Cyclophosphamide, rituximab may prevent subglottic stenosis relapses

Researchers in Italy reviewed medical records of 105 people with GPA

Margarida Maia, PhD avatar

by Margarida Maia, PhD |

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Immunosuppressants may help prevent subglottic stenosis — a condition wherein part of the windpipe becomes tight — from returning in people with a type of ANCA-related vasculitis called granulomatosis with polyangiitis (GPA), a small Italian study suggests.

The study, “Role of systemic immunosuppression on subglottic stenosis in granulomatosis with polyangiitis: Analysis of a single-centre cohort,” was published in the European Journal of Internal Medicine.

In GPA, the immune system mistakenly attacks blood vessels. This can cause inflammation and damage to various organs, most often the lungs, the kidneys, and the nose, ears, and throat.

One complication of GPA is subglottic stenosis, which occurs when there is a narrowing of the airway just below the vocal cords. Its symptoms include a dry cough and difficulty speaking and breathing. The condition is more common among young women.

To treat subglottic stenosis, doctors often use an endoscopic dilation to widen the airway. Subglottic stenosis can come back even after the procedure, however.

Researchers in Italy investigated if immunosuppressants, a type of medication that suppresses the immune system, could help prevent a relapse with GPA. They looked at the medical records of 105 people with GPA, and identified 21 (20%) with subglottic stenosis. Thirteen (62%) were women and the mean age at disease onset was 30.2, with a median 3.4 years from diagnosis to subglottic stenosis.

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Preventing subglottic stenosis relapse

Patients with subglottic stenosis were significantly younger at their diagnosis than those without the condition (30.2 vs. 47.3). They also scored a median three points lower in the Birmingham Vasculitis Activity Score (BVAS), a measure of disease activity where a lower score indicates milder disease (10.5 vs. 13.5 points).

Five patients didn’t receive any immunosuppressants to treat their subglottic stenosis and all relapsed after having an endoscopic dilation. The median number of procedures was two and the median time to a relapse was 14 months.

Among those who received immunosuppressants, almost half (44%) remained free of a relapse and didn’t require a second endoscopic dilation procedure. The proportion was higher for those on cyclophosphamide (78%) or the antibody rituximab (70%).

All six patients who didn’t require an endoscopic dilation were on cyclophosphamide, an anti-cancer medication that works as an immunosuppressant.

Patients with subglottic stenosis and generalized disease, those who received either cyclophosphamide or rituximab to induce disease remission, and those with higher cumulative doses of glucocorticoids had a median three times longer period before a relapse (36 vs. 12 months).

While subglottic stenosis “is highly prevalent in patients with GPA,” it “may define a milder systemic disease subset occurring more frequently in younger patients,” the researchers wrote, noting people “who develop [subglottic stenosis] during the course of GPA undergo an endoscopic dilation procedure or other invasive interventions, often repeatedly over time.”

“Systemic [whole body] immunosuppression provides benefit in preventing recurrence of [subglottic stenosis] in GPA patients,” the researchers said, adding that “cyclophosphamide or rituximab might have a non-redundant role in this setting.”