Constrictive (Obliterative) Bronchiolitis as Presenting Manifestation of Connective Tissue Diseases

Antonella Arcadu, MD; Jay H. Ryu, MD

Disclosures

J Clin Rheumatol. 2020;26(5):176-180. 

In This Article

Abstract and Introduction

Abstract

Background: Constrictive (obliterative) bronchiolitis (CB) is an uncommon form of obstructive lung disease that can occur in patients with identifiable causes including connective tissue diseases (CTDs) as a form of lung involvement. We explored whether CB can be the presenting manifestation of CTD.

Methods: We identified 44 patients with cryptogenic CB and examined the presenting clinical, laboratory, and radiologic features, as well as their clinical course.

Results: The mean age at presentation was 60.5 (SD, 13.8) years and included 38 women (86%); 32 (73%) were never smokers. All patients presented for evaluation of dyspnea, commonly associated with cough. An obstructive pattern on pulmonary function testing was demonstrated in 86% of patients. On chest high-resolution computed tomography, nearly all patients manifested a mosaic attenuation pattern with air trapping on expiratory views, characteristic of CB. Bronchoscopic lung biopsy (n = 10) was usually nondiagnostic (90%), whereas all 5 surgical lung biopsies yielded evidence of CB. Serologic testing for CTD was positive in 19 patients (43%) and most commonly included antinuclear antibody, rheumatoid factor, and anti-cyclic citrullinated antibodies. Seven of these patients with positive serologic results were eventually diagnosed to have CTD. Connective tissue diseases included rheumatoid arthritis in 4 patients, Sjogren syndrome in 2, and undifferentiated CTD in 1 patient.

Conclusions: Nearly one-half of patients with cryptogenic CB manifest positive CTD serology, and some of these patients have CTD not previously diagnosed. These results suggest that CB can be the presenting manifestation of a CTD.

Introduction

Constrictive bronchiolitis (CB) is an uncommon disorder involving the small airways. Also termed obliterative bronchiolitis or bronchiolitis obliterans, CB comprises a spectrum of histopathologic changes ranging from bronchiolar inflammation to peribronchiolar fibrosis and, ultimately, complete cicatrization of the bronchiolar lumen.[1] This disorder is clinically characterized by exertional dyspnea, progressive airflow obstruction, and mosaic attenuation pattern with expiratory air trapping on high-resolution computed tomography (HRCT).[1–3]

Constrictive bronchiolitis is most commonly described in patients who have undergone solid organ or hematopoietic stem cell transplantation (bronchiolitis obliterans syndrome). It can also be encountered in patients with connective tissue diseases (CTDs), inhalational injuries, or after respiratory infections (Swyer-James [MacLeod] syndrome), whereas others remain cryptogenic, that is, cryptogenic CB.[1–5]

Because respiratory manifestations can be the initial presentation in some patients with CTDs, we wondered whether CB may at times be the presenting manifestation of an evolving CTD. Thus, we analyzed a cohort of patients presenting with CB of unknown cause for evidence of CTD at initial evaluation or during the subsequent clinical course.

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