Are Behçet’s disease patients with Budd–Chiari syndrome at increased risk for the development of pulmonary hypertension?

syndrome (BCS) is a severe type of vascular involvement in Behc¸et ’ s disease with a prevalence of 2.4 – 14.4% [1, 2]. Portal hypertension is a well-known cause of pulmonary arterial hypertension (PAH). PAH due to portal hypertension has a poor prognosis; survival of these patients is less than 50% at 5 years [3]. Behc¸et ’ s disease patients with BCS may be at increased risk for the development of PAH with their vulnerable endothelium [4]. In this study

DEAR EDITOR, Budd-Chiari syndrome (BCS) is a severe type of vascular involvement in Behc¸et's disease with a prevalence of 2.4-14.4% [1,2].Portal hypertension is a well-known cause of pulmonary arterial hypertension (PAH).PAH due to portal hypertension has a poor prognosis; survival of these patients is less than 50% at 5 years [3].Behc¸et's disease patients with BCS may be at increased risk for the development of PAH with their vulnerable endothelium [4].In this study, we retrospectively investigated the frequency of pulmonary hypertension (PH) in Behc¸et's patients with BCS.
Twenty-five Behc¸et's patients with BCS among 902 patients from the Behc¸et's disease database of Hacettepe University, Rheumatology Department were included in the study.Patients' data including demographic and clinical features, were obtained retrospectively from hospital records.All BD patients with BCS fulfilled the International Study Group for Behc¸et's disease criteria [5].The first and last transthoracic echocardiogram (TTE) results of the Behc¸et's patients and serum haemoglobin levels at the times of TTE evaluations were also noted.PH was considered if estimated systolic arterial pressure was !40 mmHg by TTE.
The frequency of BCS in Behc¸et's patients was 25/902 (2.77%).Nineteen (76%) patients were male, and the mean age was 43.8 (12) years.The median follow-up time for Behc¸et's disease was 13.9 (range 9.6-22.1)years and for BCS was 5.5 (range 2.4-12.1)years.In three patients the diagnosis of BCS was made before the diagnosis of Behc¸et's disease, and in three patients the diagnosis of BCS and Behc¸et's disease were made simultaneously.The median time to BCS in patients with a previous diagnosis of Behc¸et's disease was 7.6 (range 14) years.One patient (6.25%) developed cirrhosis after BCS.Sixteen (64%) out of 25 patients had at least one TTE evaluation after the diagnosis of BCS.Among 25 patients, nine (36%) patients had dyspnoea at initial presentation.Dyspnoea was present in seven (43.8%) of those who underwent TTE evaluation and in two (22.2%) of those who did not (P ¼ 0.4).Patient characteristics of those evaluated by TTE are shown in Table 1.The median estimated sPAP of the BCS patients was 30 mmHg (range 25-35).The sPAP of nine (56.25%) patients was reported to be !30mmHg.One patient had an estimated sPAP of 40 mmHg and one other had low ejection fraction.Only six patients had a control TTE, in whom the median sPAP was 25 mmHg (range 25-31.3).The median values of haemoglobin measured simultaneously at the first and last TTE were 13.4 (±2.4) and 12.6 (±2.1) mg/dl, respectively.Two patients (12.5%) died during follow-up, but the exact cause of death could not be determined.
In this study, although many of the patients with BCS had mild elevations in estimated sPAP by TTE at presentation or during follow-up, there were no patients with severe PH leading to right heart failure.Sudden cardiac death is a complication of severe PH [6].In our study two patients died during follow-up, for whom we could not determine the exact cause of death.Although hepatic failure is considered to be the major cause of mortality in Behc¸et's patients with BCS, these two patients had PH but did not have advanced liver disease [2].On the other hand they had serious comorbidities such as left heart failure or chronic renal failure.
It is hard to determine the exact cause of PH in systemic diseases such as Behc¸et's disease.Severe group IV PH due to pulmonary arterial involvement (PAI) that needs to be treated by endarterectomy or PAH specific agents can occur in Behc¸et's disease [7,8].Moreover these patients are at increased risk for the development of PH due to cardiac involvement or drugs that are used frequently to control the disease activity such as IFN-a or cyclophosphamide [8].As Rheumatology key message

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expected, there were five patients who had both BCS and PAI in our study [1].The presence of more than one clinical condition associated with PH development is not rare in systemic diseases.Portal hypertension due to BCS may contribute to the development of severe PH in this patient group by leading to an increased exposure of pulmonary circulation to vasoactive substances [9].
Our study has some limitations.This was a retrospective study, and none of the patients were evaluated by right heart catheterization, which is the gold standard for PH diagnosis.To define the presence of PH, we used only sPAP values and did not present data about the right ventricle.
A significant number of patients with Behc¸et's disease and BCS had dyspnoea, though there were none with severe PH in our study.It seems reasonable to include TTE in the workup of Behc¸et's disease patients with BCS at presentation and at least follow them up clinically for the signs of PH both for therapeutic decision making and increasing data about the topic.
a Symptom: findings before the first transthoracic echocardiogram.b