Prognostic Factors in Interstitial Lung Disease Associated with Primary Sjögren’s Syndrome: A Retrospective Analysis of 33 Pathologically–Proven Cases

Abstract

IntroductionInterstitial lung disease associated with primary Sjögren’s syndrome (pSS–ILD) shows several patterns such as nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP). Although UIP is a well–recognized prognostic determinant in idiopathic interstitial pneumonias, whether this is also the case in pSS–ILD is unclear. The objectives of this study were to evaluate the prognostic effect of UIP, and to identify the prognostic factors in pSS–ILD.MethodsA retrospective review of medical records identified 33 consecutive patients with pathologically–proven pSS–ILD. Each patient was classified into each ILD pattern by multidisciplinary analysis. Baseline clinical–radiologic–pathologic characteristics and survival rates were compared between the ILD patterns. Finally, the prognostic factors in pSS–ILD were assessed by univariate and subsequent multivariate analyses using Cox’s proportional hazards regression model.ResultspSS–ILD patients were diagnosed with NSIP (n = 22) or UIP (n = 11). The median follow–up period was 110 months, and five-year survival rate was 87.3% in the total patient population. The prognosis of the UIP patients was not significantly different from that of the NSIP patients (NSIP to UIP, hazard ratio [HR]: 0.77, 95% confidence interval [CI]: 0.18–3.36, P = 0.73). Multivariate analysis identified PaCO2 (HR: 1.68 per 1 Torr increase, 95% CI: 1.24–2.28, P < 0.01), extent of reticular abnormality on high–resolution CT (HR: 4.17 per 1-grade increment, 95% CI: 1.18–14.73, P = 0.03), and severity of fibroblastic foci (HR: 9.26 per 1-grade increment, 95% CI: 1.74–49.35, P < 0.01) as prognostic factors in pSS–ILD.ConclusionsUIP in pSS–ILD was not related to poorer prognosis than NSIP. Assessment of detailed clinical–radiologic–pathologic findings is more important than distinguishing UIP to evaluate prognosis in this disease.

Pulmonary manifestations of Sjögren's syndrome

“…Honeycombing is rarely seen, but areas of interstitial fibrosis with enlarged air spaces may induce traction bronchiectasis. This fibrosing stage is the most common NSIP variant in Sjögren's syndrome patients [98]. These histological abnormalities result in a particular CT pattern: bibasal and symmetrically predominant reticular abnormalities with traction bronchiectasis, peri-bronchovascular extension (frequently associated with ground-glass attenuation) and uncommon features including sub-pleural sparing and pulmonary consolidations (figure 3a and b) [92].…”

Interstitial lung disease in non‐sicca onset primary Sjögren's syndrome: a large‐scale case‐control study

“…The immunological, pulmonary functional and radiological characteristics of the two groups are shown in Table 2. The case-control study revealed that hyperglobulinemia (IgG), elevated RF titers, and anti-SSA and/or anti-SSB presence were less predominant in the non-sicca onset group (IgG 16 [12][13][14][15][16][17][18][19][20][21] vs. 21 [15][16][17][18][19][20][21][22][23][24][25][26][27][28] g/L, P = 0.032; 22 vs. 104 [20-237] IU/mL, P = 0.048; 33% vs. 72%, P = 0.000), whereas the IgM, IgA, C3, C4, CRP and ESR levels were comparable.…”

“…17 Moreover, cases of ILD preceding the diagnosis of pSS have been described. 5,6,9,[18][19][20][21][22] All of these findings indicate that it is challenging to establish the SS diagnosis in this subgroup. Limited studies have focused on this aspect of pSS-ILD patients.…”

Evaluation of computer-based computer tomography stratification against outcome models in connective tissue disease-related interstitial lung disease: a patient outcome study

“…Computed tomography (CT) evaluation of patients with individual connective tissue disease-related interstitial lung diseases (CTD-ILDs) have shown that several parenchymal patterns, including honeycombing [1, 2], reticulation [3], and fibrosis extent, are associated with a poor outcome [1, 4–6]. However, while studies of prognostic indices within individual CTDs convey valuable information about specific, small patient groups, the applicability of such indices to a wider group of “all-comers” CTDs needs validation.…”