1. Internist, Rheumatologist
Iraj Salehi-Abari
The ISG, ICBD and ICBD revised Criteria are not good instruments for the diagnosis of Behcet’s Disease
Iraj Salehi-Abari, MD.
Internist, Rheumatologist
Tehran, Iran

2. In the name of God the merciful and compassionate

3. Iraj Salehi-Abari
Case 1:
. A 25 year old man with the first episode of 5 distinct oral aphthosis along with panuveitis y

4. Iraj Salehi-Abari
Case 2:
. A 22 year old woman with the first episode of one major oral aphthosis and one skin aphthosis. The Pathergy test with pustular reaction and HLA-B51 both were positive in this case. y

5. Iraj Salehi-Abari
Case 3:
. A 26 year old man with panuveitis, one dermal aphthosis and positive HLA-B51 test y

6. Iraj Salehi-Abari
Case 4:
. A 30 year old woman with recurrent oral aphthosis, positive Pathergy test with pustular reaction and HLA-B51 positivity y

7. Iraj Salehi-Abari
Case 5:
. A 20 year old woman with first episode of multiple oral aphthosis (one of them was major), along with some erythema nodosum in the skin of leg and pathergy test positivity. y

8. Iraj Salehi-Abari
Case 6:
. A 40 year old man with anterior and posterior uveitis along with retinal vasculitis and HLA-B51 positivity. y

9. Iraj Salehi-Abari
Case 7:
. A 28 year old woman with recurrent oral aphthosis, multiple erythema nodosum and pustular lesions in skin y

10. Iraj Salehi-Abari
Case 8:
. A 24 year old man with the first episode of multiple oral and scrotal aphthosis along with positive Pathergy test with pustular reaction y

11. Iraj Salehi-Abari
Case 9:
. A 35 year old man with the first episode of multiple oral aphthosis (including 2 major aphthae), multiple pustular dermal lesions, arthritis of both ankles and positive Pathergy test y

12. Iraj Salehi-Abari
Case 10:
. A 19 year old woman with anterior and posterior uveitis of both eyes and the first episode of a major aphthae in mouth and HLA-B51 positivity y

13. Clinical/laboratory Judgement:
Iraj Salehi-Abari
Clinical/laboratory Judgement:
. By clinical/laboratory judgement of an expert Rheumatologist all of above cases are involved to Behcet’s Disease (BD) y

14. By application of Classification Criteria:
Iraj Salehi-Abari
By application of Classification Criteria:
. Anyone of the above cases could not be diagnosed as Behcet’s Disease according to the ISG, ICBD and ICBD revised criteria in the initial presentation of them. Finally all of them after several weeks to several months were classified by at least one of those criteria. y

15. Iraj Salehi-Abari
But by application of 2015 Persian Gulf Criteria (PGC) for Behcet’s Disease:
. All of the above cases could be diagnosed as Behcet’s Disease in the initial presentation of them. y

16. International Study Group (ISG) Criteria: 1990
Iraj Salehi-Abari
International Study Group (ISG) Criteria: 1990
. The presence of Recurrent Oral Aphthosis
. Along with at least two of the following items:
. Recurrent Genital Aphthosis
. Ocular lesions: anterior &/or posterior uveitis &/or retinal vasculitis
. Skin lesions: erythema nodosum &/or papulopustular rash &/or pseudofolliculitis
. Pathergy test positivity y

17. International Criteria for Behcet’s Disease (ICBD, 2006):
Iraj Salehi-Abari
International Criteria for Behcet’s Disease (ICBD, 2006):
. Recurrent Genital Aphthosis points
. Ocular lesions: as same as ISG points
. Recurrent Oral Aphthosis point
. Skin lesions: erythema nodosum &/or pseudofolliculitis point
. Vascular lesions: thrombosis &/or aneurysm 1 point
. Pathergy test positivity point
The presence of at least 3 points out of 8 delivers the diagnosis of BD y

18. ICBD revised (ITR-ICBD, 2013):
Iraj Salehi-Abari
ICBD revised (ITR-ICBD, 2013):
. Recurrent Genital Aphthosis points
. Ocular lesions: as same as ISG points
. Recurrent Oral Aphthosis point
. Skin lesions: as same as ICBD point
. Vascular lesions: thrombosis &/or aneurysm 1 point
. Neurobehcet point
. Pathergy test positivity point
The presence of at least 4 points out of 10 delivers the diagnosis of BD y

19. Iraj Salehi-Abari
2015 Persian Gulf Criteria (PGC) for early diagnosis of Behcet’s Disease:
. Oral Aphthosis Up to 2 points
. Genital Aphthosis Up to 2 points
. Ocular lesions Up to 3 points
. Dermal lesions Up to 2 points
. Vascular lesions Up to 1 point
. Pathergy test positivity Up to 2 points
. HLA-B51 positivity Up to 1 point
We need > 4 points out of 13 for diagnosis of BD when upon history and physical examination: no other diagnosis can better explain each criteria y

20. Iraj Salehi-Abari
2015 Persian Gulf Criteria (PGC) for early diagnosis of Behcet’s Disease:
. Oral Aphthosis: Up to 2 points
. Multiple ( > 3) aphthosis point
. Major ( > 1 cm) aphthosis point
. Recurrent ( > 3 n/year) aphthosis points
. Genital Aphthosis: Up to 2 points
. History of aphthosis point
. The scar of aphthosis point
. Observation of aphthosis points y

21. Iraj Salehi-Abari
2015 Persian Gulf Criteria (PGC) for early diagnosis of Behcet’s Disease:
. Ocular lesions: Up to 3 points
. Anterior Uveitis points
. Posterior Uveitis points
. Retinal Vasculitis points
. Dermal lesions: Up to 2 points
. Erythema nodosum point
. Pustular lesions &/or Pseudofolliculitis 1 point
. Skin Aphthosis points y

22. Iraj Salehi-Abari
2015 Persian Gulf Criteria (PGC) for early diagnosis of Behcet’s Disease:
. Vascular lesions: Up to 1 point
. Thrombosis &/or Aneurysm point
. Pathergy test positivity: Up to 2 points
. Papular reaction with peripheral ring erythema 1 point
.Pustular reaction points
. If repeated in dermal lesions, just the highest score is acceptable
. HLA-B51 positivity point
. It can be used when the Pathergy test is negative y

23. Iraj Salehi-Abari
Case I:
. A 29 year old woman with recurrent oral aphthosis along with multiple erythema nodosum, pseudofolliculitis, pustular lesions and aphthosis in skin and HLA-B51 positivity without any other organ involvement y

24. By application of Criteria:
Iraj Salehi-Abari
By application of Criteria:
. The case I can not be diagnosed as a patient with Behcet’s Disease (BD) according to the ISG, ICBD and ICBD revised criteria but she is a case of BD by using the 2015 Persian Gulf Criteria (PGC) for Behcet’s Disease. What is your clinical Judgement about this case? y

25. Iraj Salehi-Abari
Case II:
. A 24 year old man with the first episode of 5 oral aphthosis (including 1 major aphthae) and the first episode of 3 scrotal aphthosis along with multiple erythema nodosum, pseudofolliculitis, pustular lesions and aphthosis in skin and HLA-B51 positivity without any other organ involvement. y

26. By application of Criteria:
Iraj Salehi-Abari
By application of Criteria:
. The case II can not be diagnosed as a patient with Behcet’s Disease (BD) according to the ISG, ICBD and ICBD revised criteria but he is a case of BD by using the 2015 Persian Gulf Criteria (PGC) for Behcet’s Disease. What is your clinical Judgement about this case? y

27. Iraj Salehi-Abari
Case III:
. A 21 year old man with the first episode of 7 oral aphthosis (including 3 major aphthae) and the first episode of 2 scrotal and 1 penile aphthosis along with multiple erythema nodosum, pseudofolliculitis, pustular lesions and aphthosis in skin and oligoarthritis of right knee and both ankles and positive pathergy test with pustular reaction without any other organ involvement y

28. By application of Criteria:
Iraj Salehi-Abari
By application of Criteria:
. The case III can not be diagnosed as a patient with Behcet’s Disease (BD) according to the ISG, ICBD and ICBD revised criteria but he is a case of BD by using the 2015 Persian Gulf Criteria (PGC) for Behcet’s Disease. What is your clinical Judgement about this case? y

29. Iraj Salehi-Abari
Attention please:
. The ISG, ICBD and ICBD revised criteria can be applied for classification of Behcet’s Disease (BD) in the intermediate to late stages of the disease.
. On the other hand, the established BD (may be in advanced phase) can be classified by these criteria for research purposes y

30. Iraj Salehi-Abari
Attention please:
. So, these criteria are not applied for diagnosis and they can not diagnose the Behcet’s Disease in early stage especially in the initial presentation of the disease y

31. Iraj Salehi-Abari
Attention please:
. But, the 2015 Persian Gulf Criteria is applied for diagnosis and it can diagnose the Behcet’s Disease in early stage especially in the initial presentation of the disease y