Dorota Suszek

Comparison of clinical and serological parameters in female and male patients with systemic sclerosis

Objectives The course of systemic sclerosis (SSc) can differ in female and male patients. According to the literature the incidence rates of diffuse cutaneous SSc, scleroderma renal crisis and digital ulceration are higher in male patients. The aim of the study was to compare selected clinical and serological parameters in male and female patients with SSc. Material and methods The study encompassed 101 European Caucasian patients with SSc, including 23 men, hospitalized in the Department of Rheumatology. Patients fulfilled the American Rheumatism Association (ARA) classification criteria for SSc. The study groups of men and women were assessed according to the SSc subtype, incidence of internal organ involvement and presence of antinuclear antibodies considered SSc markers. Results Diffuse cutaneous (dc) SSc was observed more commonly in men than in women (13/23 vs. 25/78; p = 0.03). The time from the development of Raynauds phenomenon to the diagnosis was significantly shorter in male compared to female patients (3.2 ±4.7 vs. 7.5 ±7.1; p = 0.01). The incidence of scleroderma renal crisis (SRC) was significantly higher (3/23 vs. 2/78; p = 0.04) and of other calcifications significantly lower in the male group compared to the female group (1/23 vs. 20/78; p = 0.02). Conclusions We concluded that the incidence of dcSSc is higher in men compared to women. The time from the development of Raynauds phenomenon to the diagnosis is shorter in the male compare to female group. The incidence of SRC is higher, whereas that of calcifications is lower in SSc men. The serological profiles of female and male patients with SSc are comparable.

β2-microglobulin as a marker of systemic lupus erythematosus activity

BACKGROUND Systemic lupus erythematosus (SLE) is characterized by alternating periods of activity and remission. A portion of the patients suffers from the chronically active form of the disease. The search for clinically useful markers of its activity is ongoing. At present, it is suggested that β2-microglobulin (β2M) may be useful in assessing SLE activity. OBJECTIVES The objective of the paper was to investigate the relationship between serum β2M concentration and SLE activity. MATERIAL AND METHODS The study group consisted of 69 SLE patients (62 women and 7 men), aged 34.5 ±11 years (19-69). Patients with kidney failure and infection were excluded from the study group. The concentration of β2M was measured using an ELISA test. SLE activity was assessed with Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K), and by measuring the levels of C3 and C4 complement components, anti-double stranded DNA antibodies (anti-dsDNA antibodies) and β2M. The relationship between β2M and the clinical manifestation of SLE was also covered in the study. RESULTS The study revealed a statistically significant correlation between β2M concentration and SLEDAI-2K disease activity index (p < 0.05; r = 0.6), anti-dsDNA titer (p < 0.05; r = 0.3), and C4 component serum level (p < 0.05; r = -0.3). β2M concentration was significantly higher in patients with arthritis and/or myositis (p = 0.005), vasculitis (p = 0.005), and hematological manifestations of SLE (p = 0.02). CONCLUSIONS Periodical determination of β2M concentration in SLE patients may prove helpful in assessing the disease activity.

Coexistence of diabetes mellitus type 1 with diffuse systemic sclerosis – case report and literature review

Diabetic sclerodactyly is a frequently recognized skin finding that may occur in patients with diabetes mellitus but coexistence of diabetes and systemic sclerosis is rare. We describe a case of coexistence of type 1 diabetes mellitus and systemic sclerosis in 42-year-old man with the history of Raynaud’s phenomenon, progressive diffuse hardening of the skin and sclerodactyly, slowly worsening with time. The medical history included type 1 diabetes since childhood with microvascular complications. The patient presented a typical capillaroscopic scleroderma-like pattern, antinuclear antibodies and sclerotic lesions in gastrointestinal system. Summing up, our case represents the rare coexistence of autoimmune diseases like diabetes mellitus type 1 and systemic sclerosis.

AB0618 Comparison of Selected Serological and Clinical Parameters in Female and Male Patients with Systemic Sclerosis

Background The course of systemic sclerosis (SSc) can differ in female and male patients. It has been suggested earlier that in male patients, the incidence rates of diffuse cutaneous SSc, scleroderma renal crisis and digital ulceration are higher. Objectives The aim of the study was to compare the selected serological and clinical parameters in male and female patients with SSc. Methods The study encompassed 101 patients with SSc, including 23 men, hospitalised in the Department of Rheumatology and Connective Tissue Diseases in the years 2006-2012 (Table 1). The study groups of men and women were assessed according to the SSc subtype, presence of antinuclear antibodies considered SSc markers and incidence of internal organ involvement Results There were no statistically significant differences in the incidence of a-Scl-70 and anti-centromere antibodies observed in the groups of female and male patients. The incidence of scleroderma renal crisis was significantly higher in the male group (3/23 vs. 2/78 p=0.04). On the other hand, the incidence of calcifications was significantly lower in the male group compared to the female group (1/23 vs. 20/78 p=0.02). Diffuse cutaneous SSc was observed more commonly in men than in women (13/23 vs. 25/78 p=0.03). The time from development of Raynauds phenomenon to the diagnosis was significantly shorter in male patients compared to female patients (3.2±4.7 vs. 7.5±7.1). Conclusions The serological profiles of female and male patients with SSc are comparable. The incidence of diffuse cutaneous SSc is higher in men compared to women. The time from development of Raynauds phenomenon to the diagnosis is shorter in the male group than in the female group. The incidence of scleroderma renal crisis is higher, whereas of calcifications - is lower in SSc men compared to SSc women. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.1286

Remission in lupus nephritis – report of three cases

W pracy przedstawiono przebieg toczniowego zapalenia nerek (TZN) u trzech chorych leczonych agresywnie od początku choroby glikokortykosteroidami (GKS), cyklofosfamidem (CYC), a następnie mykofenolanem mofetylu (MMF). U dwóch chorych potwierdzono IV klasę TZN oraz rozpoznano wtórny zespół antyfosfolipidowy (antiphospholipid syndrome – APS). Zastosowanie CYC w terapii indukującej remisję oraz MMF w leczeniu podtrzymującym doprowadziło do remisji objawów nerkowych, ale łączyło się z wystąpieniem powikłań infekcyjnych zarówno w czasie leczenia CYC, jak i MMF. Zastosowanie agresywnego leczenia od początku TZN przy uwzględnieniu ryzyka możliwych powikłań daje szansę na osiągnięcie całkowitej remisji choroby. S u m m a r y

Biological therapies in systemic lupus erythematosus

The prevention of chronic organic damage and complete inhibition of inflammatory activity of the disease are the main goals in the treatment of systemic lupus erythematosus (SLE). Current therapies of SLE are not effective enough and they may cause various serious side effects. Biological therapies, affecting important pathogenetic disturbances in the immunological system of SLE patients, give hope for the development of a new treatment for SLE. Currently the most advanced clinical trials are being conducted with anti-lymphocyte B drugs, such as rituximab, belimumab and epratuzumab. Belimumab as the first biological agent was registered for treatment of the active, seropositive form of SLE. The advances in immunology and rheumatology nowadays raise the hope of finding effective and safe treatment for SLE. In our article we present an overview of data concerning perspectives of biological treatment in SLE.