Pulmonary artery aneurysm in Behcet’s disease: a case report

The pulmonary artery is the second most common site of arterial involvement in Behcets disease. A 32-year-old man presented with bilateral ankle edema, abdominal discomfort, and hemoptysis. He had a history of recurrent oral and genital aphthous ulcerations for 1 year. The diagnosis of Behcets disease was made on the basis of the criteria published by the International Study Group for Behcets Disease. His chest X-ray revealed left hilar enlargement. A helical computed tomography (CT) scan showed a pulmonary aneurysm with intramural thrombosis in the left pulmonary artery and enlarged hepatic veins. Treatment with colchicine and cyclophosphamide was given for 24 months, and helical thoracic CT was performed again. Helical CT showed that the pulmonary aneurysm was reduced by treatment. Helical CT could be used in Behcets disease for the diagnosis and follow-up of pulmonary involvement.     

Huge femoral artery pseudoaneurysm in a patient with Behçet’s disease

Background

Behçet’s disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement.

Clinical features of Behcet’s disease in Egypt

Behcet's disease (BD) is a chronic relapsing multisystem disease of unknown etiology. Ethnic origin is one of the factors that may modulate the prevalence and the expression of BD. To study the clinical characteristics of Egyptian patients with BD, and compare the pattern of the disease in Egyptians with studies from other countries. Sixty-three patients with BD were studied over a 4-year period. A rheumatologist, dermatologist, neurologist and other specialists as indicated assessed the patients clinically. Laboratory and radiological examinations were done to confirm the diagnosis to rule out any condition that may mimic BD. Sixty-one patients were men, two were women, the mean age of the patients was: 32.8 +/- 8.3 years, age of onset of the disease varied between 17 and 37.4 years. The initial presenting manifestation was oral ulcers in 39.7% of patients, followed by orogenital ulcers in 23.8%, followed by deep venous thrombosis in 7.9% Throughout the study period, the commonest manifestation was oral ulcers (100%), followed by genital ulcers (96.8%), vascular lesions (57.1%), cutaneous (55.5%), ocular (47.6%), joint (36.5%), neurological (34.9%), gastrointestinal (19%) and cardiac (6.3%). BD in Egyptians shows higher male-to-female ratio and higher incidence of vascular and neurological lesions.     

Behcet’s disease and IgA nephropathy

Although Beh?et’s disease (BD) is a kind of systemic disease, renal involvement is rare, especially IgA nephropathy (IgAN). Renal manifestations in BD range from mild urinary abnormalities to glomerulonephritis with persistent renal failure, which includes minimal change disease, proliferative glomerulonephritis, rapidly crescentic glomerulonephritis, renal amyloidosis and IgA nephropathy. Amyloidosis seems to be the most common type of renal lesion in BD, and several cases of nephrotic syndrome secondary to amyloidosis have been documented. Co-occurrence of BD and IgA nephropathy has only been reported in only few cases. We describe two patients with the rare association of BD and IgAN. We suggested that it is important to periodically perform renal function assessment in patients with BD, through urinalysis and measurement of serum creatinine for detecting any abnormality and providing an early adequate treatment.     

Cardiac complications of Behcet’s disease

We describe two patients with Behcet’s disease (BD) with cardiac complications and their response to treatment. This report adds to the evidence that cardiac involvement can be a feature of Behcet’s disease and may be more common than previously thought.     

Pulmonary artery aneurysms in Behçet’s disease

Behçet’s disease is the most common cause of pulmonary artery aneurysms. Pulmonary artery aneurysms are rare, but they are life-threatening because of their high tendency to rupture. However, there is also a chance that the aneurysms may completely resolve with immunosuppressive therapy. A 30-year-old man was admitted with chest pain, painful oral and genital ulcers, skin rash, weakness, and intermittent hemoptysis. He had a history of Behçet’s disease for 6 years. The chest radiography showed a round opacity superior part of hilus on right. The helical thoracic computed tomography (CT) angiography demonstrated pulmonary aneurysms associated with Behçet’s disease. The patient was successfully treated with colchicine, corticosteroids, and cyclophosphamide. A discussion about pulmonary artery aneurysms associated with Behçet’s disease is provided in this case.     

Behcet’s disease and cardiovascular involvement: our experience of asymptomatic Behcet’s patients

Behcet’s disease is generally defined by oral and genital ulcers and uveitis. It is also known as a recurrent multisystemic and inflammatory disease. It is mostly seen in Mediterranean countries and the Far East.The aetiology of Behcet’s disease is associated with viral, toxic, bacterial and immunological factors. It was defined in 1963 as an auto-immune disease caused by auto-antibodies against the oral mucosa. Vascular involvement is 2–7% and it is usually seen in patients between the ages of 20 and 40 years.Behcet’s disease is a non-specific arterial and venous vasculitis.1-8 Proximal and distal anastomotic aneurysm formation after surgery is not rare one to 12 months postoperatively. Recurrent surgical interventions increase the risk of mortality and morbidity.9,10 Cardiovascular involvement in Behcet’s disease includes pericarditis, coronary arterial disease, cardiomyopathy and valvular dysfunction.11 The aim of this study was to report our experience of cardiovascular involvement with asymptomatic Behcet’s disease.     

Large pseudoaneurysm of the carotid artery in Behçet's disease

Behçet''s disease is a multisystem inflammatory disease of unknown cause. Its major pathologic feature is vasculitis. We report the occurrence of a large pseudoaneurysm of the carotid artery in a patient who had Behçet''s disease. The patient underwent surgery and was discharged on the 7th postoperative day.Ours is only the 4th such case reported in the English-language medical literature. Endovascular and surgical options are discussed herein.Key words: Aortic valve prosthesis, durable medical equipment, endocarditis, bacterial, heart valve prosthesis, human, male, postoperative complications, reoperation, time factorsBehçet''s disease is seen most often in young adults of Mediterranean or Asian descent. It is characterized by relapsing uveitis, and oral and genital ulcerations. The typical vascular feature is medium- and large-vessel involvement, which causes both arterial occlusive disease and aneurysm formation. The cessation of nutrient flow to the arterial wall due to inflammatory obliteration of the vasa vasorum can lead to perforation or pseudoaneurysm formation. The incidence of large arterial complications ranges from 2% to 6% in patients from 20 to 40 years of age with Behçet''s disease.^1,2We report the case of a man with Behçet''s disease who had a pseudoaneurysm of the right carotid artery.     

Comparison of different onset ages in patients with Behcet’s disease

Clinical Rheumatology -     

Clinical characteristics of conduction disturbance in patients with Behcet’s disease

Clinical Rheumatology - To summarized clinical features of Behcet’s patients with conduction disturbances and explore the underlying risk factors. A retrospective case-control study was...     

Aneurysm of the internal carotid artery in a female patient of Behcet’s disease: a rare presentation

Behcet’s disease is a systemic inflammatory disease characterized commonly by oral and genital ulcerations, with skin and eye involvement. Unlike other manifestations, involvement of the central nervous system and vascular manifestations can be late in the course and are the leading causes of death. Aneurysms are relatively common in visceral vessels but extremely rare in intracranial arteries, being limited to few case reports. Moreover, vascular involvement is significantly less common in female than in male patients. In this paper, we report a case of aneurysm of internal carotid artery in a female patient of Behcet’s disease who had presented with subarachnoid hemorrhage and was treated successfully by a brief course of corticosteroids followed by endovascular treatment.     

Serum levels of natriuretic peptides in patients with Behcet’s disease

The objective of our study was to elucidate serum levels of atrial natriuretic peptide (ANP), brain natriuretic peptide (BNP), and C-type natriuretic peptide (CNP) in Behcet’s disease (BD) patients with active and inactive period. The multicenter study included 53 patients with active (n = 28) and inactive (n = 25) BD (mean age, 34.3 ± 9 years; 15 men and 38 women) satisfying the International Study Group criteria and 26 healthy controls (mean age, 34.4 ± 6.1 years; seven men and 19 women) matched for age and gender from a similar ethnic background. Serum natriuretic peptides levels were determined by enzyme immunoassay kit. Mean serum ANP concentrations in the active patients (4.01 ± 1.21 ng/ml) were significantly lower than in the healthy controls (5.76 ± 1.99 ng/ml, p = 0.004). Mean serum BNP levels were found to be significantly higher in both the active (6.19 ± 2.97 ng/ml) and inactive (6.49 ± 2.88 ng/ml) BD groups compared with the control group (3.82 ± 1.1 ng/ml, p = 0.004 and p = 0.001, respectively). Mean serum CNP concentrations in the active patients (0.49 ± 0.12 ng/ml) were significantly lower than in the inactive patients (0.65 ± 0.2 ng/ml, p = 0.017) and the healthy controls (0.8 ± 0.27 ng/ml, p < 0.001). Our results suggest that changes in natriuretic peptide levels may be associated with vasculitis that play role in the etiopathogenesis of the BD.     

Genital ulcers in acute Epstein-Barr virus infection mimicking Behcet’s disease

Clinical Rheumatology -     

Behcet’s disease: from east to west

Behcet's disease (BD) is classified among vasculitides. There are actually five nationwide surveys of BD: Iran, Japan, China, Korea, and Germany. Among case series, four are on more than 200 cases (Turkey, Morocco, Tunisia, and UK). BD was classically seen around the Silk Route. Now, it is seen everywhere. The male to female ratios were, respectively (in nationwide surveys), 1.19, 0.98, 1.34, 0.63, and 1.40 to 1. The mean age at onset was 26.2, 35.7, 33.8, 29, and 26 years. Major manifestations were seen, respectively, in nationwide surveys: mucous membrane (oral aphthosis in 97%, 98%, 98%, 99%, and 98%; genital aphthosis in 65%, 73%, 76%, 83%, and 64%); skin manifestations (pseudo-folliculitis in 57%, N/A, 31%, N/A, and 62%; erythema nodosum in 22%, N/A, 38%, N/A, and 42%; ocular manifestations in 55%, 69%, 35%, 51%, and 53%). Minor manifestations were seen, respectively, in nationwide surveys: joint manifestations in 33%, 57%, 30%, 38%, and 53%; neurological manifestations in 9%, 11%, 6.5%, 4.6%, and 11%; gastrointestinal manifestations in 7%, 15.5%, 9%, 7.3%, and 12%; vascular involvement in 8.9%, 8.9%, 7.7%, 1.8%, and 13%; pulmonary manifestations in 0.3%, N/A, 2.2%, N/A, and 3.6%; cardiac manifestations in 0.5%, N/A, 4%, N/A, and 3.2%. Laboratory tests are not useful except the pathergy test, which was positive in 54%, 44%, N/A, 40%, and 34% of cases. ESR was normal in many patients. Diagnosis is based upon clinical manifestations. The International Criteria for Behcet's Disease (ICBD, 2006) may be of help, having a sensitivity of 98.2% and a specificity of 95.6% in Iranian patients.     

Clinical features of Behcet’s disease in Mongolia: a multicenter study

Clinical Rheumatology - The aim of the present study is to investigate the clinical features of patients with Behcet’s disease (BD) in Mongolia. Patients were identified and examined from six...     

Scoping beyond pulmonary artery involvement; pulmonary involvement in Behcet’s disease; a retrospective analysis of 28 patients

Clinical Rheumatology - Pulmonary involvement other than pulmonary artery involvement in Behcet’s disease still remains an area of investigation. The aim of this study was to evaluate...     

Endoscopic findings of gastrointestinal involvement in Chinese patients with Behcet’s disease

AIM:To report the incidence,clinical features and outcomes of gastrointestinal(GI)involvement in Behcet’s disease(BD).METHODS:A total of 168 consecutive patients with BD were screened and upper and lower GI endoscopies were performed in 148 patients.Four hundred age-and sex-matched controls were enrolled for comparison.RESULTS:Fifty-two(35.1%)patients had GI lesions.After a mean follow-up of 10 mo,ileocecal ulcers had been confirmed in 20 patients,including active ulcer(s)in 18 patients,but no ileocecal ulceration was found in controls.GI symptoms were present in 14 patients with active ulcer(s),while 4 patients with smaller ulcer were asymptomatic.Endoscopic features of ileocecalulcer were:a single ulcer(50%),larger than 1 cm in diameter(72.2%),and round/oval or volcano-type in shape(83.3%).Compared with patients without GI involvement,less ocular lesions,lower levels of albumin,erythrocyte count and hemoglobin,and higher levels of C-reactive protein and erythrocyte sedimentation rate were confirmed in the intestinal BD group.Four patients had esophageal ulcers in the BD group but no case in controls.The other endoscopic findings were similar between the two groups.The prevalence of Helicobacter pylori infection was similar in both groups.Most patients received an immunomodulator and responded well.CONCLUSION:GI lesions commonly occur in Chinese BD patients.The most frequently involved area is the ileocecal region.Esophageal ulcer might be a rare but unique lesion.     

Hpylori infection in patients with Behcet＇s disease

AIM To evaluate endoscopic findings and the prevalence of H pylori in patients with Behcet's disease (BD) who have upper gastrointestinal symptoms.METHODS The patients with BD diagnosed according to the International Study Group and followed up in the Department of Dermatology and other related departments and who had any upper gastrointestinal complaints, were included in this study. Forty-five patients with BD and 40 patients in the control group were evaluated by upper gastrointestinal endoscopy and two biopsied specimens were taken during endoscopy for H pylori. A two-week triple therapy for H pylori eradication was administered to H pylori positive patients. Two months after the treatment, the patients were evaluated by urea-breath test for eradication control.RESULTS Patients with BD had a mean age of 36.2 ± 11.4 years (18-67 years). The mean follow-up time was 35 ± 14 mo (16-84 mo). Aphthous or deep ulcer in esophagus, stomach and duodenum had never been confirmed by endoscopic examination. Most gastric lesions were gastric erosion (40%) and the most duodenal lesions were duodenitis (17.5%) in two groups.H pylori was positive in 33 patients (73.3%) with BD.The two-week triple eradication therapy was successful in 75% of the patients. There was no difference between the groups in respect to prevalence of H pylori(73.3% vs 75%, P ＞ 0.05), and eradication rate (75% vs 70%, P ＞ 0.05).CONCLUSION Endoscopic findings, eradication rate and prevalence of H pylori were similar in patients with BD and control group.     

Coronavirus disease 2019 in patients with Behcet’s disease: a report of 59 cases in Iran

Clinical Rheumatology - To present the clinical characteristics, disease course, management, and outcomes of COVID-19 infection in patients with Behcet’s disease (BD). In this retrospective...