PubMed · 5535102
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G Wolf, G Canigiani, E Deimer. 1970. [Bone changes in association with scleroderma].. https://pubmed.ncbi.nlm.nih.gov/5535102/
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BACKGROUND: Although primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two independent autoimmune liver diseases, it is sometimes difficult to characterize the variant forms of autoimmune liver disease. A PBC scoring system, in combination with the AIH scoring system may be helpful to characterize such patients. METHODS: A PBC scoring system was introduced that selected 14 categories characteristic of PBC. One hundred and thirty-four patients with PBC, 31 patients with autoimmune cholangitis (AIC), 22 patients with overlap syndrome, and 48 patients with AIH were included in the study. The AIC patients fulfilled the PBC criteria but were negative for anti-mitochondrial antibody and positive for anti-nuclear antibody. Overlap syndrome patients fulfilled both the PBC and AIH criteria. RESULTS: The total scores (means +/- SD) for the PBC, AIC, overlap syndrome, and AIH patients were 23.3 +/- 4.7, 9.3 +/- 4.4, 18.0 +/- 5.9, and 3.6 +/- 3.3, respectively. When definite and probable PBC patients were defined as those with a total score of over 17 and 9-17, respectively, all except for 1 patient could be classified as definite or probable PBC. Four of the 48 AIH patients were classified as probable PBC. PBC scores for the variant autoimmune liver diseases showed a wide deviation. Plotting both PBC and AIH scores in a rectangular coordinate enabled us to locate each patient with variant forms according to the deviation from classical PBC or AIH. CONCLUSIONS: The PBC scoring system might be useful in characterizing the features of variant forms of autoimmune liver disease.
Hashimoto's thyroiditis and Graves' disease represent the two most common autoimmune thyroid disorders. Whereas in Hashimoto's thyroiditis FasL expression causes thyrocytes to undergo apoptosis, additional anti-apoptotic molecules appear to protect these cells in Graves' disease. Mutations of the FasL gene were observed in systemic lupus erythematosus. Given its functional relevance for the pathogenesis of thyroid autoimmunity we wondered whether variants of the FasL gene play a role in Hashimoto's thyroiditis and Graves' disease. We genotyped families with at least one offspring affected by Hashimoto's thyroiditis (n = 86) and Graves' disease (n = 90) for two FasL gene polymorphisms (C -843 T in the promoter, A IVS2nt-124 G in intron 2). Extended transmission disequilibrium (ETDT) and chi(2) testing were performed. Neither polymorphism alone nor the promoter/intron 2 haplotypes (p = 0.91) were associated with Hashimoto's thyroiditis. No association with Graves' disease was observed for the promoter polymorphism (p = 0.91) and the intron 2 "A" allele (57.1%; p = 0.36) or the promoter/intron 2 haplotypes (p = 0.31). Moreover, intron 2 genotyping revealed no difference between an additional 251 patients with Graves' disease and 197 healthy controls (p = 0.37). Italian and German families did not differ for the studied polymorphisms. In conclusion, our data do not suggest common genetic FasL variants to significantly contribute to the pathogenesis of either Hashimoto's thyroiditis or Graves' disease.
OBJECTIVES: This article should familiarize the reader with the pharmacokinetics, mechanisms of action, potential toxicities, and current and future applications of mycophenolate mofetil. DATA SOURCES: The current medical literature. RESULTS: Mycophenolate mofetil is an immunosuppressive agent that has been used for transplant recipients. More recently, this agent has been used to treat several inflammatory conditions. CONCLUSIONS: Mycophenolate mofetil has the potential to be a useful agent in the treatment of several inflammatory conditions, including systemic lupus erythematosus.