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Aim: To assess prevalence of certain cardiac arrhythmias and conduction disturbances in the Russian population of systemic sclerosis patients using standard 12-lead and 24-hour electrocardiography (ECG); to estimate correlation between cardiac arrhythmia and clinical features of systemic sclerosis. Materials and methods: 80 systemic sclerosis patients and 60 sex- and age-matched controls were included. All patients underwent standard 12-lead and 24-hour ECG. Results: Arrhythmias on standard 12-lead ECG were demonstrated in 14 patients (17.5%) including sinus arrhythmia in 2 cases and premature beats in 13 cases (16%). Supraventricular (SV) and ventricular (V) ectopic beats were recorded in 5 (6%) and 7 (9%) patients, respectively; in one patient both SV and V ectopic beats were found. ECG signs of focal fibrosis were demonstrated in 9 patients (11%). In 24-hour ECG, frequencies of SV and V ectopic beats were 40 and 65%, respectively. Compared to the controls, systemic sclerosis patients had significantly higher prevalence and severity of cardiac arrhythmia. 36% of patients had high grade ventricular premature beats, associated with potential risk of life-threatening arrhythmia and sudden cardiac death. Conclusion: Cardiac arrhythmias and conduction disturbances are found in the majority of patients with systemic sclerosis. Standard ECG does not reflect true prevalence of cardiac arrhythmia in systemic sclerosis. In systemic sclerosis patients, 24-hour ECG is recommended as obligate method for initial examination and treatment efficacy control.

Systemic sclerosis (SS) is a progressive connective tissue disease, the prognosis of which largely depends on the time of adequate therapy initiation. Low sensitivity of the 1980 American College of Rheumatology (ACR) SS classification criteria for identifying patients with early stage of the disease, and with its limited form in particular, has necessitated revision of existing SS diagnostic standards and elaboration of more sensitive criteria that allow to establish the diagnosis when the first sign of the disease appear.Objective: to compare the sensitivity of the novel SS criteria of ACR and European League against Rheumatism (ACR/EULAR) 2013 and the 1980 ACR criteria in different stages of the disease.Subjects and methods. The investigation enrolled 302 patients who had been diagnosed by experts as having SS and followed up at the V.A. Nasonova Research Institute of Rheumatology in 2007–2013. The patients’ mean age was 49±13 years (18 to 80 years); male to female ratio – 1:9 (31 and 271), that of patients with diffuse and limited SS – 1:2 (105 and 197); mean duration of the disease from the first non-Raynaud’s syndrome was 8.2±7.0 years (6 months to 36 years). Physical examination, nailfold capillaroscopy, chest radiography or computed tomography, echocardiography for the determination of pulmonary artery systolic pressure and SS-specific antibodies evaluation were performed.Results. 273 (90%) patients fulfilled the novel ACR/EULAR 2013 SS criteria. 76 (25%) patients had skin thickening above the metacarpophalangeal (MPC) joints in both hands; 263 (87%) – finger skin thickening [70 (23%) – finger swelling, 192 (64%) – thickening of all fingers distal to the MPC joints], 141 (47%) – digital ischemia [79 (26%) – digital pitting scars, 20 (7%) – digital ulcers, 42 (14%) – digital pitting scars and ulcers], 134 (44%) – telangiectasias, 276 (91%) – capillaroscopic changes, 225 (78%) – pulmonary hypertension (PH) or interstitial lung disease (ILD) [15 (5%) – PH 185 (61%) – ILD, 35 (12%) – ILD and PH], 301 (99%) – Raynaud’s phenomenon, and 185 (61%) – SS autoantibodies [138 (46%) – anti-Scl-70 antibodies (a-Scl-70), 42 (14%) – anti-centromere antibodies (ACA), 5 (1.7%) – ACA and a-Scl-70]. 216 (72%) patients fulfilled 1980 ACR SS criteria, and all of them met the novel criteria. With the latter, SS could be additionally diagnosed in 57 more (18%) patients.Conclusion. The 2013 ACR/EULAR SS classification criteria have much higher sensitivity than the 1980 ACR criteria. The sensitivity of the novel criteria remained at the level of 90% in all, including the earliest, stages of the disease while the ACR criteria allowed to confirm diagnosis of SS in only half of patients with a disease duration of less than 1 year.

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 isdescribed 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 outconnective tissue diseases, primarily SSD, when such lung changes concurrent with extrapulmonary manifestations are detected.

AIM: To retrospectively analyze the efficacy and safety of sildenafil (Sf) in patients with systemic sclerosis (SS). Subjects an/RESULTS: Sf was used in 16 patients (including 14 women) aged 20-66 years (mean 48.6±14.6 years; median 51.5 years) with SS of a duration of 2 months to 27 years (mean 8.8±7.3 years; median 6.5 years). The indications for Sf treatment were significant Raynaud's phenomenon (RP) in 3 patients, digital ulcers (DU) and/or necroses (N) in 9, pulmonary hypertension (PH) in 5 (2 patients had PH concurrent with DU/N), and critical ischemia of the left fingers in 1 patient. RP was seen in all the patients and so the effect of Sf on the course of RP was evaluated in the whole patient group/RESULTS: There was a significant decrease in the frequency and intensity of Raynaud's attacks in 11 (73%) of the 15 patients treated with Sf. This effect was obvious just in the first days of Sf treatment and remained stable throughout the treatment. No RP changes were seen in 3 patients. All 7 patients with DUs showed a decrease in their sizes just within the first two weeks of treatment. Complete DU healing was observed within 4-12 weeks of treatment. During a month, the necrotic area reduced and the signs of reparation appeared in 4 of the 6 patients. Pain ceased just within the first 5-7 days of treatment. Sf resulted in a rapid reduction in systolic pulmonary artery pressure (sPAP); in one case the latter diminished from 60 to 40 mm Hg just 90 min after the first intake of Sf 50 mg and remained unchanged during all 6 months during which the female patient was taking the drug. Doppler echocardiography showed that sPAP decreased from 103 to 85 mm Hg in another female taking Sf 100 mg for a month. The two cases showed clinical improvement as alleviated dyspnea and increased physical activity. In another case, Sf was discontinued because of dizziness after its first intake in a dose of 12.5 mg. The initial drug intake of the drug was not followed by adverse reactions in 12 (75%) of the 16 patients. Four patients had Sf-induced complaints, including headache (1), dizziness (2), and more severe angina pectoris (1). In different periods after treatment initiation, four more patients developed complications, such as fatal myocardial infarction after 6-week treatment, atrial fibrillation at 8 weeks, more severe angina at 6 months, and congestive heart failure after 5-year treatment. These complications were observed in patients with severe ECG changes, such as myocardial focal fibrosis or blood supply impairment/CONCLUSION: Sf is an effective drug to treat the manifestations of scleroderma vasculopathy, such as RP, DU/N, and PH. Sf is well tolerated in most cases. The SS patients with pronounced ECG changes have an increased risk of severe cardiac events and they need careful ECG monitoring.