The northern part of Sweden is sparsely populated and must be regarded as a rural region. An investigation into the incidence and prevalence of primary biliary cirrhosis was conducted and the course of the disease was followed. In total, 111 patients with primary biliary cirrhosis were identified for the 10-yr period 1973 to 1982 in the northern health region of Sweden. The mean annual incidence amounted to 13.3 per million and the point prevalence was 151 per million, which is the highest reported so far. There was a significantly higher prevalence in the most northern county of Sweden, both with respect to total number of primary biliary cirrhosis patients and symptomatic patients. Asymptomatic patients amounted to 37%. During the study period 25 patients out of the 111 died (23%), 14 as a direct consequence of the liver disease. Three patients died of primary hepatocellular carcinoma, one having an asymptomatic liver disease without cirrhosis. Primary biliary cirrhosis seems to be more common in Sweden, especially in the northern part, than it is elsewhere. A high frequency of extrahepatic symptoms (85%), mainly musculoskeletal, was recorded. These symptoms may lead to the first contact with the health service, rather than signs of liver disease. Thus, an increasing number of patients are diagnosed with asymptomatic liver disease, who must be followed to check for the eventual development of symptoms.

Twenty-six consecutive patients with primary biliary cirrhosis (PBC) from northern Sweden were studied regarding the occurrence and features of Sjögren's syndrome (SS). In more than 50% of the patients the rose bengal dye test showed conjunctival and/or corneal staining. In six patients keratoconjunctivitis sicca (KCS) was present with positive rose bengal and Schirmer tests. In a further three patients only the results of the Schirmer tests were abnormal. Radiological findings of sialectasia were demonstrated in six patients, five of whom had KCS. Two of the seven patients who fulfilled our criteria for Sjögren's syndrome were HLA-B8 positive. A high prevalence of increased immune globulins and rheumatic factor was found, but this did not correlate with the presence of Sjögren's syndrome. Some features of Sjögren's syndrome were found in 73% of PBC patients, and keratoconjunctivitis sicca and/or sialectasia were found in 27% of PBC patients. This constitutes a high frequency of secondary manifestations of the liver disease.

ABSTRACT. In 22 patients with sarcoidosis, symptoms and signs of muscle involvement were scanty, but muscle biopsy showed epithelioid cell granulomas consistent with sarcoidosis in 11 patients (50%). Serial sectioning of the muscle biopsies increased the diagnostic yield. The incidence of muscle involvement was highest among patients with both acute sarcoidosis and erythema nodosum. Involvement of other organs was common in patients with muscle sarcoidosis. In two patients with negative scalene node biopsy and negative bronchial and trans‐bronchial biopsies, muscle biopsy showed histological evidence of sarcoidosis. Muscle involvement is common in patients with sarcoidosis, but in many cases there are few or no symptoms. In patients with both acute sarcoidosis and erythema nodosum, muscle biopsy frequently provides histological evidence of the disease. Good cooperation with the pathologist and serial sectioning of the biopsies are necessary if muscle biopsy is to be used as a routine in the diagnosis of sarcoidosis.

Twenty‐five patients with primary biliary cirrhosis (PBC) in different stages were investigated with respect to pulmonary function abnormalities. The results were compared with a reference sample of 17 sex‐ and age‐matched healthy subjects. A high prevalence of lung function impairment was found in the PBC patients (14/25 [56%]). Bronchial asthma was present in three patients, and severe lung emphysema in one. These four patients had an abnormal lung function, mainly of obstructive type. There was a statistically significant difference between the remaining 21 PBC patients without chronic obstructive lung disease and the reference subjects with respect to diffusion capacity. Almost all abnormal lung function data were found in the symptomatic PBC patients (i.e. symptoms of pruritus, xanthoma, xanthelasmata, jaundice, hyperpigmentation, hepatosplenomegaly), 13 out of 18 (72%), whereas only one out of seven asymptomatic patients was affected. Nine patients (36%) had reduced diffusion capacity compared with none of the reference subjects. The lung function abnormalities in PBC patients are similar to those found in sarcoidosis, another granulomatous disease.

C1 inhibitor and von Willebrand factor (vWF) levels were studied in patients with cholestatic or hepatocellular liver diseases. The vWF levels were greatly increased in hepatocellular liver diseases, whereas C1 inhibitor levels were slightly reduced. In cholestatic disease both the vWF and the C1 inhibitor levels were increased: among patients with primary biliary cirrhosis these increases were more pronounced in symptomatic patients than in asymptomatic ones. When compared with other protease inhibitors, the C1 inhibitor pattern in liver disease most closely resembled that of alpha 1-antitrypsin. Thus, C1 inhibitor levels cannot be used as a measure of residual hepatocyte mass in PBC; our data, however, suggested that antithrombin may be more suitable for that purpose.