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Nier en sclerodermie: Wat moet men weten?

Scleroderma or systemic sclerosis is a chronic, systemic, autoimmune, inflammatory, connective tissue disease of unknown etiology characterized by fibrosis of the skin (scleroderma), the joints and internal organs (esophagus, intestine, lung, heart, kidneys). The skin is more often affected on the face and fingers, while in the course of the disease the whole body may be affected.

Scleroderma or systemic sclerosis is a chronic, systemic, autoimmune, inflammatory, connective tissue disease of unknown etiology characterized by fibrosis of the skin (scleroderma), the joints and internal organs (esophagus, intestine, lung, heart, kidneys). The skin is more often affected on the face and fingers, while in the course of the disease the whole body may be affected.

Is scleroderma a common phenomenon;
The incidence in the general population is about 30 per 100,000 people and it affects more women than men, in an approximate ratio of 4:1. The diseasepresents between the ages of 25 and 50 year, but it is uncommon in childhood and elderly.

What is the clinical picture?
Hard skin is usually classified into two categories - the localized and the systemic - depending on the degree of skin damage. The localized scleroderma attacks only the hands and feet and the systemic scleroderma attacks a large area of ​​the skin and one or more of the internal organs, often the kidneys, the esophagus, the heart and the lungs. Systemic scleroderma is additionally classified into diffuse and restricted scleroderma. From the skin, the hardening and thickening of the skin are the two symptoms that give the name to the disease. In addition, patients may experience ulcers, calcinosis, telangiectasias and other skin disorders.

I am diagnosed with scleroderma. Can it affect the kidney?
Yes of course. Systemic scleroderma may be characterized by a slow progressive renal impairment often accompanied by arterial hypertension and proteinuria at lower levels than that of the nephrotic syndrome, rarely from the so-called scleroderma renal crisis and rarely by rapidly progressive glomerulonephritis.

What is scleroderma renal crisis?
Scleroderma renal crisis occurs in approximately 20% of patients with generalized scleroderma, whereas the rates are very low in patients with local scleroderma. It is a serious complication and its main manifestation is high blood pressure, intense headache and chest discomfort as well as acute renal failure.

I am diagnosed withscleroderma. Should I visit a nephrologist?
Yes, it is very important to see a nephrologist to examine whether there is a glomerulonephritis-type of kidney damage, early diagnosis of arterial hypertension should be made, and appropriate treatment should be recommended, acute renal failure must be tackled, and the proper treatment of complications from a potential chronic kidney disease should be recommended.

How can renal complications be treated when combined with scleroderma?
Your nephrologist along with the rheumatologist will plan a follow-up plan and early diagnosis of renal involvement for an immediate treatment. Angiotensin-converting enzyme inhibitors and angiotensin-receptor blockers are the drugs recommended for the treatment of hypertension and improvement of the renal function.

What tests should I take to recognize the kidney complications immediately?
Early recognition of renal involvement and its treatment greatly improves the prognosis. It is recommended that blood pressure measurements be made on a regular basis by all patients with scleroderma. More specifically, a steady increase in systolic pressure equal to or more than 20 mmHg or an increase in diastolic by 10 mmHg should be directly investigated. It is not necessary to be elevated to "pathological" values, e.g. a patient who has a systolic pressure constantly of 110 mmHg if he suffers from scleroderma and his pressure steadily increases to 130 mmHg should be evaluated by a nephrologist and for 3 months he should check his renal function by performing tests for creatinine, urea and urine test  for albumin.

If I take prophylactic drugs for hypertension, will I prevent kidney damage?
No, there are no studies to support this. What, however is evident that rapid, direct and correct treatment of scleroderma and hypertension greatly improves the progression of the disease and its prognosis.

Mesogeios Dialysis Centers Group Scientific Team

Bibliography
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