Table 1.

Overview of morbidity and mortality of different organ manifestations in BS.

	Mortality		Morbidity	References
	Mortality rate (%)	Median follow up time (y)
Behçet syndrome (all manifestations)	5–10	7.7–20	Increased mortality in major vessel and CNS disease young (15–24 year) and male patients, with high number of disease flares with worse outcome and high unemployment and dependence rate	(17, 18)
Vascular	8	7.7	35.4% of patients with recurrent vascular events	(8, 18)
Venous	6–6.5	4.75–7.7		(18, 19)
Deep venous thrombosis (DVT)	3	4.75	Severe post-thrombotic syndrome (in 50% of patients) venous claudication (in 30% of patients) most common type of recurrent vascular manifestation	(8, 11, 19)
Thrombosis of vena cava	12	4.75		(19)
Budd Chiari syndrome (BCS)	18–47	4–9	Concomitant inferior vena cava thrombosis is common sequelae: portal hypertension, liver cirrhosis, hepatic failure, lower extremity edema poor prognosis in liver failure	(13, 19–21)
Arterial	13–14	7.7	45.6% undergo surgery of which 34% had surgical complications (mainly prosthetic thrombosis, less frequent if immunosuppressants are applied)	(18, 22)
Pulmonary artery aneurysm (PAA)	26–50	1–7	Anticoagulation can worsen hemoptysis frequent thrombus formation within PAA poor prognosis with highest mortality rate in BS	(23–26)
Pulmonary artery thrombosis (PAT)	23	7	PAT can transform/progress into PAA pulmonary artery hypertension (in up to 50% of patients)	(23, 24)
Extrapulmonary arteries	4–17	4	High frequency of new aneurysms frequent graft obstruction (well-tolerated due to collateral formation)	(27, 28)
Cardiac*	15–28	3	Poor prognosis in coronary artery involvement (reduced cardiac function in 2/3 of patients) frequent relapse of pericarditis	(12, 29)
CNS	7–12	7.7–20	Worse prognosis in parenchymal involvement and abnormal CSF findings approximately 50% of patients with moderate to severe disability by 10 years	(17, 18, 30–32)
Parenchymal	11–21	4–20	44% one attack and remission 28% attacks with secondary progression 10% primary progression 21% silent neurological involvement 25–33.9% disabled or dead factors associated with poor outcome (disability or death): baseline hemiparesis or paraparesis and brainstem involvement	(17, 30–32)
Vascular	7	4.75	Good short-term outcomes predominant sequel: optic nerve atrophy/reduced visual acuity	(19, 33)
Gastrointestinal	2–5	5–7.5	Cumulative operation rate (5 years) 32% remission or mild clinical activity 72% multiple relapses/chronic symptoms 28%	(34, 35)
Musculoskeletal	6	7.7	Mainly non-erosive, but impact on quality of life	(18, 36, 37)

^*Pericarditis, valve insufficiency, coronary artery involvement.