Papilledema

A 33-year-old Moroccan man was admitted to the hospital with complaints of shortness of breath, hemoptysis, and right-sided pleuritie chest pain. There was a 20-kg weight loss over 4 preceding months. He was initially seen elsewhere a year earlier with a dry hacking cough and recurrent orogenital ulcers. He reported recurrent mouth and penile ulcers along with red eyes for more than 2 years. He had no fever or night sweats or joint pains, and he denied recent sexual contact.

On presentation, he appeared weak and had difficulty answering questions. He was afebrile. There were depigmented patches of skin on the face, penis, and scrotum. He had bilateral papilledema. Auscultation of the chest revealed bibasal eraeldes. Results of the cardiac and abdominal examination were normal. There was no lymphadenopathy.

The erythroeyte sedimentation rate was 70 mm/h. The chest radiograph showed a right hilar density (Fig 1).

[Figure 1 ILLUSTRATION OMITTED]

What is the diagnosis?

Diagnosis: Behcet's syndrome (BS) with pulmonary artery aneurysm (PAA) and venous thrombosis.

The clinical impression was B S with PAA. This was confirmed with CT scans (Fig 2) and MRI scans of the chest (Fig 3) which showed a 2-cra fight PAA, a 1.5- to 2.0-cm peripheral aneurysm in the lower lobe of the left lung, and multiple ill defined nonspecific small peripheral lung opacities probably representing small areas of infarction. Magnetic resonance angiography of the brain (Fig 4) showed thrombosis of superior sagittal and left transverse sinuses and the proximal left internal jugular vein.

[Figures 2-4 ILLUSTRATION OMITTED]

Therapy was started with oral prednisolone and azathioprine.

DISCUSSION

The clinical triad of uveitis and oral and genital ulcers was recognized by Hippocrates, but bears the name of Hulusi Behcet who described it nearly 60 years ago. The disease is systemic, with variable involvement of many organ systems. The cause is unknown and there is no universally accepted diagnostic test.

ETIOLOGY AND PATHOGENESIS

BS is a multisystem vasculitis of unknown cause. It affects virtually all sizes of arteries and veins. It also may be accompanied by thromboembolism of the superior vena cava and mediastinal veins. The pulmonary vascular tree is involved with significant morbidity. BS, of all nonspecific vasculitides, is the most common cause of PAAs, which frequently rupture and cause devastating hemorrhage.[1,2] In a case series of 2,179 patients, 24 patients were found to have PAAs.[3] There was a high prevalence of thrombophlebitis. In 1959, Hughes and Stovin reported the Hughes-Stovin syndrome, which consists of major venous thrombosis and PAA.[4] Clinical and histologic similarities suggest that Hughes-Stovin syndrome may be a variant of BS.[5]

The pulmonary arteries are frequently involved during exacerbations of BS. The presence of a PAA carries a poor prognosis, leading to death in 30% of the patients, with 80% of these occurring within 2 years of the diagnosis.[6,7] Pulmonary parenchymal involvement is associated with severe restrictive physiology. Pleurisy, recurrent pneumonitis, pulmonary fibrosis, obstructive airway disease, and pulmonary tuberculosis have also been described in association with BS. The incidence of pleuropulmonary involvement is 16%[8]

DIAGNOSIS

BS has a strong male predominance. The International Study Group for Behcet's Disease proposed the following five criteria for the diagnosis:[9]

1. Recurrent oral ulceration: minor aphthous, major aphthous, or herpetiform ulcers which have recurred 3 times in a 12-month period.

Plus any two of the following:

2. Recurrent genital ulceration noticed by physician or the patient.

3. Eye lesion: anterior or posterior uveitis, cells in vitreous on slitlamp examination, or retinal vasculitis observed by an ophthalmologist.

4. Skin lesion: erythema nodosum or papulopustular lesion.

5. Positive pathergy test read by physician at 24 to 48 h. (Positive pathergy test is the development of cutaneous nodular pustules at the site of trauma, including needle stabs, after approximately 24 h.)

The diagnosis of PAA usually is made by noninvasive imaging, and MRI scan is probably the imaging modality of choice. An increased incidence of aneurysm formation at the puncture site has been reported after pulmonary angiography, and various studies have shown an exacerbation of hemoptysis and disease activity following this procedure.[7,10,11] The erythrocyte sedimentation rate correlates with disease activity. The acute phase response is associated with an increase in C-reactive protein and elevated plasma components such as C3, C4, C9, and factor V. Reduced fibrinolytic activity is seen in BS and usually is due to decreased production of tissue plasminogen activator. However, this appears to be unrelated to the tendency to develop thrombosis. The histologic characteristics of orogenital ulcers show predominantly lymphocytic and monocytic infiltration of the dermis and epidermis as well as around small vessels.

PROGNOSIS AND TREATMENT

In one large study,[3] 50% of patients died at a mean of 9.5 months after the onset of hemoptysis, implying that PAA carries a high short-term mortality despite treatment. The aneurysms may regress or even completely disappear with corticosteroid treatment.[3,5,12] Various treatment modalities have been described with variable results. These include surgery, embolization of the aneurysm, immunosuppressive drugs such as cyclophosphamide, azathioprine, and cyclosporine alone or in combination with steroids.

Surgical resection of aneurysms should be considered cautiously due to the presence of microscopic disease in normal appearing pulmonary artery and the multiple nature of the aneurysms. If surgical intervention is indicated, it should be followed with a postoperative combination of corticosteroids and immunosuppressive drugs. These patients seem to do better than those with corticosteroid therapy alone.[13] However, there are no controlled clinical trials to show the superiority of one regimen over the others.

Young men with BS and venous thrombosis are at an increased risk of having a PAA. Therefore, those with lung lesions compatible with PAAs should be treated with cytotoxic drugs even when they are asymptomatic.

REFERENCES

[1] Efthimou J, Johnson C, Spiro S, et al. Pulmonary disease in Behcet's syndrome. Q J Med 1986; 227:259-90

[2] Erkan F, Cavder T. Pulmonary vasculitis in Behcet's disease. Am Rev Respir Dis 1992; 142:232-39

[3] Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysm in Behcet's syndrome: a report of 24 cases. Br J Rheumatol 1994; 33:48-51

[4] Hughes J, Stovin P. Segmental pulmonary artery aneurysms with peripheral venous thrombosis. Br J Dis Chest 1959; 53:19-27

[5] Duriex P, Bletry O, Huchon G, et al. Pulmonary artery aneurysm in Behcet's and Hughes-Stovin syndrome. Am J Med 1981; 71:736-41

[6] Raz I, Okon E, Chajek-Shaul T. Pulmonary manifestations in Behcet's syndrome. Chest 1989; 95:585-89

[7] Tunaci A, Yahya M, Gokmen E. Thoracic involvement in Behcet's disease: pathological, clinical, and imaging features. AJR Am J Roentgenol 1995; 164:51-6

[8] Abdullah N, Al-Dalaan, Sulaiman R, et al. Behcet's in Saudi Arabia. J Rheumatol 1994; 19:402-10

[9] Criteria for diagnosis of Behcet's disease: International Study Group for Behcet's Disease. Lancet 1990; 335:1078-80

[10] Hamza M. Large artery involvement in Behcet's disease. J Rheumatol 1987; 14:554-59

[11] Koc Y, Gulu I, Akpek G. Vascular involvement in Behcet's disease. AJR Am J Roentgenol 1992; 19:402-10

[12] Grenier P, Bletry O, Cornud F, et al. Pulmonary involvement in Behcet's disease. AJR Am J Roentgenol 1981; 137:565-69

[13] Huong DLT, Weschler B, Papo T, et al. Arterial lesions in Behcet's disease: a study in 25 patients. J Rheumatol 1995; 22:2103-13

(*) From the Section of Pulmonary and Critical Care Medicine, Department of Medicine, West Virginia University School of Medicine, Robert C. Byrd Health Sciences Center, Morgantown.

Manuscript received January 22, 1997; revision accepted April 3.

Reprint requests: Marvin R. Balaan, MD, FCCP, Section of Pulmonary and Critical Care Medicine, Department of Medicine, Room 3306 Health Sciences Center, Robert C. Byrd Health Sciences Center, West Virginia University, Morgantown, WV 26506-9166

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