Follicular Bronchiolitis: Clinical and Pathologic Findings in Six Patients

Abstract

The purpose of this study was to review our experience with patients who had a definitive diagnosis of follicular bronchiolitis (FB), and to describe in detail the clinical and pathological findings, looking for common clinical aspects that may help to identify this entity. Ours is a community 750 bed teaching hospital that acts as a tertiary referral center for several subspecialties, including thoracic surgery. Six patients with a morphological diagnosis of FB, defined by the presence of coalescent germinal centers adjacent to airways, were included. Lung biopsy was obtained by thoracotomy in all patients (2 women and 4 men, mean age 53 years). In one patient FB was associated with advanced AIDS, and in another with prolonged exposure to polyethylene-flock. In 4 patients no condition previously associated with FB was found. Five patients had a history of repeated respiratory infections, 3 patients complained of dyspnea and none had peripheral blood eosinophilia. After a mean follow-up of 25 months, 2 patients responded well to steroid therapy; 3 patients suffered symptomatic exacerbations that required an increase in the steroid dose and 1 patient was not treated with steroids. The most important contribution of this series is the description of a subset of patients with FB who were not associated with other processes. These patients present relatively homogeneous clinical and pathological pictures that do not differ greatly from secondary forms.

Successful Colchicine Therapy in a Patient With Follicular Bronchiolitis Presumed to Be Asthma

“…The therapeutic effect is thought to be related to the ability of colchicine to disrupt microtubules and to concentrate in leukocytes, especially granulocytes, where it can reach high concentrations, even at low oral doses. Neutrophilic inflammation has been reported in FB cases, [8][9][10] as in our case. This neutrophilic inflammation can predispose to a colchicine response.…”

“…6 FB is a disease of exacerbations, complicated by infections. Steroid therapy has clinical benefit in half of all patients, 8 but was not effective in our patient. Therefore, colchicine treatment was initiated, followed by improved clinical findings, pulmonary function tests, and radiological images without side effects.…”

Follicular Bronchiolitis: A Literature Review

“…FB can be generally classified into three clinicopathalogical groups based on the patients' clinical presentation and the presence or absence of underlying systemic diseases [16,33,34]: Group 1 includes patients with an underlying connective tissue disease, most commonly rheumatoid arthritis (RA) and Sjögren's syndrome (SS), who often present in their 5 th decade with progressively worsening dyspnea as the main symptom in almost all cases and in 88% of patients the diagnosis of connective tissue disease proceeds the respiratory manifestations.…”

“…In these pathological specimens the main distinguishing feature between FB and LIP is the extension of lymphoid follicles along the interlobular septa with substantial sparing of the alveolar septa in FB, where as extensive alveolar septal involvement is considered the hallmark of LIP [23,27,34]. Nonetheless, many pathologists today describe these two entities as a continuum of [table/ Fig-2]: Diseases associated with follicular bronchiolitis connective tissue disease reactive pulmonary lymphoid diseases, in which the distinction in many cases is somewhat arbitrary.…”

“…This is considered as supportive evidence of a hypersensitivity type reaction, or an undiagnosed connective tissue disease with primary respiratory manifestations as the likely aetiology in many cases of idiopathic FB. However, no treatment guidelines have yet been established for the treatment of primary FB, where a relapse of the disease with cessation of therapy and subsequent remission with reinstitution of corticosteroid treatment is commonly seen [33,34]. Macrolide antibiotics have also been used in the treatment of primary FB, with symptomatic improvement possibly related to their anti-inflammatory properties [46].…”