544 Vasculitic Subglottic Stenosis: A Question of Immunosuppression?

Introduction Subglottic stenosis (SGS) is the commonest manifestation of tracheobronchial disease in granulomatosis with polyangiitis (GPA) and carries a high degree of morbidity. Management of SGS-GPA is a double-edged sword. Delayed treatment may cause respiratory compromise and infectious complications. However, aggressive surgical management may initiate a systemic inflammatory response, reactivating the vasculitic cascade and potentially lead to long-term complications including renal vasculitis and consequential end-stage renal failure. There is currently no internationally agreed management strategy for this disease. Method This retrospective review was undertaken to analyse our unique combination of surgical dilatations and immunosuppressive-focused adjuvant management strategy between years 2011-2020. Results Sixteen of our one hundred and nine GPA patients (14.7%) had SGS and were included in our analysis. Whilst three patients (18.8%) improved solely on medical treatment, thirteen (81.3%) required combined surgery and immunosuppression (consisting of cyclophosphamide or Rituximab regimens). Thirty-nine surgical dilatations and two tracheostomies were performed over a mean 53-month follow-up period, with a calculated mean procedure rate of one every 24.8 months (2.7 - 89 months). Conclusions Our current management strategy affords a lower procedure rate at every 24.8 months compared to other published studies with combined procedure rate at every 14.9 months.

A CASE REPORT ON ILD-NSIP, PULMONARY ARTERIAL HYPERTENSION IN ASSOCIATION WITH SJOGRENS'S SYNDROME

Sjogren syndrome with various pulmonary symptoms and the common manifestations are diffuse parenchymal lung disease and tracheobronchial disease. Association with ILD-NSID, pulmonary embolism, pulmonary hypertension are rare. We experienced a case of ILD-NSIP accompanied with sjogren’s syndrome and are treated further and we present the details of the case here in.

Pulmonary manifestations of Sjögren's syndrome

In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

Wegener's granulomatosis presenting as acute systemic vasculitis following 20 years of limited tracheobronchial disease

Objective:We report a patient with a 20-year history of apparently idiopathic airways stenoses, who presented with an antineutrophil cytoplasmic antibody (ANCA) associated, acute, systemic vasculitis with necrotising glomerulonephritis, subsequently diagnosed as Wegener's granulomatosis.Methods:We present a case report and a review of the world literature on airway stenosis in Wegener's granulomatosis.Results:To our knowledge, this is the first report of Wegener's granulomatosis manifesting as local airway disease for such a prolonged period, before transforming into a systemic vasculitis.Conclusions:This case highlights the need for physicians to be alert to the possibility of Wegener's granulomatosis as a cause of apparently idiopathic airway stenosis, and to be aware that systemic disease may occur in very long-standing, limited Wegener's granulomatosis.