NEPHROGENIC SYSTEMIC FIBROSIS
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ALTERNATE NAMES
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Nephrogenic Fibrosing Dermopathy; NSD; NSF
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DESCRIPTION
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Nephrogenic Systemic Fibrosis (NSF) is a rare disease involving severe thickening and hardening of the skin (fibrosis)
overlying the extremities and trunk. The cause of nephrogenic systemic fibrosis is
attributed to the connexation of renal insufficiency and gadolinium exposure from
imaging studies. The exact degree of renal insufficiency that sets up the development
of nephrogenic systemic fibrosis is not known. Risk factors include advanced chronic
kidney disease (stages 3, 4 and 5) and acute or chronic inflammatory insults.
Symptoms of NSF include painful, burning itching skin, red/dark areas on the skin,
skin thickening, edema, loss of flexibility and severe limitations in movement at
the joints of the ankles, knees, feet, arms, wrists, and hands, and raised yellow
discoloration on sclera (white outer layer of the eyeball). Fibrosis (thickening)
involving the internal organs, including the lungs (with reduced diffusing capacity
for carbon monoxide) and diaphragm (with respiratory failure); myocardium, pericardium,
and pleura has been reported. NSF affects males and females in approximately equal
numbers. NSF may occur in children but most commonly affects the middle-aged.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: Diagnostic testing may include positive antinuclear antibody tests and the presence
of hepatitis B or C. All individuals affected with NSF have a history of renal insufficiency
of varying severity and duration and gadolinium exposure. A small number of individuals
have primary liver disease. Most individuals have had treatments that include hemodialysis,
peritoneal dialysis, or renal transplantation. However, neither dialysis nor renal
transplantation is a prerequisite for NSF. Histological findings may include thickened
collagen bundles with surrounding clefts, early lesions and spindle cells.
Physical findings: Symptoms of NSF include:
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Painful, burning itching skin;
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Red/dark areas on the skin;
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Skin fibrosis (scarring/thickening);
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Loss of flexibility and severe limitations in movement at the joints of the ankles,
knees, feet, arms, wrists, and hands;
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Raised yellow discoloration on sclera; and
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Fibrosis involving the internal organs, including the lungs (with reduced diffusing
capacity for carbon monoxide) and diaphragm (with respiratory failure), myocardium,
pericardium, and pleura.
ICD-9: 701.8
ICD-10: L90.8
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PROGRESSION
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NSF is a debilitating and sometimes fatal disease. People with a fulminant (disease
developing or progressing suddenly) form of NSF may become wheelchair dependent within
weeks due to development of flexion contractures and loss of mobility. Death may result
from complications of kidney disease or transplant surgery. In addition, fractures
and falls from wheelchair dependency may be fatal.
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TREATMENT
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NSF is usually a chronic progressive condition. Rare cases of partial to complete
spontaneous resolution have been reported in the absence of specific therapy, with
the return of renal function. A favorable response to medical intervention is anecdotal.
Of all treatments, extracorporeal photopheresis (ECP) seems to provide the best, albeit
mild and extremely expensive treatment modality for nephrogenic systemic fibrosis.
Steroids and massage therapies may be used to decrease the discomfort associated with
skin thickening and connective tissue formation.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical information documenting chronic kidney disease;
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Clinical examination including description of fibrotic changes and description of
functional limitations; and
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Laboratory studies documenting serum creatinine in the blood.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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6.03
8.09
106.03
106.05
108.09
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Use 6.03 when the adult claimant is on dialysis.
Use 106.03 when the child claimant is on dialysis.
Use 106.05 when the lab findings are at the required level.
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Equals
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1.18
6.05
14.04
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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