HARD DECISIONS IN INTENSIVE CARE UNIT

Intensivecareunitkapak

PULMONARY VASCULITIS

Songül Binay

Dr. Abdurrahman Yurtaslan Ankara Oncology Training and Research Hospital, Department of Internal Medicine Intensive Care, Ankara, Türkiye

Binay S. Pulmonary Vasculitis. In: Turan S, editor. Hard Decisions in Intensive Care Unit. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.23-37.

ABSTRACT

Vasculitis is a rare disease that progresses with inflammation of the vessel walls and presents a clinical picture according to the location and size of the affected vessel. It is a heterogeneous group of diseases clinicopathologically and is classified according to the size of the affected vessel (large vessel, me dium vessel, or small vessel). Pulmonary vasculitis involves inflammation in the pulmonary vessels, affecting vessels of varying diameters from large vessels to capillary vessels. The vasculitis that most commonly involves and affects the lungs is the capillary vessels. In the diagnosis of pulmonary vascu litis, the clinician should evaluate the patient’s clinic, radiology, laboratory findings, and pathological data. Antineutrophil cytoplasmic antibody (ANCA)associated vasculitides, including granulomatosis with polyangiitis (Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomato sis with polyangiitis (ChurgStraus syndrome), are the most common pulmonary vasculitis. Screen ing for the presence of ANCAs is a widely used diagnostic test for ANCAassociated vasculitis. An tineutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers for the diagnosis of welldefined types of smallvessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The diagnosis of ANCAassociated vasculitis should not be ignored in ANCAnegative patients, and biopsies of the affected organs should be performed in these sero negative patients. Although ANCAs are helpful in the diagnosis of ANCAassociated vasculitis, the diagnosis should be based on clinicopathologic features. Typically, vasculitis can present with a vari ety of clinical symptoms that are difficult to diagnose because they are characterized by nonspecific symptoms. Patients with pulmonary involvement often present with hemoptysis, cough, dyspnea, and asthma symptoms. Radiologically, chest Xray (CXR) is generally not specific for pulmonary vasculi tis because of its low sensitivity and specificity, and the potentially serious consequences of pulmonary involvement can often be missed if not diagnosed early. In pulmonary vasculitis, highresolution com puted tomography (HRCT), contrastenhanced computed tomography or magnetic resonance imaging are important radiological methods that help measure and detect the type of lung involvement, the spread and distribution of the disease, and changes in the vessels (systemic thrombi, pulmonary artery occlusions caused by embolism or thrombi, especially multiple aneurysmal dilatations in the bronchial and pulmonary arteries). Immunosuppressive agents are the mainstay of treatment in the treatment of vasculitis. However, these treatments may also bring immunosuppressantrelated complications. Therefore, the optimum treatment should be balanced between the risk of the disease and the risk of the treatment. Vasculitis treatment is twostage. It is organized as remission induction and maintenance treatment. While the drugs used for induction treatments are more aggressive and aim to bring the disease under rapid control, remission is of lower intensity and is continued for a longer period of time in order to prevent relapse of the disease.

Keywords: Vasculitis; Pulmonary vasculitis; Difficult cases; Immunosuppressant therapy; Antineutrophil cytoplasmic antibody

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