Document EdQgGYrR3p27xoqb0eoxVpV8R

NOTICE THIS MATERIAL MAY BE PROTECTED BY COPYRIGHT LA** Sclerc^erma and portal hypertension JOHN S. MORRIS1*, THEIN HTUT>, AND A. E. READ1 From the Department ofMedicine, University ofBristol, Bristol Royal Infirmary Relationships between scleroderma and the gastro intestinal tract are well documented but involvement of the liver in this disease is less well recognized. The present communication describes four patients with scleroderma, each of whom had coincident liver disease. Case reports Cate 1, a 53-ytar-old wooan was diagnosed as having hypertension in 1963. The blood pressure was controlled with a diuretic. Later that year she developed Raynuud'* phenomenon in both hands and dysphagia became a prominent symptom. In 1968 she was admitted to hospital with a haematemesis and melaena and after preliminary investigations was transferred to the Bristol Royal Infirmary. Examination There was anaemia, and widespread telangiectases were particularly evident on the lips. Calcific nodules were prominent over thefingersand extended into the forearms. There were nuirerous spider naevi. There was nojaundice and no hepatic foetor. The spleen was palpable 4 cm. below the left costal margin and the liver edge extended to 6 cm. below the right costal margin. The blood pressure was 140/90 mm. Hg. Investigations Haemoglobin 13*3 g. per cent.; serum bilirubin 1*2 mg per cent.; serum proteins 81 g. per cent.; serum albumin 4*4 g./lOQ ml.; serum alkaline phosphatase 69 K.A. units (normal < 13 units); SOOT 125 I.U. (normal 5-46 units); tMAAlh mijtAlfi >nlilwwtv<^iv!i(|uA' antinuclear factor-- positive 1/20; mitochondrial antibodies--negative. A barium swallow examination showed oesophageal varices and splenoportography showed the portal and splenic veins to be patent. A skin biopsy showed changes of scleroderma. Treatment andprogress At operation an end-to-side porto-caval anastomosis was performed. The gall bladder and extrahcpatic biliary tracts were normal. The liver architecture was normal. There was some accumulation of lymphocytes and epithelioid cells near bile ducts. These 'granulomata' probably repre sented extravasated bile. The postoperative course was uneventful and the patient is alive and well 12 months later. Case 2, 49-year-old woman, came to the outpatient de partment in September 1969. One month previously she had taken aspirin for a headache after which she had had melaena. She had no haematemesis. In the previous month she had gained one stone In weight, and her abdomen had become distended. After a pregnancy complicated by toxaemia, 20 years earlier, (he patient had been hypertensive and was treated with a-methyl dopu. She had suffered from migraine for many years and had noticed that her fingers became numb and changed colour in cold weather and on Immersion in cold water. ' Examination She was clinically anaemic. The blood pressure was 150/90 mm. Hg. The skin over the middle phalanges was shiny and tight. The fingers were cold. Apart from ascites there was no extrahcpatic evidence of liver disease. Investigations Haemoglobin 7*4 g. per cent; serum bilirubin 1 *2 mg. per cent.; serum proteins 5*8 g./JOO ml; serum albumin 3*5 g./lOO ml.; LG-cell preparation negative; serum alkaline phosphatase 24 K.A. units; RA latex-fixation test- positive; smooth muscle antibody--negative; mito chondrial factor--negative; antinuclear factor--positive (1/12 dil.); bromosulphthaiein retention--21 per cent at 45 min. A barium swallow examination revealed oesophageal varices; splenoportography showed the portal and splenic veins to be patent; hepatic wedge pressure 16 mm. Hg; splenic pressure 31 mm.; I'1' rose Bengal scan within normal limits. Liver biopsy (operative) revealed that the liver archi tecture was grossly disturbed. The pattern was nodular, (he nodules being surrounded by rcticulin fibres and collagen. The portal areas were infiltrated by lymphoid cells. Twin cell plates were identified in some ofthe nodules. Skin biopsy revealed histological features typical of scleroderma. Treatment andprogress After a period of rest in bed and diuretic therapy the ascites gradually disappeared. Anaemia was corrected by a blood transfusion. The portal hypertension was relived operatively by an end-to-side porto-caval anastomosis. Since operation the patient has remained well and has needed no hypotensive therapy. Accepted for publication Novnmber 4,1971. _ * Prnent dUrew: Dmrtirant oTMedicin*. Rovl Free HoP<ia). Qry' Inn Rond, London, WCIX ILF. t Rncnrch A*ibHu>l, bepnnnwAl of McUkim, brtMot Royal Infirmary. * Colombo Plan Scholar, Department of Medicine, grtolol Royal Infirmary. * Prof--or of MadtcUa, UnlvtnUy of firletol. Z009ZGIZ Case 3, a 63-year-old woman, was admitted to the Bristol Royal Infirmary in 1966. She had been in another hospital because of a haematemesis due to bleeding oesophageal varices. In 1955 she hud developed Raynaud's phenomenon for which site had been treated, unsuccessfully, by bilateral cervical sympathectomy. In 1965, telangiectases developed Termination In March, J97J, the patient died. At post mortem the liver appeared cirrhotic. Histologically there was a well-marked lymphocytic infiltration of the portal tracts, with "aggressive" fibrosis. over the face, and together with calcified subcutaneous nodules and tight skin over the fingers suggested a diag Discussion nosis of scleroderma which was confirmed by skin biopsy. Examination In the Bristol Royal Infirmary in J 966 clinical examination confirmed the diagnosis of advanced scleroderma. Abdominal examination revealed marked abdominal distension with ascites. investigations Haemoglobin 12 g. per cent.; plasma proteins 7.8 g. per cent.; serum albumin 3 g. per cent.; serum alkaline phos phatase 29 K.A. units. Liver biopsy (operative)showed activechronic hepatitis. A barium swallow examination showed evidence of oesophageal varices. Treatment ami progress Porto-caval anastomosis was not performed. The oeso phageal varices were ligated through a transthoracic approach. Liver disorder was a prominent feature in four patients with scleroderma. Whilst involvement of the gastrointestinal tract in scleroderma is well recognized, hepatic involvement is leas common and is seldom a dominant feature of the condition. A recent American post mortem study of $8 patients with scleroderma revealed cirrhosis in five of 57 of the patients in whom liver tissue was available for examination, which was less than the eleven cases of, liver disorder found in a carefully matched control group (D'Angelo, Friels, Masi, and Shulman, 1969). Bartholomew, Cain, Winkelmann, and Baggenstou, (1964) were able to find only eight instances of liver involvement in 727 patients with scleroderma, whilst another large series of 150 cases docs not mention liver disease but comments oo arterial lesions which were found in the gall bladder Termination For 3 years after the operation the patient was well, but in 1969 she died in hepatic failure. (Leinwand, Duryee, and Richter, 1954). In Great Britain the situation appears to be different, for four patients with scleroderma and liver disorder have been seen in Bristol in the period 1965-1969 Case 4, a 65-year*oU woman, first became ill in 1953, when she presented with bilateral dropped wrist and dropped foot. There were few sensory signs. At the start of the illness a diagnosis of a lead neuropathy was made and she was treated with chelating agents. The neuro logical symptoms and signs persisted and became more severe, so that she was considerably disabled. The in a hospital in which an average of fifty new of cirrhosis are seen each year. Current opinion suggests that scleroderma is an autoimmune disorder and as such occasionally occurs with other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythemato sus (Rodnan, 1966). Although overt liver disorder clinical picture became that of motor neurone disease. For most of her life the patient had complained of symptoms of Raynaud's phenomenon. RccemJy she had complained of dysphagia. is rare in autoimmune conditions, the detection of antibody markers has shown that sub-clinical liver involvement may occur (Whaley, Goudie, William son, Nuki, Dick, and Buchanan, 1970; Walker, Examination In 1969 she hud a severe haematemesis. In hospital she was found to have subcutaneous calcinosis together with typical changes of scleroderma in the terminal ph^ny* Neurological examination confirmed the bilateral wrist Doniach, and Doniach, 1970). Such relationships strengthen the possibility of a connection between scleroderma and liver disease and suggest that the connection may be autoimmune. In the present patients liver involvement was and foot drop. Abdominal examination revealed marked reflected by gastrointestinal haemorrhage, indicating hepatosplenomegaly. in each case, portal hypertension. Calvert, Barling, Investigations Hb 11*9 g. per cent.; liver function test; bilirubin 0.7 mg. percent. ;albumin3.4g percent.; globulin 5.4 g. percent, (electrophoretic strip--raised y globulin); mitochondrial antibodies--negative; antibodies to smooth muscle-- negative; antinuclear factor--positive (1/80). A barium swallow showed oesophageal varices. Sopher, and Feiwal (1958) described two patients with hepatic cirrhosis and portal hypertension in whom alimentary haemorrhage was an important feature. Gastrointestinalhaemorrhageinsderoderma, however, may be associated with other manifestations of the disease. One patient has been described in whom haematemesis and melaena were Treatment amiprogress Tlte pullcul underwent ucsopliugeiil transection. Si Jmd no furtlier episode of gastrointestinal bleeding. with bleeding telangiectases ofthe stomach (Kolodny and Balter, 1968) and yet another patient had oesoplutgeut haemorrhage arising from ulcerative oesophagitis (Berliner and Burson, 1966). 003A BFG36438 "' O K'i5 <> O fc> 'irr "V ::;iL "T P-*U TSHi ivy5 6S 6 jr33:.t=i -c S3usr.cs5 -tast;j U 83u8B5S 48;13 "s-'hi 4U0Tjj 115 :5}5 >5uur,jj " PTM I L T.~i "l i-i YuuuuuuuvuuCIuuuiKiuuuuuEi " j* -j i .i 1 1 v j r:> i"i> rr ,-j ~ Nk!MMMWMuNh!NhiHfrihii\WMMNNX Ch ~ :-ii'.i I :: -7 " 3 IH53"'iii3iv ISvaludOHl 30 ivuliu3;ivl yivlmullud 33nI~ 3Hi 30 w5y"303iv :sr4fi .i^vj >iivfi ;5B5cd :aai/hi55i Tv6`t jiju ZZv :sr,s5i b :5*r'i5n 11111111 mu mu inn mu 1111111111 mu 1111111 11111111 mu 11111 inn mu nni 11111111111111111 11111111 mil 11111 inn inn nni 11111 nni 1111111 :hm5 I id fix. ^ H-ii; r 1:;:" '-. 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