Background/Purpose

Diffuse interstitial lung disease (ILD) can be associated with connective tissue diseases (CTD), and can increase morbidity and mortality significantly. The predominant patterns of ILD associated with CTD are often nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). UIP is more common in rheumatoid arthritis (RA). Pulmonary involvement in rheumatic diseases can be the first manifestation of these pathologies, preceding the onset of extrapulmonary symptoms. The presentation, clinical features and evolution of ILD associated with CTD can be variable, and therefore, it’s important to improve the prognosis by early diagnosis and treatment. The aim of this study is to describe the clinical features, treatments and outcome of patients with ILD associated with  CTD found in the General Hospital of Mérida.

Methods

We systematically collected all cases of ILD associated with CTD reported in the Rheumatology Department from January 2008 to January 2014. We included patients over 18 years old, who had a CTD and radiological diagnosis of ILD.

Results

We found 36 patients with ILD associated with CTD: 17 rheumatoid arthritis (RA), 10 scleroderma (ES), 6 antisynthetase syndromes (SAS) and 3 Sjögren’s syndrome (SS).  The mean age was 66.08 years, with a female predominance (2-1). History of smoking was present in 33.3% of the sample, and previous lung pathology in 18.7%. The diagnosis of CTD preceded the diagnosis of ILD in 58,3 % (21). The ILD patterns were: 17  NSIP  ( 47,2 %) , more frequent in ES (6 pac) and SAS (5 pac); and 19 UIP ( 52,8% ) , predominantly in RA .  Thirty patients were treated with IV cyclophosphamide (CF) and 14 with rituximab (RTX) . We observed a sustained response in 13 patients (10 NSIP and 3 UIP): 2 patients had received CF, 6 RTX, 4 CF+RTX, and 1 antiTNF. All RA patients were rheumatoid factor (RF) positive, 33,3 % with a title over 100. The predominant pattern was UIP (73,2 %). Methotrexate was used in 64.2% of patients and it was suspended at the diagnosis of ILD, although no cases of pneumonitis were found by this drug. Patients with ES, SAS and SS were younger at diagnosis (63.7, 62 and 64.3 years respectively) than RA patients (69,23 years) and a predominance of NSIP pattern (66.7, 80 and 66.6 % respectively). All these patients had negative RF. ANAs were positive in 100 % of SAS, with a predominance of anti Ro 52 and anti JO1 (3 and 4 patients) and 77.8% of ES and 66.6 % of SS. Two patients presented with poor responses to CF, one with UIP (ES) and 1 with NSIP (SAS), who died from infectious complications.

Conclusion

Usually the first manifestation of an ETC is due to the ILD, so it is advisable to maintain close cooperation with pneumologist. According to the literature, all patients with RA and ILD in our sample had RF+, as it usually occurs  in rheumatoid extraarticular involvement. Otherwise, the UIP was the predominant pulmonary pattern. Rest of ETC associated ILD had a predominance of NSIP pattern and positive ANAs, especially anti Ro, and anti Jo1 in SAS. The RF was negative in all cases. Nine patients treated with RTX or sequentially with CF + RTX achieved better response. However, more studies need to be undertaken to reach better conclusions on the ways forward in treatment.

---

« Back to 2014 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/features-of-interstitial-lung-disease-associated-with-connective-tissue-disease-in-a-spanish-southwest-cohort/