The Respiratory System in Autoimmune Vascular Diseases

Abstract

The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

Pleuropulmonary manifestations of systemic autoimmune diseases: An 84-case series analysis

“…Most characteristic findings were opacities of different appearance from nodular masses to illdefined areas of consolidation, both of which cavitated. This finding is highly suggestive of GPA [28,29]. A series of our patients with GPA showed difference in radiological features of the lung changes when compared with the other reported series [29].…”

“…Goodpasture syndrome in one patient manifested with alveolar opacities. Diffuse, bilateral, and low-density patterns in vasculitis corresponded to diffuse hemorrhage and capillaritis on pathologic examinations [28,30,31]. Enlarged sample size could examine these findings in the future.…”

Chronic chest pain: Where is the pathology?

“…9 However, any large vessel may be affected, including the PA. Involvement of the PA may lead to the development of pulmonary nodules, pleural effusions, aneurysms, thromboses, perfusion defects and pulmonary hypertension, and presents with nonspecific chest pain, dyspnea, cough, or hemoptysis secondary to pulmonary hemorrhage. [10][11][12][13] Although not common, isolated PA involvement has been reported. 10,12 Consistent with this patient's presentation, pleural effusions have been described as the initial presentation of TA given the close proximity of the left PA to the parietal pleura.…”

Upper respiratory tract involvement in patients with ANCA-associated vasculitis

“…Микроскопический полиангиит. МПА преимущественно поражает мелкие сосуды почек и легких и не сопровождается развитием гранулематозного воспаления или эозинофилии [33]. По данным Е.М.…”

“…При поражения гортани под голосовыми складками и трахеи стратегия ведения пациентов заключается в использовании трех компонентов лечения: системная ОБЗОР ЛИТЕРАТУРЫ КЛИНИЧЕСКАЯ ФАРМАКОЛОГИЯ И ТЕРАПИЯ, 2019, 28 (4) иммуносупрессивная терапия, антибактериальные и антигрибковые препараты, а также местная терапия [98]. К последней относятся баллонная дилатация, лазерная терапия и криотерапия, аргоноплазменная коагуляция, диатермокоагуляция, местное использование ГКС, митомицина С и алемтузумаба (моноклонального антитела к антигену лимфоцитов CD52) [33].…”