Bronchiolitis obliterans organizing pneumonia in a patient with Behçet's disease

A 55-year-old male presented a productive cough, haemoptysis, and intermittent fever for 2 weeks. He was diagnosed as having Behcet's disease (BD) with manifestations of recurrent oral ulcers, genital ulcers, erythema nodosum, a gastro/duodenal ulcer, colon ulcers, and folliculitis. After admission, a chest X-ray showed bilateral ill-defined patches. Subsequent chest computerized tomography revealed multiple patchy consolidations over the left and right lower lobes. Because of poor response to oxacillin antibiotic treatment, an open lung biopsy was arranged. Bronchiolitis obliterans organizing pneumonia (BOOP) was diagnosed. Methylprednisolone pulse therapy, followed by prednisolone and cyclophosphamide maintenance, was prescribed. The patient responded well, with clinical and radiological resolution. Early diagnosis with proper treatment is crucial in the management of this lethal pulmonary disease.     

A case of Behcet's disease with intestinal involvement due to Crohn's disease

A 15-yr-old, Arabic male presented with painful, recurrent, self-resolving oral and genital ulcers, erythema nodosum and uveitis. Behcet's disease was diagnosed. A few months later, he experienced anorexia, abdominal pain, diarrhea, and weight loss. Although all routine laboratory and radiologic investigations were negative, colonoscopy revealed the presence of serpiginous ulcers with pseudopolyps and inflamed intervening mucosa in the proximal half of the colon. Therapy with oral steroids was helpful, but the disease exacerbated a few months after prednisone was discontinued. Repeat evaluation showed similar endoscopic findings and, on colonic biopsy, noncaseating granulomas compatible with Crohn's disease were seen. Again, the patient responded well to oral steroids and sulfasalazine. We believe that gastrointestinal involvement in our patient is compatible with Crohn's disease and that screening tests to rule out chronic inflammatory bowel disease should be performed in the presence of gastrointestinal involvement in Behcet's disease. Behcet's disease may be a part of the spectrum of chronic inflammatory bowel disease.     

Behçet's disease--difficulty in diagnostic and management

We present a 31-year-old female patient with Beh?et's disease. Behcet's disease is a systematic vasculitis of unknown cause involving veins and arteries of all sizes and having reccurent mucocutaneous and frequent ocular involvement. Our patient was suffering from oral and genital ulcers, fever, painful left knee and erythema nodosum. After having excluded differential diagnoses Beh?et's disease was diagnosed. The treatment must be individual and it depends on the presence and severity of symptoms. The optimal improvement was observed after treatment with azathioprine, cyclosporine A and metylprednisolon. Beh?et's disease is very seldom in our country but perhaps sometimes it isn't good diagnosed.     

Pulmonary arterial aneurysms in Behçet's syndrome. Report of 4 cases

Pulmonary involvement in Behcet's disease is uncommon and pulmonary arterial aneurysms are present in about 1 to 7% of patients. But despite their scarcity, they represent a life-threatening complication and are usually regarded as of a poor prognosis. Our study aims to describe the epidemiological and therapeutic aspects of this condition. Among 180 patients with Behcet's disease according to the criteria of the international study group for Behcet's disease, four were diagnosed as having pulmonary artery aneurysms (2.22%). All were male, the mean age at the time of the diagnosis of pulmonary artery aneurysms was 29.8 years and the mean disease duration was 6.6 years. Hemoptysis was the presenting symptom in all cases. The patients were treated by corticosteroid, colchicine and cyclophosphamide pulses. The outcome was good with disappearance of hemoptysis in all cases and disappearance of pulmonary aneurysms in three. One patient had recurrence of aneurysms 40 months after the first episode and died 18 months later. So, we can hope, thanks to medical treatment, a better outcome than previously reported in this complication.     

Embolic stroke with a patent foramen ovale and Behcet's disease

We report a case of a 53-year-old man with Behcet's disease (BD) and paradoxical stroke. This patient suddenly developed sensory aphasia, right oculomotor palsy, nystagmus, and left ataxic hemiparesis. MR images showed fresh embolic infarcts in the midbrain and the left parietotemporal lobe. He presented with uveitis, oral aphthous ulcers and genital ulcers. An electrocardiogram revealed no abnormalities, and a transesophageal echocardiogram disclosed a patent foramen ovale. Since venous involvement is common in BD, we suspected the presence of smaller thrombi probable in this patient. We conclude that embolic stroke is a notable complication of BD with right-to-left shunting.     

Intracardiac thrombus and pulmonary aneurysms in an adolescent with Beh?et disease

Behçet disease (BD) is an inflammatory disorder of unknown origin. We present here an unusual case of juvenile Behçet with hemoptysis due to large pulmonary artery aneurysms (PAA), large intra-cardiac thrombus and prolonged fever, which posed several therapeutic challenges. In this case, a 14-year-old boy was admitted with a 3-month history of fever, painful oral ulcers, skin rash and intermittent hemoptysis. A high resolution helical computed tomography angiogram demonstrated thrombi in the right ventricle, two large aneurysms located in the right lung and two smaller ones in the left. The patient was successfully treated with colchicine, prednisone, cyclophosphamide and enoxaparine. A discussion about PAA and intracardiac thrombi and their role in BD is provided in this case.     

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.     

IgA nephropathy in a patient with Behcet's syndrome--case report and review of literature

A 25-yr-old Turkish patient who presented with oral and genital ulcers, erythema nodosum and peripheral arthritis was diagnosed as having Behcet's syndrome (BS). The patient had normal renal function, but persistent proteinuria with 3 g protein loss/day. Renal biopsy revealed glomerulonephritis (GN) with IgA deposits in the membrane. Co- occurrence of BS and IgA nephropathy (IgAN) has only been reported once to date. In contrast to the statements in most current textbooks on BS, the review of the literature presented here suggests that renal involvement is not infrequent, but serious kidney disease is a rather rare event in Behcet's disease.      

Macular hole in Behcet's disease

Behcet's disease is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Ocular involvement occurs in 60-80% of patients with Behcet's disease and presents as panuveitis in most cases. Posterior segment involvement may lead to irreversible alterations and significant vision loss. The development of a partial or full-thickness macular hole, though rarely reported, may cause serious vision loss. In this report, we present two cases of macular hole in the worse eye of bilateral cases of Behcet's disease, and discuss the possible mechanisms and management in such cases.     

Esophageal varices in association with unilateral pulmonary vein atresia

A 27-yr-old woman with recurrent episodes of hemoptysis (and hematemesis due to esophageal varices) was found to have unilateral pulmonary vein atresia. Reversed flow In the left pulmonary artery, a finding highly suggestive of this rare congenital anomaly, was demonstrated during cardiac catheterization. The definitive diagnosis was afforded by pulmonary wedge angiography, which not only demonstrated the atretic leftsided pulmonary veins, but also revealed a serpiginous system of systemic collateral veins to be the cause of her esophageal varices. Pneumonectomy of the nonfunctioning hypoplastic lung, the most successful approach to this disorder, may be complicated by severe bleeding of the numerous systemic arterial collateral vessels transacted during surgical mobilization of the affected lung. Percutaneously delivered vascular occlusion coils were used in this case to occlude the large systemic to pulmonary artery collateral arteries immediately prior to pneumonectomy. Intra- and perioperative bleeding was minimal, and the patient has done well without further episodes of hemoptysis or hematemesis. Percutaneous occlusion of systemic to pulmonary collaterals may prove useful as a preoperative step for other congenital heart disease patients requiring lung or heart/lung transplantation. © 1996 Wiley-Liss, Inc.     

A case of primary racemose hemangioma of bronchial artery with recurrent hemoptysis]

A 42-year old man was admitted to our hospital because of hemoptysis. Bronchial arteriography revealed a tortuous and dilated left bronchial artery with a shunt formation between the bronchial and pulmonary arteries. Bronchial artery embolization using a sponge was performed three times to treat the hemoptysis, but all attempts failed. The patient therefore underwent left lower lobectomy, after which no hemoptysis was observed. Histopathologically, the resected tissue showed no inflammatory change. Interestingly, abnormal vessels resembling arteriovenous malformations were also found. Although the embolization therapy was effective in several reported cases, we concluded that surgery was required for this patient with persistent hemoptysis because of the development of collaterals and a bronchial-pulmonary artery shunt.     

Esophageal Involvement in Behcet''s Disease

Esophageal involvement in Behcet's disease is very uncommon, only 21 cases have previously been reported. We have observed five cases with this disease, and investigated the clinical, morphological, and histological aspects of these cases. Esophageal lesions in Behcet's disease occurred more frequently in male patients with the incomplete type. Laboratory data revealed an elevated erythrocyte sedimentation rate and a strongly positive C-reactive protein. The middle portion of the esophagus was most commonly involved. There were several morphological forms of esophageal lesions including erosions, perforated ulcers, widely spreading esophagitis, and severe stenosis. Therefore, there was no unique form to the esophageal lesions in contrast to the ileocolonic ulcers in intestinal Behcet's disease. Histological examination revealed lymphocytic or neutrophilic infiltration in ulcerative lesions. Vasculitis was rarely observed.     

Severe unilateral pulmonary vascular changes in child with polysplenia

A 12-year-old boy with polysplenia and single ventricle experienced recurrent episodes of pneumonia, hemoptysis, and pulmonary hypertension. Unilateral pulmonary vein obstruction was diagnosed, and a left pneumonectomy was performed. Microscopy of the resected specimen revealed pulmonary veno-occlusive disease in the small pulmonary venules, and old arteritis in the small pulmonary arteries.     

Observation of bleeding sites by fiberoptic bronchoscopy in a case of vasculo-Beh?et's disease with pulmonary involvement

A 45-year-old man complained of fever and bloody sputum. Chest X-ray films showed a round opacity in the left hilum. He developed massive hemoptysis (500 ml) and emergency embolization of the bronchial artery was performed. Pulmonary artery aneurysm and thrombosis were detected angiographically, and fiberoptic bronchoscopy revealed a reddish irregular eminence of the left main bronchus and lingulate++ ++ bronchus. These sites could have been the bleeding sites. Because of recurrent hemoptysis, left pneumonectomy was performed 26 days after the massive hemoptysis. Pathological findings showed panarteritis, luminal thromboembolism of the pulmonary artery and markedly dilated vessels of the bronchial wall. He developed genital ulceration after the operation and these findings suggested incomplete vasculo-Beh?et's disease with rare pulmonary involvement. The disease is now well-controlled by corticosteroid therapy 27 months after the operation.     

Post coital hemoptysis

Ischemic mitral regurgitation is a complication of coronary artery disease specifically in the settings of prior myocardial infarction. It is a dynamic lesion and its severity may increase with different forms of exercise leading to flash pulmonary edema. We present the case of an 81-year-old man with ischemic mitral regurgitation who presented with long standing history of hemoptysis limited to sexual intercourse only. After a thorough negative work-up for the usual causes of hemoptysis, comprehensive right and left cardiac catheterization studies revealed marked increase in baseline mitral regurgitation and pulmonary capillary wedge pressure with isometric exercise. This case illustrates that sexual intercourse, a form of isometric exercise, can cause severe increase in ischemic mitral regurgitation and more so compared to other forms of exercise due to heightened sympathetic activity, leading to flash pulmonary edema and hemoptysis.     

Massive lower gastrointestinal bleeding as a presenting feature of Behcet's disease

This reports a 27 yr old female Patient with seronegative polyarthritis who presented with lower GI bleeding and subsequently developed evidence of cutaneous vasculitis (palpable purpura) and orogenital ulcers. The cause of the GI bleeding was multiple ulcerations in the terminal ileum, cecum ascending colon and transverse colon. Continued and massive bleeding necessitated surgical resection of the involved segment of gut which on histopathological examination confirmed the presence of vasculities. This patient was thus diagnosed as having Behcet's disease. Although major GI bleeding is a rare manifestation of Behcet's disease with vasculitis of the gut, one must consider this possibility if the patient has multi-system involvement with evidence of vasculitis involving the skin in the form of palpable purpura.     

Multiple pulmonary arterial aneurysms in Behçet's disease: clinical and radiologic remission after cyclophosphamide and corticosteroid therapy

Beh?et's disease (BD) with pulmonary arterial aneurysm is rare and often associated with a poor prognosis. But there is also a chance that the aneurysm may completely resolve with medical therapy. A 39-year-old man presented with fever, malaise, bilateral chest pain, recurrent oral ulcers, and hemoptysis. The chest radiograph showed a round opacity in the right hilum. Computed tomography and magnetic resonance angiography (MRA) further revealed multiple, bilateral pulmonary arterial aneurysms consistent with the diagnosis of BD. The patient was started on a course of cyclophosphamide and corticosteroid therapy that resulted in cessation of his symptoms and complete resolution of radiologic findings. The chest radiograph and MRA reverted to normal on long-term follow-up. He is still alive and symptom-free 20 months after diagnosis.     

Successful treatment of pulmonary artery aneurysms secondary to possible Behçet's disease

Pulmonary artery aneurysm (PAA) secondary to Beh?et's disease (BD) is a rare condition. The commonest presentation is hemoptysis, which can be fatal. Though the classical triad of recurrent oral and genital ulcers and relapsing iritis is present in most patients of BD, isolated pulmonary artery involvement termed as incomplete BD has been reported. Prompt diagnosis and immunosuppressive therapy can cause regression of aneurysm and prevent fatal hemoptysis. We report a case of PAA due to BD who presented with hemoptysis and responded to steroid therapy.     

Massive gastrointestinal bleeding due to aneurysmal rupture of ileo-colic artery in a patient with Behcet's disease]

Behcet's disease has been recognized as a systemic vasculitis characterized by the involvement of multiple organs such as orogenital ulcers, eye lesions including uveitis and optic neuritis, and skin lesions including folliculitis and erythema nodosum. Vascular involvement occurs occasionally and is classified into thrombosis and aneurysm. However, massive gastrointestinal bleeding from arterial aneurysm is a rare manifestation of intestinal Behcet's disease. Recently, we experienced a case of intestinal Behcet's disease presenting with massive gastrointestinal bleeding due to aneurysmal rupture of ileo-colic artery. A 30-year-old male with Behcet's disease was admitted because of massive gastrointestinal bleeding. A large ileo-cecal ulcer was revealed as a bleeding focus on colonoscopic examination. Celiac angiography showed aneurysm and stenosis of ileo-colic artery. After the failure of hemostasis with arterial embolization, ileocecectomy was performed. After the resection hematochezia was completely stopped.