Bronchiolitis obliterans organizing pneumonia as the pulmonary manifestation of lupus: A review of three cases

Abstract

Objective Bronchiolitis obliterans organizing pneumonia (BOOP) is a clinico-patho-radiological diagnosis which rarely presents as a pulmonary manifestation of lupus. In this concise report, organizing pneumonia was found as the sole pulmonary manifestation of SLE in different age groups. Method All three patients diagnosed with SLE according to SLICC 2012 classification criteria, were admitted in rheumatology ward of NIMS hospital, Hyderabad, India from May to November, 2018. Their diagnosis of BOOP was either biopsy proven or imaging guided. Review of literature was done with MeSH terms (SLE, BOOP) in PubMed and approximately 10 articles were reviewed including latest of 2019 published in Scientific Reports. Result There were three patients – one juvenile lupus and two adults. Two patients were male and one female. All three patients had SLE with high disease activity. They all had organising pneumonia as pulmonary manifestation with other organ involvement. Juvenile patient had a fatal outcome while the others had a good recovery with steroid and immunosuppressive. Conclusion BOOP is a rare pulmonary manifestation in lupus. It can be diagnosed early with more precision using computerised tomography of lung without waiting for biopsy report. This will result in a better prognosis by rapid initiation of corticosteroid and immunosuppressive treatment.

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

“…In cases #2 and #4, 5,11 steroid monotherapy was ineffective for OP (death due to respiratory failure). In case #14, 6 the effect of steroid monotherapy on OP remained undetermined (myocarditis and death from causes other than respiratory failure). In case #5, 15 40 mg/day of PSL was initially effective and the radiographic abnormalities resolved; however, when the PSL dose was tapered to 15 mg/day, OP relapsed.…”

“…In cases #2 and #4, 5,11 steroid monotherapy was ineffective for OP (death due to respiratory failure). In case #14, 6 the effect of steroid monotherapy on OP remained undetermined (myocarditis and death from causes other than respiratory failure). In case #5, 15…”

“…In case #5, 15 40 mg/day of PSL was initially effective and the radiographic abnormalities resolved; however, when the PSL dose was tapered to 15 mg/day, OP relapsed. In four cases, steroid monotherapy was ineffective against OP, requiring the administration of other immunosuppressive drugs and therapy (belimumab, mycophenolate mofetil, tacrolimus, and IVCY in cases #11, 7 #13, 6 #16, and #17, respectively). Immunosuppressive agents were administered with steroids from the beginning of the clinical course in cases #9, #12, and #15; 1,6,8 they were effective in all three cases.…”

“…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.…”

“…However, in some cases, increasing the steroid doses and initiating steroid monotherapies were effective for other extrapulmonary symptoms, but not sufficiently effective for OP lesions; additional immunosuppressive therapies were needed. 6,7 In two cases, steroid monotherapies were ineffective, leading to death from respiratory failure. 5,11 These findings emphasize that OP is a serious manifestation of SLE that requires careful management.…”