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 salehiabari@sina.tums.ac.ir

In the name of God the merciful and compassionate

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

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

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

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

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

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

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

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

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

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

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

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

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

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

International Criteria for Behcet’s Disease (ICBD, 2006): Iraj Salehi-Abari International Criteria for Behcet’s Disease (ICBD, 2006): . Recurrent Genital Aphthosis 2 points . Ocular lesions: as same as ISG 2 points . Recurrent Oral Aphthosis 1 point . Skin lesions: erythema nodosum &/or pseudofolliculitis 1 point . Vascular lesions: thrombosis &/or aneurysm 1 point . Pathergy test positivity 1 point The presence of at least 3 points out of 8 delivers the diagnosis of BD y

ICBD revised (ITR-ICBD, 2013): Iraj Salehi-Abari ICBD revised (ITR-ICBD, 2013): . Recurrent Genital Aphthosis 2 points . Ocular lesions: as same as ISG 2 points . Recurrent Oral Aphthosis 2 point . Skin lesions: as same as ICBD 1 point . Vascular lesions: thrombosis &/or aneurysm 1 point . Neurobehcet 1 point . Pathergy test positivity 1 point The presence of at least 4 points out of 10 delivers the diagnosis of BD y

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

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

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

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

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

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

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

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

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

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

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

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

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