Scleroderma and kidney damage - Symptoms

Symptoms of kidney damage in scleroderma

Kidney damage in systemic scleroderma most often develops in patients with the diffuse cutaneous form of the disease, with its acute progressive course, within 2 to 5 years from the onset, although nephropathy may also develop with a chronic slowly progressive course of scleroderma. The main clinical signs of scleroderma nephropathy are proteinuria, arterial hypertension, and impaired renal function.

• Proteinuria is typical for most patients with systemic scleroderma with kidney damage. As a rule, it does not exceed 1 g/day, is not accompanied by changes in urinary sediment and in 50% of patients is combined with arterial hypertension and/or renal dysfunction. Nephrotic syndrome develops extremely rarely.
• Arterial hypertension is observed in 25-50% of patients with scleroderma nephropathy. Most patients are characterized by moderate arterial hypertension, which can persist for years without causing renal dysfunction. This form of arterial hypertension is observed in the slowly progressive form of systemic scleroderma and is characterized by late development. In 30% of hypertensive patients, malignant arterial hypertension develops, which is characterized by a sudden and sharp increase in arterial pressure over 160/100 mm Hg, combined with progressive deterioration of renal function up to the development of oliguric acute renal failure, with retinopathy, CNS damage and heart failure. This form of arterial hypertension has an unfavorable prognostic value.
• Chronic renal failure in patients with systemic scleroderma, like arterial hypertension, is associated with an unfavorable prognosis. In most cases of scleroderma nephropathy, chronic renal failure is combined with arterial hypertension, but it can also develop with normal blood pressure. In this case, a frequent combination of renal dysfunction with microangiopathic hemolytic anemia and thrombocytopenia is noted, which can manifest chronic renal failure.

The course and symptoms of scleroderma

Symptoms of scleroderma are characterized by significant polymorphism and include the following manifestations:

• skin lesion manifested by dense edema, induration, atrophy, foci of de- and hyperpigmentation;
• vascular damage with the development of generalized Raynaud's syndrome in more than 90% of patients, vascular-trophic disorders (digital ulcers, necrosis), telangiectasias;
• damage to the musculoskeletal system in the form of polyarthralgia, arthritis, myositis, muscle atrophy, osteolysis, soft tissue calcification, most often periarticular;
• damage to the respiratory organs with the development of fibrosing alveolitis and diffuse pneumofibrosis in most patients. With a long-term chronic course of the disease, pulmonary hypertension develops;
• gastrointestinal tract damage with predominant involvement of the esophagus (dysphagia, dilation, narrowing in the lower third, reflux esophagitis, strictures and ulcers of the esophagus), less often - the intestines (duodenitis, malabsorption syndrome, constipation, in some cases - intestinal obstruction);
• heart damage, most often represented by the development of interstitial myocarditis, cardiosclerosis, myocardial ischemia, which is clinically manifested by rhythm and conduction disturbances, increasing heart failure. Less common is endocardial damage with the formation of valve defects, primarily mitral, pericarditis;
• damage to the nervous and endocrine systems in the form of polyneuropathy, hypothyroidism, dysfunction of the sex glands.

General symptoms of scleroderma may include fever, weight loss, and weakness, which are observed during high disease activity.

Classification of systemic scleroderma

There are several subtypes (clinical forms) of systemic scleroderma. Depending on the prevalence and severity of skin changes, two main forms are distinguished - diffuse and limited.

In limited systemic scleroderma, observed in more than 60% of patients, the process is limited to the skin of the hands, neck and face, organ disorders occur late - 10-30 years after the onset of the disease.

Diffuse systemic scleroderma is characterized by generalized lesions of the skin of the trunk and extremities with early - within 5 years - development of visceral lesions. The limited form has a more benign course and a favorable prognosis compared to the diffuse one. According to the nature of the course, acute, subacute and chronic systemic scleroderma are distinguished.

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Kidney damage

The first description of kidney damage in systemic scleroderma was made in 1863 by H. Auspitz, who reported the development of acute renal failure in a patient with scleroderma, considering it an accident. Eighty years later, J. H. Talbot and co-authors first described intimal hyperplasia and fibrinoid necrosis of interlobular arteries in systemic scleroderma. However, it was only in the early 1950s, after H. C. Moore and H. L. Shechan described morphological changes in the kidneys of three patients with systemic scleroderma who died of uremia, that the possibility of severe nephropathy in scleroderma was recognized.

The exact incidence of nephropathy in systemic sclerosis is difficult to determine, since moderate renal damage in most cases is not clinically evident. The incidence of renal damage depends on the methods used to detect it: clinical symptoms of nephropathy are observed in 19-45% of patients with systemic sclerosis. The use of functional research methods allows us to detect a decrease in renal blood flow or glomerular filtration rate (GFR) in 50-65% of patients, and morphological signs of vascular renal damage are detected in 60-80% of patients.

Kidney damage, along with other visceritis, can develop in patients with a limited form of systemic scleroderma, but it mainly occurs with a diffuse cutaneous form of the disease. At the same time, a severe form of nephropathy in the form of increasing arterial hypertension and acute renal failure ("true scleroderma kidney") is observed in no more than 10-15% of patients.