1. Nagral Aabha (First and corresponding author)
Institution:
** Department of Medicine, Kasturba Hospital of Infectious Diseases, Mumbai
# Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai
Email id:
aabhanagral@gmail.com
Address:
7, Sneha Sagar, Prabha Nagar, Prabhadevi Mumbai – 400025
2. Jhaveri Ajay
Institution:
# Department of Gastroenterology, Jaslok Hospital and Research Centre, Mumbai
3. Nalawade Smita
Institution:
** Department of Medicine, Kasturba Hospital of Infectious Diseases, Mumbai
4. Momaya Nandini
Institution: * Department of Ophthalmology, Bhatia Hospital, Mumbai
5. Chakkarwar Vaibhav
Institution:
** Department of Medicine, Kasturba Hospital of Infectious Diseases, Mumbai
6. Malde Priya
Institution:
** Department of Medicine, Kasturba Hospital of Infectious Diseases, Mumbai
15-198_L_Edited
LETTER to the Editor
Kayser-Fleischer rings or bile pigment rings ?
Nagral A**#, Jhaveri A#, Nalawade S**, Momaya N*, Chakkarwar V**, Malde P**
Department of Medicine, Kasturba Hospital of Infectious Diseases, Mumbai 400 011, India**,
Department of Gastroenterology, Jaslok Hospital, 15, Dr. Deshmukh Marg, Pedder Road,
Mumbai 400 026, India#, Department of Ophthalmology, Bhatia Hospital, Tukaram Javaji Marg,
Grant Road West, Tardeo, Mumbai 400 007, India *
Dear Editor:
We read with interest the case study by Sood V et al. “Cholestatic hepatitis masquerading as
Wilson disease” [1] where the authors emphasize that Wilson disease may be falsely diagnosed
by the AASLD guidelines for this disease [2]. They described 4 such patients who were falsely
diagnosed as Wilson disease on the basis of “guidelines” and were actually patients of sclerosing
cholangitis (mimickers). In addition to a low ceruloplasmin all four patients had a KayserFleischer (K-F)-ring
We would like to share our observation on 40 adult patients of “prolonged acute hepatitis” with
K-F-like rings. Forty patients (31:9, M:F) with acute hepatitis of varied etiologies (hepatitis E:12,
hepatitis A:1, hepatitis B:11, alcoholic hepatitis:4 and 12 with unknown etiology) with total
bilirubin >20 mg/dL of more than 6 weeks duration (range 15–45 days) were subjected to naked
eye and slit lamp examination of the eyes by a single experienced ophthalmologist (who was
blinded to the diagnosis of the patients) at the time of admission and after drop in bilirubin below
10 mg/dL. Median serum bilirubin level was 26 mg/dL (range 21-55.2 mg/dL) at the time of the
first slit lamp examination. A K-F-like ring was seen in 37 of 40 patients (92.5%). It was not
visible on naked eye examination in any of the patients. A slit lamp examination was repeated
when serum bilirubin decreased to less than 10mg/dL. The K-F-like ring had disappeared in all
the patients. Four patients had low serum ceruloplasmin, but normal 24 hour urinary copper. The
ceruloplasmin levels normalised in all 4 patients after the bilirubin returned to normal values.
K-F-rings signify brain involvement in Wilson disease and are seen in 95 % of patients with
neurological presentation of Wilson disease [2]. Though KF rings have been occasionally
described in chronic cholestasis, we suspect that these were actually K-F-like rings. There is no
documentation in literature of K-F or K-F-like rings in the setting of prolonged acute hepatitis.
We have anecdotally had the experience of patients being wrongly diagnosed as Wilson disease
in the acute and chronic setting based on a positive K-F-ring which later disappeared. In our
study, K-F-like rings were seen in prolonged acute hepatitis, most of which were cholestatic, due
to bile staining of the peripheral stromal layer of the cornea (Fig. 1). The differences between the
K-F-rings and K-F-like rings (bile pigment rings) as experienced and perceived by us are shown
in Table 1.
Table 1 Differences between Kayser-Fleischer rings and Kayser-Fleischer like rings
Kayser-Fleischer rings
Kayser-Fleischer-like
rings
(bile
pigment
rings)
Naked eye appearance
Rings may be seen
Rings not seen
Site of the ring
Superior/inferior/
Always circumferential
circumferential
Color
Golden brown
Yellowish green
Texture
Granular
Homogenous
Layer of cornea involved
Descemet’s membrane
Peripheral stroma
Related to bilirubin level
No
Yes
Response
to
chelation Yes
Not applicable
therapy
Wilson disease is the most common cause of the K-F-ring and was once thought to be
pathognomonic of Wilson disease. However, K-F-rings have been reported in many other
conditions like chronic cholestatic diseases, [2, 3] primary biliary cirrhosis [3], autoimmune
chronic active hepatitis [5]. K-F-like rings, better termed bile pigment rings are seen in majority
of patients with high bilirubin and disappear when serum bilirubin levels below 10 mg/dl. We
believe that most of the cases described in literature seem to be K-F-like rings. The term “bile
pigment rings” may be more appropriate term to describe these K-F-like rings seen in the setting
of severe hyperbilirubinemia. Ophthalmologists and hepatologists need to be vigilant about
interpreting K-F-rings in the setting of severe jaundice.
References
1. Sood V, Rawat D, Khanna R, Alam S. Cholestatic liver disease masquerading as Wilson
disease. Indian J Gastroenterol. 2015;34:174-7.
2. Roberts EA1, Schilsky ML, American Association for Study of Liver Diseases
(AASLD).
Diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;
47:2089-111.
3. Fleming CR, Dickson ER, Wahner HW, Hollenhorst RW, McCall JT. Pigmented corneal
rings in non-Wilsonian liver disease. Ann Intern Med. 1977;86:285-8.
4. Williams EJ, Gleeson D, Burton JL, Stephenson TJ.
Kayser-Fleischer like rings in
alcoholic liver disease: a case report. Eur J Gastroenterol Hepatol. 2003;15:91-3.
5. Zargar SA, Thapa BR, Sahni A, Mehta S. Kayser-Fleischer like ring in autoimmune
chronic active hepatitis. Indian J Gastroenterol. 1991,10:101-2.