Takayasu arteritis and interstitial lung disease: a case report and literature review

Introduction and importance: Although pulmonary artery involvement is well recognized, the incidence of interstitial lung disease (ILD) with Takayasu arteritis is very rare. The pathophysiology of ILD in Takayasu is still incompletely understood, in contrast to several studies establishing the relationship between ANCA-associated vasculitis and ILD. The management of this patient involved a multidisciplinary approach with long-term follow-up.

Case presentation: The authors present a case of HRCT-proven interstitial lung disease in a patient with Takayasu arteritis and heart failure. The patient was on long-term corticosteroids on and off for several years and recently developed progressive dyspnea with a dry cough. After reviewing her history and physical examination, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) were performed, and interstitial lung disease was diagnosed. The patient was managed by a team of pulmonologists, rheumatologists, and cardiologists and gradually improved after adjustment of medications, including corticosteroids and mycofenolate, and via long-term oxygen therapy.

Clinical discussion: Takayasu arteritis is a rare form of systemic vasculitis that can involve the pulmonary vasculature, such vasculitis with associated parenchymal involvement is rare. ILDs have been demonstrated with ANCA-associated vasculitis; however, whether the pathophysiology applies to Takayasu is unknown. Since Takayasu can be debilitating to the patient, the association of ILDs can have further prognostic implications. Given that no established guidelines exist to address this association, management is based on clinical expertise.

Conclusion: The authors report a case of Takayasu arteritis and associated ILD and its pharmacological management. Takayasu arteritis is a very uncommon type of vasculitis, and pulmonary parenchymal involvement further contributes to this case's rarity. As the management of Takayasu arteritis alone is cumbersome, the addition of another significant comorbidity, such as ILD, can pose several threats to the patient. Given the rarity of this association, no established guidelines exist, making clinical expertise crucial for managing such patients. Further research is needed to explore the underlying mechanisms and develop evidence-based treatment strategies for this rare combination.