True scleroderma kidney.

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 the diffuse cutaneous form of scleroderma with a progressive course (rapid progression of skin lesions over several months). Additional risk factors are old and senile age, male gender, and belonging to the Negroid race. They are also unfavorable in terms of the prognosis of acute scleroderma nephropathy.

The diagnosis of true scleroderma kidney is usually straightforward, 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 at the onset of the disease, simultaneously with skin manifestations and Raynaud's syndrome, or, which is especially difficult to diagnose, ahead of them ("scleroderma without scleroderma"). In a small number of patients, true scleroderma kidney develops after many years of favorable chronic renal disease.

Risk factors for true scleroderma kidney

Risk factors	Risk factors are not
Diffuse cutaneous form of systemic scleroderma Rapid progression of the skin process Duration of illness < 4 years Development of anemia de novo Development of cardiac damage de novo: pericardial effusion heart failure High doses of glucocorticoids	Existing arterial hypertension Changes in urine analysis Existing elevated blood creatinine Existing elevation of plasma renin

Acute scleroderma nephropathy is an urgent nephrological pathology, the diagnosis of which is based on the following criteria: sudden development of severe or increasing severity of existing arterial hypertension (blood pressure> 160/90 mm Hg); grade III-IV hypertensive retinopathy (hemorrhages in the fundus, plasmarrhea, optic disc edema); rapid deterioration of renal function; plasma renin activity increased at least twice as much as normal. Other typical signs are hypertensive encephalopathy (characterized by seizures), heart failure (often with the development of pulmonary edema), and 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, observed in almost all patients, usually precedes arterial hypertension and increases during the development of a true scleroderma kidney, although nephrotic syndrome does not develop. Erythrocytes and erythrocyte casts are determined in the urine sediment.

Until now, true scleroderma kidney remains the most common cause of death in patients with systemic scleroderma, despite the fact that the introduction of ACE inhibitors into practice has radically changed its prognosis (prior to the use of ACE inhibitors, patients died within 3-6 months). In order not to miss the onset 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 blood pressure monitoring is necessary, once every 3 months - determination of daily proteinuria and monitoring of renal function (Reberg test). Proteinuria exceeding 0.5 g / day, a decrease in SCF to 60 ml / min, persistent arterial hypertension require immediate treatment of scleroderma.