Systemic Sclerosis and Systemic Lupus Erythematous
Conditions
Brief summary
* Evaluate renal resistive index in SSc and SLE patients for early detection of renal impairment. * Evaluate renal multi-parametric MRI in SSc and SLE patients for early detection of renal impairment. * Measure the serum levels of CD147 in SSc and SLE patients and its correlation with renal impairment. * Correlation between detected markers and other assessment tools.
Detailed description
Systemic sclerosis (SSc) is an autoimmune disease characterized by endothelial dysfunction, collagen deposition, and fibrosis in the skin and internal organs. Systemic lupus erythematosus (SLE) is a complex, multi-organ autoimmune disease. The presence of autoantibodies and circulating immune complexes can cause vasculitis and damage the endothelial cells, leading to multiple organ dysfunctions. Renal involvement in SSc, ranges from urinary abnormalities, reduction of glomerular filtration rate (GFR), and high resistive indices, to scleroderma renal crisis (SRC). Subclinical renal vasculopathy is characterized by progressive increase of intrarenal stiffness and reduction of parenchymal thickness due to post ischemic fibrosis secondary to the renal Raynaud phenomenon.Conversely, in SLE-related kidney manifestations, renal damage results from glomerular involve-ment secondary to immune-complex acti¬vation. Cluster of differentiation 147 (CD147) is, also named extracellular matrix metalloproteinase inducer (EMMPRIN), a transmembrane glycoprotein in the immunoglobulin superfamily that is widely expressed on the surface membrane of various cells. Higher levels of soluble CD147 were found in SSc patients compared to healthy controls and higher levels in SSc patients with scleroderma renal crisis (SRC). Suggesting that CD147 could be a useful tool for identifying SRC risk. In patients with active SLE, CD147 is overexpressed on CD3 T lymphocytes. Renal resistive index (RRI) is a useful non-invasive technique for evaluation of renal disease activity in SLE patients. It can differentiate between patients with active lupus nephritis (LN) and inactive LN. RRI was higher in SSc patients than other autoimmune diseases since subclinical renal vasculopathy is the main pathogenic mecha¬nism of all SSc renal manifestations.Multiparametric renal Magnetic Resonance Imaging (MRI) shows great promise as a non-invasive method to assess kidney structure and function without exposure to radiation or gadolinium contrast agents. In MRI microstructural changes of lupus nephritis kidney such as inflammatory cell infiltration or fibrosis could influence water molecular movement or diffusion, which indicates that diffusion-weigted imaging (DWI) may become a valuable tool in diagnosis of LN. However in systemic sclerosis there is decline of renal blood flow values that indicate microvascular pathology.
Interventions
Laboratory investigations: erythrocyte sedimentation rate (ESR) Serum C-reactive protein (CRP) complete blood picture (CBC) liver function tests (LFT): aspartate transaminase (AST), alanine transaminase (ALT), serum albumin, serum bilirubin. kidney function tests (KFT): serum creatinine and blood urea. Estimated glomerular filteration rate (eGFR) by CKD-EPI Creatinine Equation (2021). Complete urine analysis for: proteinuria, urinary casts (hyaline, granular), hematuria (RBCs/ high power field), pyuria (WBCs/ high power field). 24 hour protein in urine and Creatinine clearance. Complement 3and 4(C3,C4). Immunological investigations : Antinuclear antibody (ANA) and Anti-double stranded DNA (ds-DNA). Serum CD147 Radiological investigations: * Chest X-ray * Renal Doppler * Multi-parametric MRI
Sponsors
Study design
Eligibility
Inclusion criteria
1. Adult (aged above 18 years) SSc patients who fulfilled the 2013 European League Against Rheumatism/American College of Rheumatology (EULAR / ACR) for SSC \[13\] and 1980 ACR criteria \[14\]. 2. Adult SLE Patients who fulfilled the 2019 EULAR / ACR classification criteria for systemic lupus erythematosus \[15\]
Exclusion criteria
1. Individuals with other autoimmune diseases. 2. Smoking. 3. Infections as chest infection, hepatitis C, hepatitis B and HIV infection. 4. Tumors as hepatocellular carcinoma. 5. Hypertension, diabetes, heart failure, hepatic diseases, chronic renal failure, intrarenal arteriovenous fistula, obstructive nephropathy and urinary tract obstruction. 6. Contraindication for MRI as implanted metallic devices.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Evaluate serum levels of CD147 in SSc and SLE patients | One year | Its correlation with renal impairment |
| Evaluate renal resistive index in SSC and SLE patients | One year | It's correlation with renal impairment |
| Evaluate Multiparametric MRI in SSC and SLE | One year | It's correlation with renal impairment |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Utility of Multiparametric MRI in SSC and SLE | One year | For early detection of renal impairment |
| Utility of CD 147 in ssc and SLE patients | One year | for early detection of renal impairment inandSLE patients |
| Utility of renal resistive index in SSC and SLE patients | One year | For early detection of renal impairment |