Cutaneous vasculitis in systemic lupus erythematosus patients: potential key players and implications.

OBJECTIVES: The aim of the present work was to study the clinical characteristics of cutaneous vasculitis (CV) in systemic lupus erythematosus (SLE) patients and find possible potential key players in its development and implicated associations with the disease manifestations. PATIENTS AND METHODS: Fifty adult female SLE patients underwent full history taking, thorough clinical examination and laboratory investigations. The SLE Disease Activity Index (SLEDAI) and accumulated damage using the Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR DI) were assessed. RESULTS: The mean age of the patients was 29.1 ± 6.1 years and was significantly lower in those with CV (p = 0.018). The disease duration was 4.9 ± 3.7 years. CV was present in 30% of the patients. Musculoskeletal manifestations and hypocomplementemia were present in all patients with CV. The SLEDAI and SLICC/ACR DI tended to be higher in those with CV. Complement (C3 and C4) was significantly consumed in CV patients (p < 0.0001). Antiphospholipids were comparable between those with and without CV. Lupus nephritis, cardiovascular manifestations and Sjögren syndrome were significantly linked to the development of CV (p = 0.025, p = 0.023 and p < 0.0001, respectively). Both C3 and C4 showed a high sensitivity (93.3% and 86.7%) to detect CV in SLE at cut-off values below 81.4 mg/dl and 16.8 mg/dl, respectively. CONCLUSION: CV is closely related to hypocomplementemia but not to antiphospholipids and is associated with lupus nephritis, musculoskeletal manifestations and Sjögren syndrome.

Anti-dsDNA titre in female systemic lupus erythematosus patients: relation to disease manifestations, damage and antiphospholipid antibodies.

Background Attempts are ongoing to unveil unresolved queries about anti-double-stranded deoxyribonucleic acid (anti-dsDNA), their precise pathogenic effects and to what extent blocking them would be a useful therapeutic goal. Objectives The aim of the present study was to determine the anti-dsDNA antibodies titre in systemic lupus erythematosus (SLE) patients and investigate their relation to the disease characteristics, activity, damage and antiphospholipid autoantibodies (aPL). Methods Seventy female SLE patients and 35 age- and sex-matched controls were included. The anti-dsDNA level and aPL were measured. Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) were assessed. Results The mean age of the patients was 27.5 ± 5.1 years, disease duration 7.7 ± 5.4 years, and SLEDAI and SLICC/ACR-DI scores were 6.8 ± 8.04 and 1.2 ± 1.3, respectively. Anti-dsDNA was positive in 61.4% of the patients and the titre (133.2 ± 100.5 IU/ml) was significantly higher compared to controls (22.03 ± 17.2 IU/ml) ( p < 0.0001). The anti-dsDNA level was significantly increased in those with musculoskeletal manifestations ( p = 0.007) and positive anti-ß2 glycoprotein (anti-ß2GP) ( p = 0.037) and decreased in those with neuropsychiatric manifestations ( p = 0.004) and those receiving cyclophosphamide (CYC) ( p = 0.013). The anti-dsDNA level tended to be higher in active patients. The anti-dsDNA titre significantly correlated with the erythrocyte sedimentation rate ( p = 0.001), anticardiolipin IgG and IgA antibodies ( p = 0.008) and anti-ß2GP IgG ( p = 0.03) and IgA ( p = 0.002) and inversely with the total leucocytic count ( p < 0.0001) and SLICC/ACR-DI ( p = 0.001). Conclusion Anti-dsDNA is remarkably increased in SLE patients especially those with musculoskeletal manifestations and aPL. A protective role seems likely in those with neuropsychiatric manifestations and those receiving CYC and may form a shield against disease tissue damage.

Toll-like receptor 9 in systemic sclerosis patients: relation to modified Rodnan skin score, disease severity, and functional status.

The objective of this study is to assess toll-like receptor-9 (TLR9) expression in CD3-positive T lymphocytes and CD19-positive B lymphocytes in systemic sclerosis (SSc) patients and to study their relation to the extent of skin fibrosis, disease characteristics, and severity as well as the functional status. Fifty-five female SSc patients and 30 matched controls were included. Skin thickness was scored according to the modified Rodnan skin score (mRss). The severity of major organ involvement was assessed using the Medsger severity score (MSS). Scleroderma health assessment questionnaire (SHAQ) was measured to evaluate patients' functional status. Expression of TLR9 in CD3-positive T lymphocytes and CD19-positive B lymphocytes was studied using flow cytometry. The mean age of the patients was 40.5 ± 9.1 years, and their disease duration was 6.7 ± 3.3 years. There were 21 (38.2%) with diffuse (dcSSc) and 34 (61.8%) with limited cutaneous (lcSSc) subtypes. There was a significant increase in the expression of TLR9/CD3 and TLR9/CD19 in the SSc patients (44.9 ± 18.1 and 24.1 ± 9.6) compared to that in the control (1.4 ± 0.97 and 1.3 ± 0.94; p < 0.0001 for both, respectively) being higher in those with dcSSc. TLR9/CD3 expression was significantly increased in SSc patients with arthralgia/arthritis and digital resorption compared to those without. The TLR9/CD3 significantly correlated with the mRss and MSS (r = 0.37, p = 0.006 and r = 0.31, p = 0.02; respectively). Both the TLR9/CD3 and TLR9/CD19 expressions were significantly correlating (r = 0.53, p < 0.0001). On regression analysis, only TLR9/CD3 was a significant risk factor of the mRss and MSS (ß = 0.43, p = 0.009 and ß = 0.33, p = 0.015, respectively). TLR9, especially TLR9/CD3, is highly expressed in SSc patients particularly those with dcSSc subtype and could form a potential marker for skin fibrosis and disease severity.