Organizing Pneumonia in Rheumatoid Arthritis Patients: A Case-Based Review

Abstract

We treated 21 patients with organizing pneumonia (OP) associated with rheumatoid arthritis (RA) or related to biological disease-modifying antirheumatic drugs (DMARDs) at our institution between 2006 and 2014. Among these cases, 3 (14.3%) preceded articular symptoms of RA, 4 (19.0%) developed simultaneously with RA onset, and 14 (66.7%) occurred during follow-up periods for RA. In the case of OP preceding RA, increased levels of anti-cyclic citrullinated peptide antibodies and rheumatoid factor were observed at the OP onset. RA disease activity was related to the development of OP in the simultaneous cases. In the cases of OP developing after RA diagnosis, 10 of 14 patients had maintained low disease activity with biological DMARD therapy at the OP onset, and among them, 6 patients developed OP within the first year of this therapy. In the remaining four patients, RA activity was not controlled at the OP onset. All patients responded well to systemic steroid therapy, but two patients suffered from relapses of articular and pulmonary symptoms upon steroid tapering. In most of the RA patients, DMARD therapy was introduced or restarted during the steroid tapering. We successfully restarted a biological DMARD that had not been previously used for patients whose RA would otherwise have been difficult to control. In this study, we also perform a review of the literature on RA-associated or biological DMARD-related OP and discuss the pathogenesis and management of OP occurring in RA patients.

Clinical Characteristics and Predictors of the Recurrence of Organising Pneumonia Associated with Rheumatoid Arthritis

“…This proportion of cases in our study is similar to those reported in previous studies: 11% [14] or 14% [22] of RA-OP patients. Meanwhile, the di culty in differentiating RA-OP from bacterial pneumonia in clinical practice may be, in part, explained by our ndings: 42% of the OP events showed unilateral shadows, 76% had normal KL-6 titres, and most patients presented with u-like symptoms, all of which are the typical characteristics of bacterial pneumonia.…”

“…Our study clari ed that 30% of the patients with RA-OP experienced OP recurrence even with proper glucocorticoid treatment. This proportion corresponds to those in previous reports on RA-OP: 21% [14] or 10% [22]. These proportions of RA-OP might be lower than in those of cryptogenic OP: 58% [28], 56% [29], 30% [26], or 27% [27].…”

“…In this study, we clari ed the clinical characteristics of RA-OP patients and events, showed that OP development is not associated with RA exacerbation, revealed the high rate of the rst recurrence of RA-OP, and identi ed four predictive patient characteristics at the rst OP onset for later OP recurrence: lower age at the rst OP onset, shorter period from the RA onset to the rst OP onset, OP-preceding, and history of smoking. This study is the third retrospective cohort study for RA-OP [14,22] and the rst to establish the predictors of RA-OP recurrence.…”

“…Only a few studies have reported RA-OP's clinical characteristics [14,22], and further studies are required to understand the relationship between OP development and RA exacerbation. In addition, these studies did not exclude drug-induced and post-infectious OP in patients with RA, which may mask the true characteristics of RA-OP; therefore, they emphasise the need for studies focussing exclusively on RA-OP.…”

Successful treatment of three patients with organizing pneumonia associated with rheumatoid arthritis using clarithromycin and prednisolone

“…According to a case-based review, the PSL dosage for OP in patients with RA was 25-60 mg/day or 1-1.5 mg/kg/day (17). In Case 1, although CAM was ceased due to its adverse side effect of diarrhea, when OP shadows grew worse with PSL (5 mg/day) treatment, CAM was successfully added to the treatment.…”