Nephrogenic Systemic Fibrosis
Nephrogenic Systemic Fibrosis
This is a relatively infrequent disease usually occur in the patients of advance renal failure with or without dialysis. Nephrogenic systematic fibrosis is characterized by scleroderma and scleromyxedema with thickening and darkening developing on larger skin areas. This type of systematic fibrosis affects internal organs as well like heart, kidneys, lungs and muscles and tendons of joints.
Symptoms
Signs and symptoms include:
- Skin swelling and tightening
- Skin become hardened and thickened on arms and legs but never on face or head
- Skin starts to feel woody developing orange-peel appearance and excess pigmentation
- Loss of joint flexibility
- Ulcers and blisters sometimes appear
- Sharp pain, burning and itchy sensation on the affected skin
- Weakness of muscles
- Bone pain
- Contractures
- Blood clots
- Presence of yellow plaques on white part of eye
Causes
Although exact cause of this condition is not known but studies have found that exposure to gadolinium-containing contrast agents during magnetic resonance imaging (MRI) is a major trigger for the development of nephrogenic systemic fibrosis.
Some conditions exaggerate this disease when severe kidney failure is present or when exposure to gadolinium-containing contrast occur. Those conditions are:
- High dose of erythropoietin (EPO)
- Recently undergone vascular surgery
- Blood clotting problems
- Severe infection
Diagnosis
Diagnosis of this condition depends upon detailed medical history and complete general physical examination of the patient. Other tests include biopsy of the sample taken from skin or muscle.
Treatment
There is no permanent cure of this disease. Some techniques and procedures however aid in managing the symptoms and prevent any fatal consequences.
- Physical therapy
- Hemodialysis
- Kidney transplant
- Ultraviolet A phototherapy
- Extracorporeal photopheresis
- Plasmapheresis
- Certain medications are also used but their side effects limit their frequent use. Some of them are pentoxifylline (Pentoxil), imatinib (Gleevec).