1. General. Chronic liver disease is characterized by liver cell necrosis, inflammation, or scarring (fibrosis or cirrhosis),
due to any cause, that persists for more than 6 months. Chronic liver disease may
result in portal hypertension, cholestasis (suppression of bile flow), extrahepatic
manifestations, or liver cancer. (We evaluate liver cancer under 13.19.) Significant
loss of liver function may be manifested by hemorrhage from varices or portal hypertensive
gastropathy, ascites (accumulation of fluid in the abdominal cavity), hydrothorax
(ascitic fluid in the chest cavity), or encephalopathy. There can also be progressive
deterioration of laboratory findings that are indicative of liver dysfunction. Liver
transplantation is the only definitive cure for end stage liver disease (ESLD).
2. Examples of chronic liver disease include, but are not limited to, chronic hepatitis, alcoholic liver disease, non-alcoholic
steatohepatitis (NASH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis
(PSC), autoimmune hepatitis, hemochromatosis, drug-induced liver disease, Wilson’s
disease, and serum alpha-1 antitrypsin deficiency. Acute hepatic injury is frequently
reversible, as in viral, drug-induced, toxin-induced, alcoholic, and ischemic hepatitis.
In the absence of evidence of a chronic impairment, episodes of acute liver disease
do not meet 5.05.
3. Manifestations of chronic liver disease.
a. Symptoms may include, but are not limited to, pruritis (itching), fatigue, nausea, loss of
appetite, or sleep disturbances. Symptoms of chronic liver disease may have a poor
correlation with the severity of liver disease and functional ability.
b. Signs may include, but are not limited to, jaundice, enlargement of the liver and spleen,
ascites, peripheral edema, and altered mental status.
c. Laboratory findings may include, but are not limited to, increased liver enzymes, increased serum total
bilirubin, increased ammonia levels, decreased serum albumin, and abnormal coagulation
studies, such as increased International Normalized Ratio (INR) or decreased platelet
counts. Abnormally low serum albumin or elevated INR levels indicate loss of synthetic
liver function, with increased likelihood of cirrhosis and associated complications.
However, other abnormal lab tests, such as liver enzymes, serum total bilirubin, or
ammonia levels, may have a poor correlation with the severity of liver disease and
functional ability. A liver biopsy may demonstrate the degree of liver cell necrosis,
inflammation, fibrosis, and cirrhosis. If you have had a liver biopsy, we will make
every reasonable effort to obtain the results; however, we will not purchase a liver
biopsy. Imaging studies (CAT scan, ultrasound, MRI) may show the size and consistency
(fatty liver, scarring) of the liver and document ascites (see 5.00D6).
4. Chronic viral hepatitis infections.
(i) Chronic viral hepatitis infections are commonly caused by hepatitis C virus (HCV), and to a lesser extent,
hepatitis B virus (HBV). Usually, these are slowly progressive disorders that persist
over many years during which the symptoms and signs are typically nonspecific, intermittent,
and mild (for example, fatigue, difficulty with concentration, or right upper quadrant
pain). Laboratory findings (liver enzymes, imaging studies, liver biopsy pathology)
and complications are generally similar in HCV and HBV. The spectrum of these chronic
viral hepatitis infections ranges widely and includes an asymptomatic state; insidious
disease with mild to moderate symptoms associated with fluctuating liver tests; extrahepatic
manifestations; cirrhosis, both compensated and decompensated; ESLD with the need
for liver transplantation; and liver cancer. Treatment for chronic viral hepatitis
infections varies considerably based on medication tolerance, treatment response,
adverse effects of treatment, and duration of the treatment. Comorbid disorders, such
as HIV infection, may accelerate the clinical course of viral hepatitis infection(s)
or may result in a poorer response to medical treatment.
(ii) We evaluate all types of chronic viral hepatitis infections under 5.05 or any
listing in an affected body system(s). If your impairment(s) does not meet or medically
equal a listing, we will consider the effects of your hepatitis when we assess your
residual functional capacity.
b. Chronic hepatitis B virus (HBV) infection.
(i) Chronic HBV infection can be diagnosed by the detection of hepatitis B surface antigen (HBsAg) or hepatitis
B virus DNA (HBV DNA) in the blood for at least 6 months. In addition, detection of
the hepatitis B e antigen (HBeAg) suggests an increased likelihood of progression
to cirrhosis, ESLD, and hepatocellular carcinoma. (HBeAg may also be referred to as
“hepatitis B early antigen” or “hepatitis B envelope antigen.”)
(ii) The therapeutic goal of treatment is to suppress HBV replication and thereby
prevent progression to cirrhosis, ESLD, and hepatocellular carcinoma. Treatment usually
includes interferon injections, oral antiviral agents, or a combination of both. Common
adverse effects of treatment are the same as noted in 5.00D4c(ii) for HCV, and generally
end within a few days after treatment is discontinued.
c. Chronic hepatitis C virus (HCV) infection.
(i) Chronic HCV infection is diagnosed by the detection of hepatitis C viral RNA in the blood for at least
6 months. Documentation of the therapeutic response to treatment is also monitored
by the quantitative assay of serum HCV RNA (“HCV viral load”). Treatment usually includes
a combination of interferon injections and oral ribavirin; whether a therapeutic response
has occurred is usually assessed after 12 weeks of treatment by checking the HCV viral
load. If there has been a substantial reduction in HCV viral load (also known as early
viral response, or EVR), this reduction is predictive of a sustained viral response
with completion of treatment. Combined therapy is commonly discontinued after 12 weeks
when there is no early viral response, since in that circumstance there is little
chance of obtaining a sustained viral response (SVR). Otherwise, treatment is usually
continued for a total of 48 weeks.
(ii) Combined interferon and ribavirin treatment may have significant adverse effects
that may require dosing reduction, planned interruption of treatment, or discontinuation
of treatment. Adverse effects may include: Anemia (ribavirin-induced hemolysis), neutropenia,
thrombocytopenia, fever, cough, fatigue, myalgia, arthralgia, nausea, loss of appetite,
pruritis, and insomnia. Behavioral side effects may also occur. Influenza-like symptoms
are generally worse in the first 4 to 6 hours after each interferon injection and
during the first weeks of treatment. Adverse effects generally end within a few days
after treatment is discontinued.
d. Extrahepatic manifestations of HBV and HCV. In addition to their hepatic manifestations, both HBV and HCV may have significant
extrahepatic manifestations in a variety of body systems. These include, but are not
limited to: Keratoconjunctivitis (sicca syndrome), glomerulonephritis, skin disorders
(for example, lichen planus, porphyria cutanea tarda), neuropathy, and immune dysfunction
(for example, cryoglobulinemia, Sjögren’s syndrome, and vasculitis). The extrahepatic
manifestations of HBV and HCV may not correlate with the severity of your hepatic
impairment. If your impairment(s) does not meet or medically equal a listing in an
affected body system(s), we will consider the effects of your extrahepatic manifestations
when we assess your residual functional capacity.
5. Gastrointestinal hemorrhage (5.02 and 5.05A). Gastrointestinal hemorrhaging can result in hematemesis (vomiting
of blood), melena (tarry stools), or hematochezia (bloody stools). Under 5.02, the
required transfusions of at least 2 units of blood must be at least 30 days apart
and occur at least three times during a consecutive 6-month period. Under 5.05A, hemodynamic instability is diagnosed with signs such as pallor (pale skin), diaphoresis (profuse perspiration),
rapid pulse, low blood pressure, postural hypotension (pronounced fall in blood pressure
when arising to an upright position from lying down) or syncope (fainting). Hemorrhaging
that results in hemodynamic instability is potentially life-threatening and therefore
requires hospitalization for transfusion and supportive care. Under 5.05A, we require
only one hospitalization for transfusion of at least 2 units of blood.
6. Ascites or hydrothorax (5.05B) indicates significant loss of liver function due to chronic liver disease.
We evaluate ascites or hydrothorax that is not attributable to other causes under
5.05B. The required findings must be present on at least two evaluations at least
60 days apart within a consecutive 6-month period and despite continuing treatment
7. Spontaneous bacterial peritonitis (5.05C) is an infectious complication of chronic liver disease. It is diagnosed by
ascitic peritoneal fluid that is documented to contain an absolute neutrophil count
of at least 250 cells/mm3. The required finding in 5.05C is satisfied with one evaluation documenting peritoneal
fluid infection. We do not evaluate other causes of peritonitis that are unrelated
to chronic liver disease, such as tuberculosis, malignancy, and perforated bowel,
under this listing. We evaluate these other causes of peritonitis under the appropriate
body system listings.
8. Hepatorenal syndrome (5.05D) is defined as functional renal failure associated with chronic liver disease
in the absence of underlying kidney pathology. Hepatorenal syndrome is documented
by elevation of serum creatinine, marked sodium retention, and oliguria (reduced urine
output). The requirements of 5.05D are satisfied with documentation of any one of
the three laboratory findings on one evaluation. We do not evaluate known causes of
renal dysfunction, such as glomerulonephritis, tubular necrosis, drug-induced renal
disease, and renal infections, under this listing. We evaluate these other renal impairments
9. Hepatopulmonary syndrome (5.05E) is defined as arterial deoxygenation (hypoxemia) that is associated with
chronic liver disease due to intrapulmonary arteriovenous shunting and vasodilatation
in the absence of other causes of arterial deoxygenation. Clinical manifestations
usually include dyspnea, orthodeoxia (increasing hypoxemia with erect position), platypnea
(improvement of dyspnea with flat position), cyanosis, and clubbing. The requirements
of 5.05E are satisfied with documentation of any one of the findings on one evaluation.
In 5.05E1, we require documentation of the altitude of the testing facility because
altitude affects the measurement of arterial oxygenation. We will not purchase the
specialized studies described in 5.05E2; however, if you have had these studies at
a time relevant to your claim, we will make every reasonable effort to obtain the
reports for the purpose of establishing whether your impairment meets 5.05E2.
10. Hepatic encephalopathy (5.05F).
a. General. Hepatic encephalopathy usually indicates severe loss of hepatocellular function.
We define hepatic encephalopathy under 5.05F as a recurrent or chronic neuropsychiatric
disorder, characterized by abnormal behavior, cognitive dysfunction, altered state
of consciousness, and ultimately coma and death. The diagnosis is established by changes
in mental status associated with fleeting neurological signs, including “flapping
tremor” (asterixis), characteristic electroencephalographic (EEG) abnormalities, or
abnormal laboratory values that indicate loss of synthetic liver function. We will
not purchase the EEG testing described in 5.05F3b; however, if you have had this test
at a time relevant to your claim, we will make every reasonable effort to obtain the
report for the purpose of establishing whether your impairment meets 5.05F.
b. Acute encephalopathy. We will not evaluate your acute encephalopathy under 5.05F if it results from conditions
other than chronic liver disease, such as vascular events and neoplastic diseases.
We will evaluate these other causes of acute encephalopathy under the appropriate
body system listings.
11. End stage liver disease (ESLD) documented by scores from the SSA Chronic Liver Disease (SSA CLD) calculation (5.05G).
a. We will use the SSA CLD score to evaluate your ESLD under 5.05G. We explain how
we calculate the SSA CLD score in b. through g. of this section.
b. To calculate the SSA CLD score, we use a formula that includes three laboratory
values: Serum total bilirubin (mg/dL), serum creatinine (mg/dL), and International
Normalized Ratio (INR). The formula for the SSA CLD score calculation is:
9.57 x [Loge(serum creatinine mg/dL)]
+3.78 x [Loge(serum total bilirubin mg/dL)]
+11.2 x [Loge(INR)]
c. When we indicate “Loge” in the formula for the SSA CLD score calculation, we mean the “base e logarithm” or “natural logarithm” (ln) of a numerical laboratory value, not the “base
10 logarithm” or “common logarithm” (log) of the laboratory value, and not the actual
laboratory value. For example, if an individual has laboratory values of serum creatinine
1.2 mg/dL, serum total bilirubin 2.2 mg/dL, and INR 1.0, we would compute the SSA
CLD score as follows:
9.57 x [Loge(serum creatinine 1.2 mg/dL) = 0.182]
+3.78 x [Loge(serum total bilirubin 2.2 mg/dL) = 0.788]
+11.2 x [Loge(INR 1.0) = 0]
= 1.74 + 2.98 + 0 + 6.43
= 11.15, which is then rounded to an SSA CLD score of 11.
d. For any SSA CLD score calculation, all of the required laboratory values must have
been obtained within 30 days of each other. If there are multiple laboratory values
within the 30-day interval for any given laboratory test (serum total bilirubin, serum
creatinine, or INR), we will use the highest value for the SSA CLD score calculation.
We will round all laboratory values less than 1.0 up to 1.0.
e. Listing 5.05G requires two SSA CLD scores. The laboratory values for the second
SSA CLD score calculation must have been obtained at least 60 days after the latest
laboratory value for the first SSA CLD score and within the required 6-month period.
We will consider the date of each SSA CLD score to be the date of the first laboratory
value used for its calculation.
f. If you are in renal failure or on dialysis within a week of any serum creatinine
test in the period used for the SSA CLD calculation, we will use a serum creatinine
of 4, which is the maximum serum creatinine level allowed in the calculation, to calculate
your SSA CLD score.
g. If you have the two SSA CLD scores required by 5.05G, we will find that your impairment
meets the criteria of the listing from at least the date of the first SSA CLD score.
12. Liver transplantation (5.09) may be performed for metabolic liver disease, progressive liver failure, life-threatening
complications of liver disease, hepatic malignancy, and acute fulminant hepatitis
(viral, drug-induced, or toxin-induced). We will consider you to be disabled for 1
year from the date of the transplantation. Thereafter, we will evaluate your residual
impairment(s) by considering the adequacy of post-transplant liver function, the requirement
for post-transplant antiviral therapy, the frequency and severity of rejection episodes,
comorbid complications, and all adverse treatment effects.