Renal problems in scleroderma: pathogenesis, clinical manifestations, and modern treatment methods

Аннотация

Systemic sclerosis (SSc) is an autoimmune disease of connective tissue characterized by fibrosis of the skin and internal organs, vascular damage, and immunological changes. One of the most severe complications is renal involvement, particularly scleroderma renal crisis (SRC). Renal crisis is one of the most serious complications of SSc and often occurs in diffuse SSc. This article discusses the pathogenesis, clinical manifestations, diagnostic methods, and modern approaches to treatment of renal involvement in SSc. Keywords: Systemic sclerosis, scleroderma renal crisis, chronic kidney disease, renovascular hypertension, antifibrotic therapy. with systemic sclerosis. The disease mainly develops Introduction: SSc is an autoimmune disease within the first 3-5 years. Among patients with characterized by impaired collagen turnover and scleroderma renal crisis, 40-50% lose their ability to progressive fibrosis of connective tissues. The disease work due to long-term complications. The mortality affects various internal organs, including the rate is 10-20%. In systemic sclerosis, various clinical and cardiovascular system, lungs, digestive system, and morphological forms of nephrological diseases occur, kidneys. Renal involvement in SSc is a serious and although their development depends on several complication that significantly impacts the patient's factors, all of them affect kidney function. In cases of quality of life and prognosis. Sclerodermic renal proteinuria or mild kidney dysfunction, the glomerular involvement, related to SSc, is a pathological process filtration rate (GFR) is 60-90 ml/min/1.73m². With the that develops slowly and manifests as progressive onset of scleroderma renal crisis, the GFR rapidly kidney failure [1, 8]. This condition is observed in a decreases and may drop below 30 ml/min/1.73m², significant portion of patients with SSc and is mainly leading to the development of chronic kidney disease associated with fibrosis of the renal glomeruli, [2]. SSc is a rare but severe systemic disease interstitial tissue, and blood vessels. Due to its slow characterized by endothelial dysfunction, tissue progression, sclerodermic renal involvement often fibrosis, and abnormal immune responses. Renal does not manifest clinically in the early stages and can involvement occurs in 20-50% of patients, and in 10- only be detected through laboratory tests. Another 15% of cases, kidney failure develops, which is a life- form of renal damage in SSc is SRC, which presents with threatening condition. This article provides an sudden onset of severe hypertension, kidney failure, overview of the pathogenesis, clinical manifestations, and microangiopathic hemolytic anemia. SRC is and treatment of renal complications in SSc. distinguished by its acute and severe progression, while sclerodermic renal involvement is a chronic and slowly Clinical Features progressing process. According to some researchers, Hypertension: This is a common problem in patients scleroderma renal crisis occurs in 5-20% of patients with SSc. Blood pressure remains consistently high, International Journal of Medical Sciences And Clinical Research 31 https://theusajournals.com/index.php/ijmscr International Journal of Medical Sciences And Clinical Research (ISSN: 2771-2265) leading to impaired kidney function. Proteinuria: Stages of CRF: CRF develops gradually and may be Kidney damage causes protein loss in the body, which, unnoticed in the early stages. However, as the disease in turn, leads to nephrotic syndrome. Increased progresses, the following symptoms may appear: Creatinine and Urea Levels: Due to worsening kidney Fatigue and weakness itchy and dry skin, loss of function, kidney dysfunction can result in the appetite, nausea, and vomiting swelling in the arms and accumulation of toxic substances in the body. Azotemia legs. Increased blood pressure sensation of tightness and Uremia: These conditions are characterized by an and shortness of breath sleep disturbances and increased concentration of nitrogenous compounds in difficulty concentrating. Diagnosis of CRF: to diagnose the blood, which occur when kidney filtration function CRF, the following tests and examinations are is impaired. If kidney function fails, nitrogenous waste conducted: Blood tests: Creatinine, urea, electrolytes products accumulate, leading to poisoning of the body. (Na, K, Ca, P) Calculation of Glomerular Filtration Rate Pathogenesis of Renal Involvement in SSc Scleroderma (GFR)Urine tests: Proteinuria, microalbuminuria, Renal Crisis is the most dangerous and acute erythrocytes. Kidney ultrasound (US) and biopsy (if manifestation of renal involvement, typically occurring necessary) [7]. Treatment Methods for CRF: Although in the diffuse form of systemic sclerosis. This condition CRF cannot be completely cured with specific is observed in 10-20% of patients and can lead to severe medications, the progression of the disease can be kidney failure [3]. Main Features