28.1. Behc¸et Disease
Also known as the Silk Road disease, Behçet disease (BD) has a worldwide distribution but is seen most frequently in Turkey, Japan, China, and the Mediterranean littoral. Men and women are affected equally. The disease is a triad of oral ulcers (Figure 28.1a, b), genital ulcers, and uveitis (Figure 28.2a, b, c). In a study consisting of 2179 patients, pulmonary involvement occurred in about 1.1%. Pulmonary vascular lesions lead to the formation of pulmonary artery aneurysms, the most common pulmonary lesion in BD almost always associated with hemoptysis, thrombosis and occlusion of the vessels, pul- monary infarction, and hemorrhage (Figure 28.3). Symptoms include dyspnea, cough, hemoptysis, and pleural effusion. Pathological changes include arterial aneurysm, arterial and venous thrombosis, infarction, and vasculitis (Figures 28.4 and 28.5).
Current treatments of BD are topical, paraocular, and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide, and chlorambucil. Patients with small nonspecific radiologic abnormalities should be fol- lowed up closely because early diagnosis of vascular lesions may be life-saving. Immuno- suppression is the main therapy for the treatment of a vasculitis, and it is important that it is not mistaken for pulmonary thromboembolic disease because fatalities have occurred in BD shortly after initiation of anticoagulation/thrombolytic treatment.
28.2. Veno-Occlusive Lung Disease
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension and interstitial disease that mainly affects children and young adults. PVOD is more common in men in contrast with primary pulmonary hypertension, which is more common in women. The predominant lesion is intimal fibrosis of small veins and venules (Figure 28.6). Changes in the arterial side cause muscular hypertrophy of the circulation and can result in pulmonary edema and alveolar hemorrhage. In about 50% of the patients, pulmonary arteries are affected.
Most cases of PVOD are idiopathic, and several theories have been proposed. Known causes of secondary PVOD include bleomycin, vincristine, cisplatin, carmustine, mito- mycin, radiation, appetite-suppressant drugs, and herbal preparations. The most common symptom of PVOD is dyspnea, initially on exercise. Most of the patients have fatigue and sometimes sensation of fainting at the time of diagnosis; fewer have orthop- nea and paroxysmal dyspnea. Progression of the disease leads to narrowing of the pulmonary veins, pulmonary hypertension, congestion, and pulmonary edema. Chest radiographs show enlarged pulmonary arteries and in some cases mild-to-moderate peripheral interstitial infiltrates or septal lines (Figure 28.7). Chest CT scan reveal the presence of ground-glass opacities (particularly with a centrilobular distribution), septal lines, and adenopathy (Figure 28.8).
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156 Clinical Atlas of Interstitial Lung Disease
Figure 28.1. Behc¸et disease: Characteristic aphthous ulcers under the tongue (a). Aph- thous ulcers inside the mouth (b).
Figure 28.2. Ocular lesions occur in more than half of the cases of Behc¸et disease;
frequently bilateral, they can eventually lead to blindness. The figure shows the poste- rior sinechiae; the obliteration of the lens and the iris obviating dilatation of the iris (a). Retinal vein occlusion. This occurred secondary to periphlebitis (b). The retina can also be involved in the form of vasculitis associated with lekage of inflamed retinal vessels (c).
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Figure 28.3. Chest x-ray film in a Japanese patient with Behc¸et disease showing pul- monary artery dilatation and aneurysm. (Courtesy Dr. Sonoko Nagai, Kyoto University, Japan.)
Figure 28.4. A lung biopsy specimen showing vasculitis with perivascular inflamma- tion (small artery stained for elastic tissue) and lumen totally occupied with organized thrombus, which is partially recanalized, typical of Behc¸et disease.
Figure 28.6. Y-shaped vein with partial duplication of elastica and thickening of intima.
Figure 28.5. Elastica stain shows fragmentation of the vessel wall with degenerative changes in the elastic lamina. Small vessel is totaly filled with the clot (thrombus).
Figure 28.7. Chest x-ray film in a patient with veno-occlusive disease of the lung showing bilateral, soft patchy infiltrate.
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The differential diagnosis of PVOD includes pulmonary parenchymal diseases, con- nective tissue disorders, HIV infection, recurrent pulmonary thromboembolism, and primary pulmonary hypertension (Table 28.1). PVOD is often difficult to distinguish from severe primary pulmonary hypertension. The treatment of primary pulmonary hypertension with prostacyclin can be fatal in patients with veno-occlusive disease, and an early pretreatment diagnosis is critical.
Bibliography
1. Evereklioglu C. Current concepts in the etiology and treatment of Behcet disease. Surv Ophthalmol 2005;50:297–350.
2. Uzun O, Akpolat T, Erkan L. Pulmonary vasculitis in Behcet disease: A cumulative analysis. Chest 2005;127:2243–2253.
3. Veeraraghavan S, Koss M, Sharma, OP. Pulmonary veno-occlusive disease. Curr Opin Pulm Med 1999;5:310–313.
4. Resten A, Maitres S, Humbert M, Rabiller A, Sifbon O, Capron F, Simmoneau G, Musset D. Pulmonary hyper- tension: CT of the chest in pulmonary venooclusive disease. AJR Am J Roentgenol 2004;183:65–70.
Figure 28.8. CT scan of the chest of the same patient as in Figure 28.7 showing diffuse nodular infiltrate.
Table 28.1. Differences between primary pulmonary hypertension and pulmonary veno-occlusive lung disease.
Features Primary Pulmonary Hypertension Veno-occlusive Disease
Age Adults Children, young adults
Sex Common in women Common in men
Chest radiograph Prominent pulmonary arteries Large pulmonary arteries, Kerley-B lines, hilar adenopathy Computed tomography No centrilobular nodules or septal Centrilobular nodules, septal lines, adenopathy
thickening
Vasodilators May be useful May cause pulmonary edema
Prognosis Median survival 30 months Most patients die within 2 years of diagnosis
Lung transplantation Effective Effective
