Systemic scleroderma (SSD) is a clinically heterogeneous disease characterized by obliterating microangiopathy, autoimmune activation, and fibrosis of the skin and viscera. Interstitial lung fibrosis (ILF) is a characteristic visceral injury in SSD and considered to be a main cause of disability and death. The diagnosis of SSD-associated ILF is made on the basis of a cluster of symptoms, physical examination, external respiratory function changes, and high-resolution computed tomography. Pulmonary fibrosis is included in the 1980 American College of Rheumatology (ACR) classification criteria and in the joint ACR and 2013 European League against Rheumatism diagnostic criteria. According to the definition given in these criteria, pulmonary fibrosis in SSD is described as bilateral changes, most pronounced in the basal lung segments, which are not a sign of primary lung disease. The paper describes a case of a SSD patient with a complete spectrum of characteristic signs of unilateral pulmonary fibrosis. This case is the first description of unilateral ILF in SSD and shows the need for ruling out connective tissue diseases, primarily SSD, when such lung changes concurrent with extrapulmonary manifestations are detected.

Background Studies of bone mineral density (BMD) in patients with systemic sclerosis (SSc) showed its decreasing in comparison with healthy control. Prevalence of low bone mass and severity of BMD decreasing differ significantly between patients from different countries and vary from 17% in China (Mok CC, et al. 2012) to 77% in Spain (Rios-Fernández R, et al, 2012). Objectives To assess BMD and frequency of osteoporosis (OP) in patients with SSc in Russia. Methods In case-control study BMD was evaluated in 52 postmenopausal women with SSc (16 – diffuse and 36 – limited form), mean age 57,6±7,1yrs and mean disease duration 11±8 yrs. Forty four healthy postmenopausal women (mean age 59,2±6,6yrs) served as control. BMD was measured at the lumbar spine (LS), femoral neck (FN) and total hip (TH) by DXA (Hologic 4500A). BMD decreasing grade was determined in according to WHO criteria Results BMD was significantly decreased in SSc women in comparison with control group: in LS – 0,804±0,090 vs 0,861±0,092 g/sm2 (p=0,025); in FN – 0,670±0,128 vs 0,736±0,112 g/sm2 (p=0,037), and in TH – 0,801±0,160 vs 0,884±0,124 g/sm2 (p=0,03). Frequency of OP in SSc group was significantly more often than in control group (59% vs 11%, p<0,0001). BMD didn't differ between pts with diffuse or limited SSc. Decreasing of BMD in LS, FN and TH associated with age (r=-0,40, p<0,001; r=-0,48, p<0,001; r=-0,38, p=0,002, respectively), duration of postmenopausal period (r=-0,44, p<0,001; r=-0,42, p<0,001; r=-0,33, p=0,016, respectively), duration of SSc (r=-0,21, p=0,033; r=–0,37, p=0,001; r=-0,36, p=0,004, respectively) and hsCRP level (r=-0,22, p=0,041; r=-0,26, p=0,045; r=-0,35, p=0,011). BMD of FN and TH correlated also with daily dose of glucocorticoids (GC) (r=-0,34, p=0,025; r=-0,37, p=0,023, respectively). T-score was significantly lower in pts treated with GC than in GC free pts in LS (-2,01±1,52 vs -1,17±1,42, p=0,012), FN (-2,38±1,18 vs -1,26±1,25, p=0,003) and TH (-1,98±1,34 vs -0,84±1,19, p=0,011) Conclusions OP occurs in more than half cases of SSc pts. Low BMD in SSc pts apart from traditional risk factors of OP is associated with disease duration and inflammatory activity. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5426

Objectives To study a frequency of cardiac arrhythmias and conduction disturbances by standard ECG and 24-hour Holter monitoring (HM) Methods Eighty pts with systemic sclerosis (SSc) (28 – diffuse, and 52 – limited form), age 21-80y (52±11), and disease duration of 1-36y (9,8±8,6). All pts underwent standard ECG and 24-hour HM Results By standard ECG cardiac arrhythmias were found in 14 patients (17%), including premature supraventricular beats (PSVB) in 10 (13%) patients and premature ventricular beats (PVB) in 7 (9%) of them. Conduction disorders were seen in 28 (35%) patients, including right bundle branch block in 7 (9%) patients, left bundle branch block in 16 (20%), and combined right and left bundles branch block in 5 (6%) from 80 pts. By HM, PSVB were found in 74 (93%) patients, and PVB in 52 (65%). Twenty nine (36%) patients showed grade III, IVA, IVB or V of ventricular arrhythmias, according to Lown's classification. There also were atrioventricular block of 1st grade in 1 case, and 2nd grade – in 2 patients. Twenty one patients had coronary artery disease (CAD) and there weren't any differences of arrhythmias' frequency between patients with or without CAD. Arrhythmias in group of patients under 50 year and in group of older patients were found in 10 from 28 (35%) patient and in 34 from 52 (65%) patients, respectively (p=0,001). There weren't any associations of the frequency of arrhythmias with duration of disease and clinical forms of SSc. The frequency of cardiac arrhythmias didn't differ between patients group with disease duration lesser 5 years and in patients with disease duration greater than 5 years. Conclusions Cardiac arrhythmias and conduction abnormalities are common in patients with SSc and are revealed significantly often by HM in comparison with standard ECG Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5182

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