Systemic sclerosis (SS), is characterized by the presence of thickening and hardening of the skin (from the Greek "sclero"), it has forms of limited and diffuse manifestation. It is chronic in pattern and manifests with vascular dysfunction and severe systemic connective fibrosis.

Systemic sclerosis (SS), is characterized by the presence of thickening and hardening of the skin (from the Greek "sclero"), it has forms of limited and diffuse manifestation. It is chronic in pattern and manifests with vascular dysfunction and severe systemic connective fibrosis. Sclerodermic renal crisis (SRC) is the severe manifestation in the kidney, and is characterized by:

• Malignant hypertension, of sudden onset associated with increased plasmarenin activity. There is a clinical variant,which presents with normotension, which has a worse prognosis. (UTAH)
• Acute Renal Failure(ARF).
• Proteinuria in the non-nephrotic range. Microscopic hematuria and casts are uncommon.

In our experience, we found the case of a 60-year-old woman with a history of systemic scleroderma with severe skin involvement and diagnosed Raynaud's phenomenon, due to skin ulcers, sclerodactyly, microstomia and marked sclerosis of the face and trunk; pulmonary hypertension and arterial hypertension. At the time of onset, he presentedwith severe arterialhypertension, which required systemic vasodilator drugs for its control, associated acute renal failure with signs of fluid overload. In the laboratory, increased nitrogen products, negative immunological markers of glomerular disease. After ruling out renal artery stenosis with Doppler ultrasound, treatment was started with enalapril 10mg every 12 hours, achieving rapid and effectivecontrol of blood pressure figures. Transthoracic echocardiogram shows increased pressure in the right heart chambers, and pulmonaryhypertension, starting treatmentwith tadalafil. Due to a tendency to oliguria, a renal  biopsy was performed, which reported "thrombotic

microangiopathy and subintimal arteriolar sclerosis linked to scleroderma renal crisis".

Given the findings in the pathology report, it was decided to continue treatment with enalapril 2.5mg every 12 hours, tolerating doses of up to 10mg per day. Renal failurepersists, associated with a decrease in urine volume, and he begins treatment with three-weekly hemodialysis, which continues until discharge from the clinic.He currently retainsurinary volume.

SRC is characterized by arterial hypertension, progressive renal failure, and thrombotic microangiopathy. It occurs in 5% of patients with SS. Diagnosisis based on clinical symptoms, associated with a higher risk of SRC, however, the presence of anti topoisomerase and anti- centromere antibodies are associated with a better prognosis. ACE inhibitors are the main treatment, even in the presence of renal failure. By being able to competitively inhibit the conversion of angiotensin I to angiotensin II, vasoconstriction and hypertension are reduced, and although both angiotensin I and renin continue to accumulate, they are biologically inactive.

Dialysis is indicatedfor fluid overloadand hypertension refractory to pharmacologicalmanagement, however, 55% of patients requiring renal replacement therapy (RRT) manage to gradually come out of it and present a decrease in nitrogen values. Ifafter 12 to 18 months, the patient continues with an indication for dialysis, transplantation is a therapeutic option. There is the possibility of relapse with the transplanted kidney, although this situationmust be differentiated with histological studies from toxicitydue to immunosuppressants.