Scleroderma and kidney damage: symptoms

Symptoms of kidney damage in scleroderma

The defeat of the kidneys in systemic scleroderma most often develops in patients with diffuse cutaneous form of the disease, with acute progressive course of the disease, at a period of 2 to 5 years from the beginning, although nephropathy can develop and with a chronic slowly progressing 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 it is combined with arterial hypertension and / or renal dysfunction. Nephrotic syndrome develops extremely rarely.
• Arterial hypertension is noted in 25-50% of patients with scleroderma nephropathy. For the majority of patients, moderate arterial hypertension is typical, which can persist for years without leading to impaired renal function. This form of arterial hypertension is noted with a slowly progressing form of systemic scleroderma and is characterized by late development. 30% of hypertensive patients develop malignant hypertension, which is characterized by a sudden and sharp increase in blood pressure more than 160/100 mm Hg, combined with progressive deterioration of kidney function until the development of oliguric acute renal failure, with retinopathy, CNS damage and cardiac insufficiency. This form of hypertension has unfavorable prognostic significance.
• Chronic renal failure in patients with systemic scleroderma, like arterial hypertension, is associated with an unfavorable prognosis. In most cases of scleroderma nephropathy chronic chronic insufficiency is combined with arterial hypertension, but it is possible to develop it even with normal blood pressure figures. In this case, there was a frequent combination of renal dysfunction with microangiopathic hemolytic anemia and thrombocytopenia, which chronic renal failure may manifest.

The course and symptoms of scleroderma

Symptoms of scleroderma differ in significant polymorphism and include the following manifestations:

• damage to the skin, manifested by dense edema, induration, atrophy, foci of de-and hyperpigmentation;
• vascular lesions with the development of generalized Raynaud's syndrome in more than 90% of patients, vascular-trophic disorders (digital ulcers, necrosis), telangiectasias;
• defeat of the musculoskeletal system in the form of polyartralgia, arthritis, myositis, muscle atrophy, osteolysis, calcification of soft tissues, more often periarticular;
• the defeat of the respiratory system with the development in most patients of fibrosing alveolitis and diffuse pneumofibrosis. With prolonged chronic course of the disease, pulmonary hypertension develops;
• gastrointestinal lesion with the primary involvement of the esophagus (dysphagia, enlargement, constriction in the lower third, reflux esophagitis, strictures and ulcers of the esophagus), less often - intestines (duodenitis, syndrome of impaired absorption, 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 a violation of rhythm and conduction, increasing heart failure. Less often mark the defeat of the endocardium with the formation of valvular defects, mainly mitral, pericarditis;
• defeat of the nervous and endocrine systems in the form of polyneuropathy, hypothyroidism of the thyroid gland, violation of the function of sexual glands.

Common symptoms of scleroderma can be represented by fever, weight loss, weakness, which are noted with 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.

With limited systemic scleroderma, which is noted in more than 60% of patients, the process is limited to the skin of the hands, neck and face, organ damage is late - 10-30 years after the onset of the disease.

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

[1], [2], [3], [4], [5], [6], [7], [8]

Renal damage

The first description of kidney damage in systemic scleroderma was made in 1863 by N. Auspitz, who reported on the development of acute renal failure in a patient with scleroderma, considering it an accident. Eighty years later, JH Talbot and co-authors for the first time described intimal hyperplasia and fibrinoid necrosis of interlobular arteries in systemic scleroderma. However, only in the early 50-ies of the XX century, after the description of NS. Moore and HL Shechan morphological changes in the kidneys of three patients with systemic scleroderma who died from uremia, the possibility of developing severe nephropathy in scleroderma was recognized.

The exact incidence of nephropathy in systemic scleroderma is difficult to determine, because moderately severe renal damage is not clinically evident in most cases. The frequency of renal failure depends on the methods used for its detection: clinical symptoms of nephropathy are noted in 19-45% of patients with systemic scleroderma. The use of functional research methods can detect a decrease in renal blood flow or glomerular filtration rate (GFR) in 50-65% of patients, and morphological signs of vascular renal involvement are revealed in 60-80% of patients.

Kidney damage, along with other viscerites, can develop in patients with a limited form of systemic scleroderma, but predominantly occurs with a diffuse cutaneous form of the disease. In this case, a severe form of nephropathy in the form of increasing arterial hypertension and acute renal failure ("true scleroderma kidney") is noted in no more than 10-15% of patients.