Restricted use of Vlambda genes in POEMS syndrome.
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Base Sequence↗
Biomedical subjects
Jean Charles Piette
Publications and source records attributed to Jean Charles Piette.
Anetoderma and its prothrombotic abnormalities.
BACKGROUND: Anetoderma is characterized by circumscribed areas of flaccid skin due to the loss of elastic tissue in the dermis. It may be primary or secondary to various dermatoses. The primary form has been reported in association with autoimmune diseases and recently with antiphospholipid antibodies. Its etiology remains unknown. OBJECTIVES: To analyze clinical and laboratory data from a series of patients with anetoderma referred in our university reference center for connective tissue disorders. PATIENTS AND METHODS: All the consecutive patients with histologically confirmed anetoderma followed in our clinic from 1996 to 2001 were enrolled in this study. Laboratory investigations included the screening for prothrombotic abnormalities and classical immunological investigations for systemic lupus erythematosus. Clinical and laboratory data were analyzed retrospectively. RESULTS: Anetoderma was primary in 9 cases and secondary to lupus profundus in 2 cases. Prothrombotic abnormalities were detected in 10 patients (9/9 with primary and 1/2 with secondary anetoderma). Antiphospholipid antibodies were detected in 9 patients. Only 4 patients fulfilled criteria for definite antiphospholipid syndrome which was primary for 3 and associated with systemic lupus erythematosus in the other. CONCLUSION: Patients with anetoderma should be evaluated for the possible presence of a prothrombotic state and warned of its potential risks when present.
Adolescent↗
Hepatitis C virus infection in systemic lupus erythematosus: a case-control study.
OBJECTIVE: Viruses might be one of the elements that trigger systemic lupus erythematosus (SLE). Steroid therapy may influence the natural history of virus infections. The most frequent extrahepatic manifestations of hepatitis C virus (HCV) including arthralgia, myalgia, sicca syndrome, and antinuclear antibodies, may mimic a connective tissue disease, particularly SLE. Reports on the association between SLE and HCV infection are scarce. We investigated the association of HCV infection and SLE. METHODS: Retrospective case-control monocentric study of 19 patients with SLE and anti-HCV antibodies versus 42 randomized SLE patients without anti-HCV antibodies, matched for age and sex, coming from our cohort of 700 patients with SLE. SLE and HCV-infection features were reviewed. RESULTS: Mode of infection was blood product transfusion, drug addiction, or unknown. Prevalence of lupus clinical manifestations, antinuclear, anti-dsDNA, anti-extractable nuclear antigen antibodies, and complement levels was not different between HCV positive and negative SLE patients. Prevalence of cryoglobulin was higher in SLE patients with anti-HCV antibodies (p < 0.04), but none had a mixed cryoglobulinemia syndrome. ALT activity was increased in 11 HCV positive patients and 13 had detectable HCV RNA. Liver biopsy showed cirrhosis in 2 and mild fibrosis and activity in 5. One patient treated with interferon-alpha had a sustained virological response without SLE flare. Steroid therapy did not seem to alter HCV course. CONCLUSION: SLE in HCV positive patients shows higher prevalence of cryoglobulin without mixed cryoglobulinemia syndrome. HCV infection has moderate signs of biochemical and liver pathological severity. SLE by itself or treated with steroids does not seem to worsen HCV infection.
Adolescent↗
Human leukocyte antigen class II alleles may contribute to the severity of hepatitis C virus-related liver disease.
Whether the host's immune response genes influence the severity of hepatitis C virus (HCV) liver disease is controversial. Human leukocyte antigen (HLA) class II alleles were analyzed in 233 HCV RNA-positive patients with chronic active hepatitis (197 patients with Knodell index of fibrosis F0-F3 and 36 patients with index of F4). The 2 groups did not differ by sex, duration of infection, mode of contamination, alcohol consumption, or HCV genotype. Patients with cirrhosis were older than those without (56+/-12 vs. 46+/-14 years; P<10-4) and had a lower DRB1*11 allele frequency (5.6% vs. 14.5%; P=.037), whereas DRB1*03 and DQB1*0201 frequencies appeared to be higher (DRB1*03, 18.1% vs. 9.6%; DQB1*0201, 37.5% vs. 23.4%; P=.04, corrected P value is not significant). Mean index of fibrosis was higher in DR3-positive than in DR11-positive patients (2.14 vs. 1.58; P=.05). By multivariate analysis, cirrhosis was associated with male sex and age >50 years. HLA class II alleles may weakly contribute to the severity of HCV liver disease. Of persons infected with HCV, only 15%-20% spontaneously clear the virus, and the rest become chronically infected.
Adolescent↗
Drug-induced hemophagocytosis.
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Anti-Bacterial Agents↗
Impact of treatment on extra hepatic manifestations in patients with chronic hepatitis C.
BACKGROUND/AIMS: Fatigue and other extra hepatic manifestations of hepatitis C have never been studied prospectively in a large cohort. The aim was to assess the prevalence of these symptoms prior to any treatment, and on prolonged follow-up in treated and untreated patients. METHODS: A single-center cohort of consecutive patients with chronic hepatitis C was investigated prior to any treatment. A questionnaire was completed every 6 months for 18 months of follow-up. RESULTS: Of 1614 patients, 431 met the inclusion criteria (56% male; age 49 years; 60% with significant fibrosis or cirrhosis; 46% with cryoglobulinemia). Seventy-six were untreated; of the treated patients, 83 were sustained responders, 47 relapsers and 225 non-responders. At baseline, fatigue and other extrahepatic manifestations were present in 254 (59%) and 225 (52%) patients. Fatigue was improved in 29 of 83 (35%) responders versus 75 of 348 (22%) patients with detectable hepatitis C virus (HCV)-RNA (P=0.01). The impact of virologic response on fatigue persisted after adjusting for age, gender, fibrosis stage, and depression (odds ratio: 0.34, P<0.001). A cryoglobulin was detectable in two of 34 (6%) responders versus 38 of 115 (33%) patients with detectable HCV-RNA (P<0.001). CONCLUSIONS: In hepatitis C, a sustained virologic response is associated with a reduction in fatigue and cryoglobulin, but fatigue frequently persists despite a virologic response.
Adult↗