From the Guidelines
The latest management guideline for subglottic stenosis in pediatric patients with Granulomatosis with Polyangiitis (GPA) recommends treating with immunosuppressive therapy over surgical dilation with intralesional glucocorticoid injection alone, according to the 2021 American College of Rheumatology/Vasculitis Foundation guideline 1.
Key Recommendations
- For patients with GPA and actively inflamed subglottic and/or endobronchial tissue with stenosis, immunosuppressive therapy is conditionally recommended as the initial treatment, which usually comprises glucocorticoids and other agents 1.
- Surgical dilation with intralesional glucocorticoid injection may be more appropriate for stenoses that are longstanding, fibrotic, or unresponsive to immunosuppression 1.
- Subglottic or endobronchial stenoses should be managed by an otolaryngologist or pulmonologist with expertise in management of these lesions 1.
Treatment Approach
- The treatment approach involves a multidisciplinary team, including an otolaryngologist with expertise in treating GPA, to determine the best course of treatment 1.
- Immunosuppressive therapy is recommended for initial treatment of active inflammatory stenoses, with the degree of immunosuppressive therapy utilized based on the severity of other disease manifestations 1.
- Regular monitoring with pulmonary function tests and endoscopic evaluation is essential, with treatment adjustments based on disease activity 1.
Important Considerations
- Collaboration with an otolaryngologist with expertise in treating GPA is crucial to determine whether interventions such as nasal rinses, topical nasal therapies, and reconstructive surgery are beneficial 1.
- Reconstructive surgery should be performed after a period of sustained remission, by an otolaryngologist with expertise in treating GPA 1.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Subglottic Stenosis in GPA in Pediatric Patients
- Subglottic stenosis (SGS) is a known complication of granulomatosis with polyangiitis (GPA) that may result from active disease or from chronic recurrent inflammation 2.
- The leading symptoms of SGS are dyspnoea on exertion and stridor, and SGS presents high morbidity 2.
- In pediatric patients, SGS is among the most common causes of airway obstruction, with 90% of cases resulting from endotracheal intubation 3.
Latest Management Guideline and Treatment
- The therapeutic approach depends on the severity of the SGS and the patient's symptoms 3.
- Endoscopic balloon dilatation of SGS has been successfully used in children, providing a promising alternative to tracheostomy 4.
- Systemic therapy, such as leflunomide, may increase dilation intervals in patients with GPA-associated SGS 5.
- A multidisciplinary approach is required for the diagnosis and management of SGS/TS, including the use of glucocorticoids and methotrexate 6.
- Despite treatment, relapses of stenoses are common, and relapse rates do not differ between causes 6.
Treatment Options
- Tracheal dilatation may provide symptomatic relief, but recurrences may occur 2.
- Tracheostomy may be necessary in cases of severe airway-limiting stenosis 2.
- Endoscopic procedures, such as balloon dilatation, may be required in a significant proportion of patients 6.
- Immunomodulatory therapy, such as leflunomide, may be beneficial in increasing dilation intervals and reducing the frequency of surgical interventions 5.