0.8

ADA

1.4

Steroid-dependent uveitis.

Uveitis subsided with VA improvement. Persistent oral ulcer, high ESR and CRP levels.

None

#6

M

PD, 5-ASA

5.8

ETN

2.7

Neurologic involvement. Steroid dependent disease status with poor drug compliance.

Improved arthralgia, oral ulcer and bloody stool. CRP level returned to normal range. All drugs were discontinued after 6 months.

None

  1. aTNF anti-Tumor Necrosis Factor alpha therapy, PD prednisolone, HCQ hydroxychloroquine, AZA azathioprine, 5-ASA mesalazine, CsA cyclosporine, ETN Etanercept, ADA Adalimumab, ESR erythrocyte sedimentation rate, CRP C-reactive protein, Hgb Hemoglobin, VA visual acuity