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 113.03.) 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, biliary atresia, chronic hepatitis, 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. Children can also have congenital
abnormalities of abdominal organs or inborn metabolic disorders that result in chronic
liver disease. Acute hepatic injury is frequently reversible as in viral, drug-induced,
toxin-induced, and ischemic hepatitis. In the absence of evidence of a chronic impairment,
episodes of acute liver disease do not meet 105.05.
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. Children can also have associated developmental delays
or poor school performance. 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 105.00D6).
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 age, medication tolerance, treatment response,
adverse effects of treatment, and duration of the treatment. Comorbid disorders, such
as HIV infection, may affect the clinical course of viral hepatitis infection(s) or
may alter the response to medical treatment.
(ii) We evaluate all types of chronic viral hepatitis infections under 105.05 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 whether
your impairment(s) functionally equals the listings.
b. Chronic hepatitis B virus (HBV) infection.
(i) Chronic HBV infection is diagnosed by the detection of hepatitis B surface antigen (HBsAg) in the blood
for at least 6 months. In addition, detection of the hepatitis B envelope antigen
(HBeAg) suggests an increased likelihood of progression to cirrhosis and ESLD.
(ii) The therapeutic goal of treatment is to suppress HBV replication and thereby
prevent progression to cirrhosis and ESLD. Treatment usually includes a combination
of interferon injections and oral antiviral agents. Common adverse effects of treatment
are the same as noted in 105.00D4c(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 determine whether your impairment(s) functionally equals the listings.
5. Gastrointestinal hemorrhage (105.02 and 105.05A). Gastrointestinal hemorrhaging can result in hematemesis (vomiting
of blood), melena (tarry stools), or hematochezia (bloody stools). Under 105.02, the
required transfusions of at least 10 cc of blood/kg of body weight must be at least
30 days apart and occur at least three times during a consecutive 6-month period.
Under 105.05A, 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 105.05A, we require
only one hospitalization for transfusion of at least 10 cc of blood/kg of body weight.
6. Ascites or hydrothorax (105.05B) indicates significant loss of liver function due to chronic liver disease.
We evaluate ascites or hydrothorax that is not attributable to other causes under
105.05B. 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 (105.05C) 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 105.05C 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 (105.05D) 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 105.05D 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 (105.05E) 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 105.05E are satisfied with documentation of any one of the findings on one evaluation.
In 105.05E1, 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 105.05E2; 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 105.05E2.
10. Hepatic encephalopathy (105.05F).
a. General. Hepatic encephalopathy usually indicates severe loss of hepatocellular function. We
define hepatic encephalopathy under 105.05F 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 105.05F3b. 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 105.05F.
b. Acute encephalopathy. We will not evaluate your acute encephalopathy under 105.05F 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 (105.05G1) and SSA Chronic Liver Disease-Pediatric (SSA CLD-P)
a. SSA CLD score.
(i) If you are age 12 or older, we will use the SSA CLD score to evaluate your ESLD
under 105.05G1. We explain how we calculate the SSA CLD score in a(ii) through a(vii)
of this section.
(ii) 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 [Loge (serum creatinine mg/dL)]
+3.78 [Loge (serum total bilirubin mg/dL)]
+11.2 [Loge (INR)]
(iii) 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 an example of SSA CLD calculation, see 5.00D11c.
(iv) 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.
(v) Listing 105.05G 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.
(vi) 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.
(vii) If you have the two SSA CLD scores required by 105.05G1, we will find that your
impairment meets the criteria of the listing from at least the date of the first SSA
b. SSA CLD-P score.
(i) If you have not attained age 12, we will use the SSA CLD-P score to evaluate your
ESLD under 105.05G2. We explain how we calculate the SSA CLD-P score in b(ii) through
b(vii) of this section.
(ii) To calculate the SSA CLD-P score, we use a formula that includes four parameters:
Serum total bilirubin (mg/dL), International Normalized Ratio (INR), serum albumin
(g/dL), and whether growth failure is occurring. The formula for the SSA CLD-P score
4.80 [Loge (serum total bilirubin mg/dL)]
+18.57 [Loge (INR)]
-6.87 [Loge (serum albumin g/dL)]
+6.67 if the child has growth failure (<-2 standard deviations for weight or height)
(iii) When we indicate ‘‘Loge’’ in the formula for the SSA CLD-P 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 a female child is 4.0 years old, has
a current weight of 13.5 kg (10th percentile for age) and height of 92 cm (less than the third percentile for age),
and has laboratory values of serum total bilirubin 2.2 mg/dL, INR 1.0, and serum albumin
3.5 g/dL, we will compute the SSA CLD-P score as follows:
4.80 [Loge (serum total bilirubin 2.2 mg/dL) = 0.788]
+18.57 [Loge (INR 1.0) = 0]
-6.87 [Loge (serum albumin 3.5 g/dL) =1.253]
= 3.78 + 0 -8.61 + 6.67= 1.84, which is then rounded to an SSACLD-P score of 2.
(iv) For any SSA CLD-P score calculation, all of the required laboratory values (serum
total bilirubin, INR, or serum albumin) must have been obtained within 30 days of
each other. We will not purchase INR values for children who have not attained age
12. If there is no INR value for a child under 12 within the applicable time period,
we will use an INR value of 1.1 to calculate the SSA CLD-P score. If there are multiple
laboratory values within the 30-day interval for any given laboratory test, we will
use the highest serum total bilirubin and INR values and the lowest serum albumin
value for the SSA CLD-P score calculation. We will round all laboratory values less
than 1.0 up to 1.0.
(v) The weight and length/height measurements used for the calculation must be obtained
from one evaluation within the same 30-day period as in D11b(iv).
(vi) Listing 105.05G2 requires two SSA CLD-P scores. The laboratory values for the
second SSA CLD-P score calculation must have been obtained at least 60 days after
the latest laboratory value for the first SSA CLD-P score and within the required
6-month period. We will consider the date of each SSA CLD-P score to be the date of
the first laboratory value used for its calculation.
(vii) If you have the two SSA CLD-P scores required by listing 105.05G2, we will find
that your impairment meets the criteria of the listing from at least the date of the
first SSA CLD-P score.
12. Extrahepatic biliary atresia (EBA) (105.05H) usually presents in the first 2 months of life with persistent jaundice.
The impairment meets 105.05H if the diagnosis of EBA is confirmed by liver biopsy
or intraoperative cholangiogram that shows obliteration of the extrahepatic biliary
tree. EBA is usually surgically treated by portoenterostomy (for example, Kasai procedure).
If this surgery is not performed in the first months of life or is not completely
successful, liver transplantation is indicated. If you have had a liver transplant,
we will evaluate your impairment under 105.09.
13. Liver transplantation (105.09) 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.