True scleroderma kidney

The true scleroderma kidney is the most severe manifestation of scleroderma nephropathy. It develops in 10-15% of patients with systemic scleroderma, usually in the first 5 years from the onset of the disease, more often in the cold season. The main risk factor for its development is a diffuse cutaneous form of scleroderma with progressive course (rapid progression of skin lesions within several months). Additional risk factors - elderly and senile age, male gender, belonging to the Negroid race. They are also unfavorable for the prognosis of acute scleroderma nephropathy.

Diagnosis of a true scleroderma kidney usually does not cause difficulties, since this form of nephropathy develops in patients with established systemic scleroderma. However, in 5% of cases, the development of acute scleroderma nephropathy is noted in the onset of the disease, concomitantly with cutaneous manifestations and Raynaud's syndrome, or, which is especially difficult to diagnose, ahead of them ("scleroderma without scleroderma"). In a small number of patients, the true scleroderma kidney develops after many years of a favorable course of the chronic renal process.

Risk factors for true scleroderma kidney

Risk factors	Risk factors are not
Diffusive cutaneous system scleroderma Rapid progression of the skin process Duration of the disease <4 years Development of anemia de novo Development of the defeat of the heart de novo: effusion in the pericardial cavity of heart failure High doses of glucocorticoids	Existing arterial hypertension Changes in the analysis of urine Existing increase in blood creatinine Existing elevation of plasma renin

Acute scleroderma nephropathy is an urgent nephrologic pathology for the diagnosis of which the following criteria are used: sudden development of severe or increasing severity of existing arterial hypertension (arterial pressure> 160/90 mm Hg); hypertensive retinopathy of III-IV degree (on the fundus of hemorrhage, plasmorrhagia, edema of the optic nerve disk); rapid deterioration of kidney function; The plasma renin activity increased at least twice as compared with the norm. Other typical signs are hypertensive encephalopathy (characterized by convulsions), heart failure (often with the development of edema of the lungs), microangiopathic hemolytic anemia. In some cases of true scleroderma kidney, oliguric acute renal failure develops in the absence of arterial hypertension or with a moderate increase in blood pressure. Proteinuria, noted in almost all patients, usually precedes arterial hypertension and increases during the development of a true scleroderma kidney, although the nephrotic syndrome is not formed. In the urine sediment, erythrocytes and erythrocyte cylinders are determined.

Until now, the true scleroderma kidney remains the most common cause of death of patients with systemic scleroderma, despite the fact that the introduction of ACE inhibitors into the practice radically changed its prognosis (before the use of ACE inhibitors, patients died within 3-6 months). In order not to miss the start of acute scleroderma nephropathy, taking into account the peculiarities of its development, all patients with diffuse systemic scleroderma, especially in the first 5 years of the disease, should be carefully monitored. Monthly monitoring of blood pressure is necessary, once every 3 months - determination of daily proteinuria and control of kidney function (Reberg's test). Proteinuria, exceeding 0.5 g / day, a reduction in GFR to 60 ml / min, persistent hypertension requires the immediate start of treatment for scleroderma.