Background Adult Still’s disease (ASD) is an inflammatory disorder characterized by persistent fevers, arthritis, and an evanescent rash. Interstitial lung disease (ILD) is rare, reported in 6%1) of ASD. Some patients progressed to acute respiratory distress syndrome. However, clinical characteristics and treatment strategies of ILD associated with ASD are not established.
Objectives To characterize clinical features and outcome of ASD-associated ILD (ASD-ILD).
Methods We retrospectively reviewed clinical charts of all the patients diagnosed with ASD according to Yamaguchi criteria for ASD at our institutes from 2005 to 2011. ILD was diagnosed based on chest high-resolution CT (HRCT). We extracted the patients with ASD-ILD and compared their clinical characteristics with the non-complication group. Also, we characterized the HRCT findings of ASD-ILD.
Results Total 84 cases of ASD was identified, male to female ratio was 22:62. Most cases fall into two age groups (20-30 and 50-60 y.o.), the average age was 42.4 (16-86 y.o.). ILD was identified in 7 out of 84 cases (8.3%), male to female ratio was 1:6. The average age was 63.6 (36-76 y.o.). Patient number (Pt) 1-3 developed ASD and ILD at the same time. The rest of the cases (Pt4-7) developed ASD-ILD about 1 year after suffering ASD. HRCT showed marked thickening of the interlobular septi, the bronchovascular bundles, and the pleura in all cases. These finding involved whole of the lungs evenly in all cases but one which had lower lobe predominance. There was no honeycombing formation. Three (43%) out of 7 patients (Pt1-3) suffered from dyspnea with hypoxemia, which paralleled with the activity of ASD. As ILD complication rapidly progressed, intravenous cyclosphophamide (IVCY) or tocilizumab (TCZ) were introduced with an increasing doze of glucocorticoid (GC), resulting in an improvement of dyspnea and ILD. Even though Pt4-6 did not show respiratory symptom, HRCT finding of ILD was worsen with the recurrence of ASD. To deal with the recurrence of ASD, increasing dosage of GC and ciclosporin was used. Pt7 did not show any respiratory symptom and his ILD was stable during the course. As for other clinical characteristics of ILD complication, it was of note that hemophagocytic syndrome was complicated in 5 cases with ILD (71%). Six patients in ILD (85.7%) showed a recurrence during the course. All of them were remitted after the intensified treatment. There was no death.
Conclusions ASD-ILD tended to occur in older female. ILD complicated at the initial diagnosis of ASD was symptomatic and progressive, which required an aggressive immunosuppressive therapy such as IVCY and TCZ. HRCT finding included marked thickening of the interlobular septi, the bronchovascular bundles, and the pleura, which could be pathognomonic imaging characteristics of ASD-ILD. HRCT findings suggest massive infiltration of macrophages and lymphocytes2) with lymphangiectasis in the inter septal space. Histologic investigation is definitely needed.
Ohta A et al. Adult Still’s disease: a multicenter survey of Japanese patient. J Rheumatol. 1990;17:1058-63.
Sato H et al. A case of Adult onset Still’s disease complicated with cryptogenic organizing pneumonia. Inten Med. 2011;50:247-51.
Disclosure of Interest None Declared