Shrinking lung syndrome (SLS) is a rare and less known complication mainly associated with systemic lupus erythematosus (SLE). In this study, we analyze the clinical features, investigation findings, approaches to management, and outcome in a case series of 9 adult patients with SLE and SLS diagnosed during a 35-year period in 3 referral tertiary care hospitals in Spain. Additionally, we reviewed 80 additional cases previously reported (PubMed 1965–2015). These 80 cases, together with our 9 patients, form the basis of the present analysis.The overall SLS prevalence in our SLE population was 1.1% (9/829). SLS may complicate SLE at any time over its course, and it usually occurs in patients without previous or concomitant major organ involvement. More than half of the patients had inactive lupus according to SELENA-systemic lupus erythematosus disease activity index (SLEDAI) scores. Typically, it presents with progressive exertional dyspnea of variable severity, accompanied by pleuritic chest pain in 76% of the cases.An important diagnostic delay is common. The diagnostic tools that showed better yield for SLS detection are the imaging techniques (chest x-ray and high-resolution computed tomography) along with pulmonary and diaphragmatic function tests. Evaluation of diaphragm dome motion by M-mode ultrasonography and phrenic nerve conduction studies are less useful.There are no standardized guidelines for the treatment of SLS in SLE. The majority of patients were treated with medium or high doses of glucocorticoids. Several immunosuppressive agents have been used in conjunction with steroids either if the patient fails to improve or since the beginning of the treatment. Theophylline and beta-agonists, alone or in combination with glucocorticoids, have been suggested with the intent to increase diaphragmatic strength.The overall long-term prognosis was good. The great majority of patients had significant clinical improvement and stabilization, or mild to moderate improvement on pulmonary function tests. The mortality rate was very low.

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Objectives To analyze if the advanced age influence on the type of renal injury and the prognosis of lupus nephritis (LN). Methods We evaluated 243 patients with systemic lupus erythematosus (SLE) followed in our department from 1980 until 2013 that were registered in a specific data basis (register AQUILES). Patients with biopsy-proven LN and 2 years of time tracking (n=79) were selected as the study population. We realize a comparative study between patients older (n=30) and younger (n=49) than 50 years old. In the evaluation of the response, we considered “responders” those patients who were in remission (partial or complete) after complete the induction treatment and “non responders” those who did not showed improvement in the analytics, as well as in those who we proved transformation of histologyc class and those who died by nephritis-related reasons. To compare between groups with continuous variables we had used the Student's t test and Mann–Whitney U test (on non-normal distributions). To compare categorical variables we used chi-square test and Fisher's exact test when sample sizes were small than 5. The significance level was established for p value under 0.05. Results The cohort included 79 patients (64 females) with a mean age at LN diagnosis of 45±14 (rank, 17-80) years and mean LN disease duration of 15.9 months (rank, 0 - 456). 81% (64/79) of patients presented renal disease at LN diagnosis or during the first year of tracing. The mean SLEDAI score was 15±7.6. The comparative study results between age groups are showed at table 1. We did not note significant differences between groups in evolution course neither in the prognosis of SLE. About the treatment, we did not detect significant differences in the percentage of patients who get hydroxychloroquine, glucocorticoids or immunosuppressant drugs, but we found differences in a higher use of statins (p=0.038) and ACE inhibitors (p=0.033) in the elderly group of patients. Conclusions The advanced age does not determine the type of renal injury and the prognosis of lupus nephritis. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5048