1 AIkugust 1970
Scleroderma and Primary Biliary Cirrhosis
I. M. MURRAY-LYON,* M.B., B.SC., M.R.C.P. (LOND., ED.)
I. D. ANSELL,4 M.A., M.R.C.PATH.
; R. P. H. THOMPSON,t D.M., M.R.C.P.
; ROGER WILLIAMS,§ M.D., F.R.C.P.
BritishMedical_Journal, 1970, 3, 258-259
sclerosis with primary biliary cirrhosis of six year' dura-
tion at the stage of ductular prolifetion. The odte
the C.R.S.T. syndrome (calcinis, Raynaud's phenome-
biliary cirrhosis at the florid stage. Several similar cases
were found in a review
of other reports, and
suggested that the association may be due to a common
of scieroderma and primary
described. One had systemc
Though there are reports, dating from 1934, of liver disease
occuring in patients with scieroderma few are given in suf-
ficient detail to allow a valid assessment of the type of invol-
vement. Two of the cases described by Bartholomew et al.
(1964), however, had features suggestive of primary biliary
cirrhosis and Reynolds et al. (1970) drew attention to this
association. In this paper we describe the cases of two patients
with scleroderma who have the distinctive immunological and
histological picture of primary biliary cirrhosis, and discuss
the possible mechanisms involved.
In 1963 a 64-year-old woman with mild Raynaud's phenomenon
was found to have hepatosplenomegaly. In 1965 she complained of
generalized itching, and telangiectases of face and arms were noted.
In 1966 bone radiographs revealed an unusual patchy osteoporosis,
but a biopsy specimen from the right radius showed normal his-
tology. It was followed by a fracture, and the skin of the right
tongue, and lips. They were bluish red and blanched on pressure,
but had no central arteriole. No bleeding occurred, and there was
no family history of bleeding or telangiectases.
oesophagus with absence of peristalsis, and radiographs of the
hands showed acrolysis of the terminal phalgeal bones. Liver
function tests fluctuated, but changed little overall in three years:
plasma bilirubin 1-6-3*5 mg./100 ml., alkalin
units/100 ml., serum cholesterol 225-370 mg./100 ml. Serum IgA
immunoglobulin (300 mg./100 mL) was raised and IgM (185) and
IgG (1,300) fractions were normal. Immunofluorescent and com-
plement fixing antibodies to mitochondria had been present in low
titre since 1966 and smooth muscle antibodies and antinuclear
factor had developed in the past year (Dr. Deborah Donmach).
Needle liver biopsy specimens taken in 1966 and 1969 were
similar and showed considerable ductular proliferation
with portal fibrosis and early septum formation in the later biopsy
lymphocytes, neutrophil polymorphs, and plasma cells and there
was piecemeal necrosis, pronounced in the second biopsy speci-
men. The features were those of primary biliary cirrhosis
stage of ductular proliferation.
so. Macular telangiectases were present on the face, arms,
*Senior Registrar in Medicine.
tM.R.C. Clinical Research Fellow and Honorary Registrar.
Lecturer in Morbid Anatomy.
Physician and Director of Liver Research Unit.
Kng's College Hospital, London S.E.5.
FIG. l.-Case 1. Portal tract with ductular proliferation and piecemeal
necrosis. (haematoxylin and eosin.
A62-year-old woman with Raynaud's phenomenon had noted
telangiectases for 10 years. There were sclerodermatous changes
in the fingers with small pitted
Telangiectases were present on the hands, lips, tongue, and feet,
and there was pronounced hepatosplenomegaly. There was no
relevant family history and examination
Investigations.-Serum bilirubin 0 7 mg./100 ml., aspartate trans-
aminase 190 units/100 ml. (normal
alkaline phosphatase 44 K.A. units/100 ml., serum cholesterol
236 mg./100 ml. Serum IgG (1,850 mg./100 ml.) and IgM
(560 mg./100 ml.) were raised and IgA was normal. Mitochondrial
antibodies and the sensitized sheep cell test were positive at a
titre of 1/256, and smooth muscle antibodies and
factor were weakly positive. (Dr. Deborah Doniach). Radiographs
of the hands showed acrolysis of the terminal phalanges with soft
tissue calcfication and the barium swallow diminished peristalsis.
A needle liver biopsy specimen showed retention of normal
scars and areas of calcinosis.
110 units/100 ml.),
FIG 2.-Case 2. Portal tract showing dense infiltrate of lymphocytes
parenchyma is normaL (H. & E. X 170)