Clinical scenario:
A 19yr old female presented with a history of fever and fatigue of 15 days duration.
Prior to current illness, she had an acute hepatitis-like illness without any viral prodrome or offending drug intake 7 months back. The illness had settled of its own within a month.
There was no other comorbidity and family history was insignificant.
Clinical examination :
The patient was conscious oriented hemodynamically stable with mild Jaundice.
There was no lymphadenopathy. A note was made of acne .
There was hepatosplenomegaly but no ascites. Other systemic examination was normal
Investigations
Kayser-Fleischer ring is a brownish-yellow annular deposition of copper located in the periphery (limbus) of the cornea (Descemet’s membrane) due to abnormal copper handling by the liver and with abnormalities of the basal ganglia of the brain, liver cirrhosis and splenomegaly.
Clinical challenge: Now are we dealing with AIH or WILSON disease?
The only test that could determine was the quantitative dry weight of copper in the liver biopsy which turned out to be high
The final diagnosis was Wilson disease and not Autoimmune hepatitis.
The patient was put on treatment. She is doing fine and is on follow up
Teaching message:
1. Acute hepatitis where AST is disproportionately more than ALT, always think of Wilson.
2. Always rule out Wilson in young patients with autoimmune hepatitis features.
3. Biopsy doesn't differentiate autoimmune hepatitis and Wilson. In fact, it may be similar in both as in this case.
4. Read, discuss and never shy away from discussing and taking help from your colleagues.
5. Always discuss with the patient and their caregivers your management plan.
Further Reading click the link: Wilson s disease
Acknowledgement:
I express my sincere thanks to Dr Zeeshan Consultant Gastroenterologist, for his assistance in the estimation of dry copper weight in Liver biopsy at Indian Institute of Liver and biliary sciences, New Delhi. This test clinched the diagnosis and the test is not done routinely at all centres
This case was contributed by
Dr Manzoor Ahmad Wani
D.M (Gastroenterology)
Consultant Gastroenterologist &Hepatologist
Govt.JLNM Hospital Rainawari
Srinagar Kashmir
.
A 19yr old female presented with a history of fever and fatigue of 15 days duration.
Prior to current illness, she had an acute hepatitis-like illness without any viral prodrome or offending drug intake 7 months back. The illness had settled of its own within a month.
There was no other comorbidity and family history was insignificant.
Clinical examination :
The patient was conscious oriented hemodynamically stable with mild Jaundice.
There was no lymphadenopathy. A note was made of acne .
There was hepatosplenomegaly but no ascites. Other systemic examination was normal
Investigations
- Her Bilirubin levels were elevated 3.4mg
- AST levels were elevated 200 and AST>ALT
- USG abdomen: Hepatosplenomegaly, no free fluid.No gallstones and Biliary radicals were normal
- Hepatitis serology A, B, C, and E were negative
- Serum ANA, ASMA, LKM1 negative
- Serum Ceruloplasmin, Iron profile were normal at first presentation.
- Serum IgG levels were high
- Fibroscan was 21kpa.
- The liver biopsy was reported as autoimmune hepatitis.
- Current presentation
- Repeat Wilson profile: Ceruloplasmin was 18 low
- 24-hour urinary copper was normal,
- KF ring was doubtful.
- A d_penicillamine challenge was done which turned out to be positive.
 |
| Fig 1 A typical KF ring. |
Kayser-Fleischer ring is a brownish-yellow annular deposition of copper located in the periphery (limbus) of the cornea (Descemet’s membrane) due to abnormal copper handling by the liver and with abnormalities of the basal ganglia of the brain, liver cirrhosis and splenomegaly.
Clinical challenge: Now are we dealing with AIH or WILSON disease?
The only test that could determine was the quantitative dry weight of copper in the liver biopsy which turned out to be high
The final diagnosis was Wilson disease and not Autoimmune hepatitis.
The patient was put on treatment. She is doing fine and is on follow up
Teaching message:
1. Acute hepatitis where AST is disproportionately more than ALT, always think of Wilson.
2. Always rule out Wilson in young patients with autoimmune hepatitis features.
3. Biopsy doesn't differentiate autoimmune hepatitis and Wilson. In fact, it may be similar in both as in this case.
4. Read, discuss and never shy away from discussing and taking help from your colleagues.
5. Always discuss with the patient and their caregivers your management plan.
Further Reading click the link: Wilson s disease
Acknowledgement:
I express my sincere thanks to Dr Zeeshan Consultant Gastroenterologist, for his assistance in the estimation of dry copper weight in Liver biopsy at Indian Institute of Liver and biliary sciences, New Delhi. This test clinched the diagnosis and the test is not done routinely at all centres
This case was contributed by
Dr Manzoor Ahmad Wani
D.M (Gastroenterology)
Consultant Gastroenterologist &Hepatologist
Govt.JLNM Hospital Rainawari
Srinagar Kashmir
.
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