Morphologic capillary changes and manifestations of connective tissue diseases in patients with primary biliary cirrhosis

Abstract

The objective of this study was to investigate the presence of nailfold capillary abnormalities and extrahepatic signs of connective tissue disease in patients with primary biliary cirrhosis (PBC), as compared to patients with other chronic liver diseases. We evaluated 22 patients with PBC and 15 patients with other chronic liver diseases as a control group. Nailfold capillaroscopy was performed by two observers blinded to clinical findings using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. We detected nailfold capillary abnormalities in 20 out of 22 (91%) PBC patients. Twelve of these 20 patients (54%) showed capillary alterations characteristic of systemic sclerosis. In the control group only two out of 15 patients (13%) presented alterations and in both cases they were a non-specific type. The presence of nailfold capillary abnormalities was significantly greater in PBC patients than in the control group (P < 0.001). Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease and most of them were related to systemic sclerosis; patients with other chronic liver diseases did not present rheumatic manifestations (P < 0.001). In PBC patients there was a significant association between systemic sclerosis capillary pattern and rheumatic manifestations (P < 0.03). The high prevalence of nailfold capillary abnormalities characteristic of systemic sclerosis in patients with PBC and the correlation with sclerodermal manifestations suggests that this capillaroscopic finding could be a useful indicator to investigate rheumatic manifestations in these patients.

Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis

“…The use of nailfold capillaroscopy in patients suspected of having connective tissue disease may be a useful indicator. 56 In conclusion, patients with PBC and SSc had a lower rate of liver transplantation and a lower rate of liver related deaths, with a slower rate of bilirubin increase with respect to patients with PBC alone. However, these differences were not due to earlier SSc related deaths.…”

Diagnostic and clinical significance of anti-centromere antibodies in primary biliary cirrhosis

“…A recent report from EUSTAR has suggested that the early diagnosis of SSc should be based in three domains: skin (puffy fingers/puffy swollen digits turning into sclerodactily), vascular (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory (testing for ANA in general and ACA and ATA in particular) domains [162]. In addition, it has been suggested that videocapillaroscopy should be incorporated in the diagnostic algorithm of PBC patients with suspected SSc [163]. Besides capillaroscopy, assessment of lung function by spirometry and echocardiography should be considered in the group of PBC patients diagnosed with SSc [26].…”

Emerging Issues in the Immunopathogenesis, Diagnosis and Clinical Management of Primary Biliary Cirrhosis Associated with Systemic Sclerosis

“…Experimental and clinical observations suggest that endothelial dysfunction is present in PBC patients. One study found nailfold videocapillaroscopy abnormalities in 91% of patients with PBC, and 54% had capillary alterations characteristic of SSc (Fonollosa et al, 2001). Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease with most being related to SSc, while patients with other types of chronic liver disease did not present with rheumatic manifestations (Fonollosa et al, 2001).…”

“…One study found nailfold videocapillaroscopy abnormalities in 91% of patients with PBC, and 54% had capillary alterations characteristic of SSc (Fonollosa et al, 2001). Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease with most being related to SSc, while patients with other types of chronic liver disease did not present with rheumatic manifestations (Fonollosa et al, 2001). The high prevalence of nailfold capillary abnormalities characteristic of SSc in patients with PBC, and correlation with sclerodermal manifestations, suggests that this capillaroscopic finding could be a useful indicator to investigate rheumatic manifestations in these patients (Fonollosa et al, 2001).…”

“…Eleven out of the 22 PBC patients (50%) had extrahepatic signs of connective tissue disease with most being related to SSc, while patients with other types of chronic liver disease did not present with rheumatic manifestations (Fonollosa et al, 2001). The high prevalence of nailfold capillary abnormalities characteristic of SSc in patients with PBC, and correlation with sclerodermal manifestations, suggests that this capillaroscopic finding could be a useful indicator to investigate rheumatic manifestations in these patients (Fonollosa et al, 2001). Further clinical assessment of organ involvement (especially lung by spirometry) in association with evaluation of pulmonary artery pressure on echocardiography, should be considered in PBC patients diagnosed with SSc.…”