Bronchiolitis obliterans organising pneumonia as an initial manifestation in a patient with systemic lupus erythematosus: a rare presentation

Abstract

Bronchiolitis obliterans organising pneumonia as an initial manifestation of systemic lupus erythematosus (SLE) is a rare and uncommon presentation. We describe a case of SLE presenting with shortness of breath, found to have pneumothorax, bilateral nodular infiltrates along with pleural effusions and pericardial effusion. Work-up suggested a diagnosis of active SLE with anaemia, thrombocytopenia, positive antinuclear antibodies (ANAs) and positive anti-double-stranded DNA. On retrospective review of patient records, from 8 years prior to presentation, lung biopsy histology consistent with bronchiolitis obliterans organising pneumonia with positive ANA serology was found, without any further autoimmune work-up. In our opinion, bronchiolitis obliterans organising pneumonia was the index presentation of SLE. Treatment with steroids and subsequent management with immunosuppressive therapy could have prevented subsequent hospitalisations. Prompt work-up for autoimmune diseases should be considered in patients with positive ANA and histological evidence of bronchiolitis obliterans organising pneumonia.

Organizing pneumonia as a possible pulmonary manifestation of systemic lupus erythematosus: Three cases and a review of literature

“…In five cases, OP was pathologically confirmed by parenchymal lung biopsy (surgical lung biopsies: cases #1, 7, 9, and 10; autopsy: case #4). 5,8,10,11,13…”

“…We identified 10 reports on 15 cases of OP that developed at the initial diagnosis or relapse of SLE. 1,[5][6][7][8][9][10][11][12][13] We have summarized 18 cases (including our three cases). In one of these reports, Enomoto et al described 55 patients with SLE-related interstitial pneumonia; these included 12 patients with a radiological OP pattern, 14 who were excluded from our analysis due to insufficient clinical data.…”

“…In five cases, OP was pathologically confirmed by parenchymal lung biopsy (surgical lung biopsies: cases #1, 7, 9, and 10; autopsy: case #4). 5,8,10,11,13 Extrapulmonary symptoms of systemic lupus erythematosus were observed along with the development of organizing pneumonia. One or more of the following extrapulmonary symptoms of SLE were observed along with OP development (Table 2): fever (14 cases [77.8%]), cutaneous Outcomes of OP occurring at or after the diagnosis of systemic lupus erythematosus.…”

“…In seven cases (38.8%; cases #1, 3, 6, 7, 8, 10, and 18), steroid therapy was effective for OP and no recurrence was noted. 5,9,10,12,13 In the remaining eight cases, the outcomes were as follows. In cases #2 and #4, 5,11 steroid monotherapy was ineffective for OP (death due to respiratory failure).…”

Bronchiolitis and Bronchiolar Disorders

“…Bronchiolar complications of CTDs are less well recognized than other pulmonary complications (e.g., ILD and pleural disease) of these disorders and occur most commonly in females with RA or Sjogren's syndrome, 74,75 less frequently in lupus erythematosus, ankylosing spondylitis, and systemic sclerosis (SSc). 76 Pulmonary function tests (PFTs) usually show poorly reversible airflow limitation that may be preceded by distal airflow limitation 77 ; in RA patients, factors significantly associated with abnormal forced expiratory flow between 25 and 75% of forced VC (forced expiratory flow, FEF25-75%) are respiratory symptoms, smoking history, and RA duration. 78 Patients with CTD-related bronchiolitis may have lower FEV1 when histology shows constrictive bronchiolitis compared with patients with nonconstrictive bronchiolitis (such as cellular or follicular); however, the rate of change of FEV1 does not seem to vary significantly in either subgroup over time.…”