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Biomedical subjects

Jean Cabane

Publications and source records attributed to Jean Cabane.

15 recordsLinked to original sources
PubMed2006

Occupational exposure to solvents and gender-related risk of systemic sclerosis: a metaanalysis of case-control studies.

OBJECTIVE: In 2001 a metaanalysis reported an excess risk of systemic sclerosis (SSc) related to solvents exposure. The magnitude of risk varied among studies and sources of heterogeneity have not been investigated due to a lack of statistical power. We conducted a new metaanalysis to identify features associated with the magnitude of SSc risk in patients exposed to solvents. METHODS: We searched 4 databases (Medline, Pascal, Pascal Biomed, Francis). Inclusion criteria were: case-control study, occupational exposure to solvents (OES) assessed by questionnaire and summarized to "any solvent" or "any organic solvent," SSc defined by the American College of Rheumatology or the consultant's criteria. The quality of studies within this metaanalysis was scored according to the Newcastle-Ottawa scale. Odds ratios (OR) were adjusted for the "publication bias" and validated by a sensitivity analysis. Subgroup analyses investigated the effect of gender, quality of studies, and the type of controls. RESULTS: Among 11 studies (1291 patients and 3435 controls), 9 involved a majority of women (76.2 to 100%), while 2 involved men only. The risk of SSc associated with OES was variable among studies (p for heterogeneity = 0.01) and overrepresentation of higher OR values in smaller studies (p = 0.003) suggested "publication bias." SSc was associated with OES (OR 2.4; 95% CI 1.7-3.4; p < 0.0001), including after adjusting for bias (OR 1.8; 95% CI 1.2-2.5; p = 0.002). The relative risk was higher (p = 0.03) in men (OR 3.0; 95% CI 1.9-4.6; p < 0.0001) than in women (OR 1.8; 95% CI 1.5-2.1; p < 0.0001). CONCLUSION: Whereas SSc affects women predominantly, among subjects with occupational exposure to solvents, men are at higher risk than women for the disease.

Case-Control Studies↗
PubMed2006

Severe strongyloidiasis in corticosteroid-treated patients: case series and literature review.

OBJECTIVE: To describe the main features of severe strongyloidiasis in corticosteroid-treated patients METHODS: We report on 3 cases of corticosteroid-treated patients with severe strongyloidiasis and review cases of severe strongyloidiasis in corticosteroid-treated patients reported in the literature. RESULTS: One hundred and fifty-one cases of severe strongyloidiasis complicated a therapy with corticosteroids were evaluated. The mean age of the patients was 48+/-17 years and 71% were men. Corticosteroids were given for hematological malignancies in 34 (23%), systemic lupus erythematosus or vasculitis in 27 (18%), and nephropathy or renal transplantation in 32 (21%). At time of infection, the mean daily dosage of prednisone-equivalent was 52+/-42 mg (median: 40 mg) and 84% of patients had received a cumulative dosage of prednisone-equivalent higher than 1000 mg. The total duration of treatment ranged from 4 days to 20 years (6 months or less: 69%). Non-specific gastro-intestinal symptoms were reported in 91% of these patients associated or not with pulmonary complaints. Low-grade fever was present in 54% of patients. Fifty-nine patients (39%) experienced severe bacterial or yeast infection during the course of severe strongyloidiasis. Peripheral eosinophilia was detected at presentation in 32% of patients. Strongyloidiasis was usually confirmed by repeated stool examinations. Thiabendazole was the treatment the more widely used. Eighty-nine patients (59%) deceased during the course of the disease. CONCLUSIONS: Severe strongyloidiasis is a risk in every corticosteroid-treated patient who has traveled to a soil-infested country, even if the contact was 30 years prior. This diagnosis should be suspected in patients who either experience unusual gastro-intestinal or pulmonary symptoms or suffer from unexplained Gram-negative bacilli sepsis.

Adrenal Cortex Hormones↗
PubMed2006

Visual loss mimicking a treated temporal arteritis relapse.

We describe a patient who, during tapering of corticosteroids prescribed for temporal arteritis (TA), developed nocardiosis that was mimicking a specific relapse. An isolated choroidal ischemia was thought to be a symptom of TA. Steroid therapy was intensified and, while on aggressive steroid therapy, the patient died of septic shock. Autopsy revealed occult disseminated nocardiosis with septic arteritis and apparently cured TA. Visual loss occurring during TA treatment should lead clinicians to consider a specific relapse as well as an opportunistic infection like nocardiosis.

Journal Article↗
PubMed2006

[Classification criteria of scleroderma].

Classification criteria of scleroderma aim to delineate the different clinical forms of the disease, which are associated with different prognoses. The basic classification between localized and systemic scleroderma remains pertinent. Among the former, the problems are essentially esthetic or sometimes functional; but the second may yield many other problems which are pertinent to function but also to life, owing to the involvement of body organs, including lungs, heart, digestive tract, kidneys and locomotor system. Overall, the quality of life of scleroderma patients is often poor. The patients are subclassified according to the skin involvement. Those patients with systemic scleroderma involving the trunk are classified as "Cutaneous diffuse systemic scleroderma"; the association of Raynaud's phenomenon, capillaroscopic abnormalities and specific autoantibodies defines "limited systemic scleroderma"; among the latter patients, those with distal skin involvement are classified "Cutaneous limited systemic sclerosis". The old term "CREST" tends to be abandoned due to its lack of specificity.

Biomarkers↗
PubMed2005

Analysis of immune reconstitution after autologous bone marrow transplantation in systemic sclerosis.

OBJECTIVE: To analyze hematopoietic and immune reconstitution after autologous hematopoietic stem cell transplantation (HSCT) in 7 patients with systemic sclerosis (SSc). METHODS: Two groups of patients were retrospectively constituted according to whether they had a favorable clinical response (group A; n = 4) or no response or a relapse of disease (group B; n = 3) after HSCT. Immune reconstitution was analyzed every 3 months using lymphocyte immunophenotyping, alpha/beta T cell receptor (TCR) diversity analysis, and ex vivo thymic function analysis by quantification of TCR rearrangement excision circles (TRECs). RESULTS: Patients had similar characteristics at study entry, except for a lower modified Rodnan skin thickness score (P = 0.03) and a lower Health Assessment Questionnaire score (P = 0.05) in group A than in group B. The number of reinjected cells and the time to hematopoietic reconstitution were similar in both groups. The absolute numbers of CD19+ and CD20+ B cells were lower in group A than in normal controls (P < 0.05) and within the normal range in group B. Absolute numbers of T and natural killer lymphocytes were normal before HSCT. Numbers of CD3+ cells remained low thereafter. Numbers of CD8+ cells were back to normal 3 months after HSCT in both groups. B cell counts were low until 6 months after HSCT in group A and stayed in the normal range in group B. The CD3+ defect was sustained in group A, with an opposite trend and a faster CD4+ reconstitution profile in group B. The T cell repertoire was skewed before and until 1 year after HSCT, with shared expansions before and after transplant in a given individual. TREC values correlated negatively with C-reactive protein levels (r(s) = -0.41, P = 0.001) and positively with CD19+ (r(s) = 0.35, P = 0.001) and CD20+ (r(s) = 0.34, P = 0.002) lymphocyte counts. CONCLUSION: B and T lymphocyte populations remained disturbed for at least 1 year after HSCT in SSc patients, which may reflect the persistence of an underlying disease mechanism.

Adolescent↗
PubMed2005

Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study.

OBJECTIVE: Screening allows for early management of pulmonary arterial hypertension (PAH), a severe complication of systemic sclerosis (SSc). Since no consensus has been reached on the method and criteria for optimal screening, we sought to develop an algorithm based on symptoms, Doppler echocardiography, and right heart catheterization (RHC) for application to a nationwide multicenter SSc population in France. METHODS: This prospective study was conducted from September 2002 to July 2003 by experts at 21 SSc centers. At each center, SSc patients without severe pulmonary function abnormalities underwent Doppler echocardiography by an experienced cardiologist. Patients with a peak velocity of tricuspid regurgitation (VTR) of >3 meters/second or 2.5-3 meters/second with unexplained dyspnea were asked to undergo RHC to confirm PAH according to international guidelines. RESULTS: Of the 599 patients analyzed, 29 had known PAH and 33 had suspected PAH, based on Doppler echocardiography, and underwent RHC. Of these 33, 18 were found to have PAH, 3 had left ventricular dysfunction, and 12 had no PAH. Newly diagnosed cases of PAH were of mild severity (mean +/- SD pulmonary artery pressure [mPAP] 30 +/- 9 mm Hg, mean +/- SD total pulmonary resistance [TPR] 524 +/- 382 dynes x second/cm(5)). Hemodynamic findings in patients with known PAH were mPAP 49 +/- 17 mm Hg and TPR 1,007 +/- 615 dynes x second/cm(5). The estimate of PAH prevalence was 7.85% (95% confidence interval 5.70-10.00). CONCLUSION: This screening algorithm, based on dyspnea, Doppler echocardiographic evaluation of VTR, and RHC, enabled early detection of PAH at a mild stage. Whether mild PAH will evolve to severe PAH in reported cases and whether this early diagnosis translates into improved prognosis for patients with mild PAH will be evaluated in the ongoing 3-year followup of this cohort.

Adult↗
PubMed2004

[Physiopathological data of Horton's syndrome].

GREAT PROGRESS: The understanding of the physiopathology of Horton's syndrome has been made in view of the prevalence of the disease, the access to affected tissue and new molecular biology techniques. And it is now possible to specify the intricacy between the genetic, immunological and vascular components. HORTON'S SYNDROME, A GENETIC DISEASE: The preferential association of the disease with some alleles pf the HLA DR4 group has helped to emphasize the fundamental role of a few amino acids of the second hypervariable area of the HLA-DR molecule. An immunogenetic predisposition appears favourable, or even necessary, for the development of the disease. HORTON'S SYNDROME, AN IMMUNOLOGICAL DISEASE: The temporal arteries of patients presenting with Horton's syndrome are infiltrated by polymorphous inflammatory cells, fundamentally including CD4 T-cell lymphocytes, macrophages, and a few giant cells. Some CD4 lymphocytes have undergone clonal proliferation and show signs of recent antigenic recognition. A fraction of them secrete interferon gamma, which plays a crucial role in the onset, maintenance and orientation of the immunological response. The macrophages play multiple roles notably in maintaining the inflammatory reaction, but also in the destruction of certain structures of the arterial wall. HORTON'S SYNDROME, A VASCULAR DISEASE: The destruction of the arterial wall at the acute stage of the disease appears responsible, sometimes later on, for aneurismal dilatations observed on the aorta of certain patients. Moreover, intimal hyperplasia (mediated by the PDGF (platelet derived growth factor) A and B) and thrombosis (related to the inflammation) join up in reducing the arterial flow and enhancing the risk of ischemic complications during the acute stage. PATHOGENESIS, HYPOTHESES: Various candidate-antigens have been incriminated in the onset of the inflammatory reaction during Horton's syndrome. Some epidemiological and molecular studies are in favour of an exogenous antigen, possibly infectious, but no evidence has been demonstrated in the studies published. A parietal, endogenous antigen might also be at the origin of the cascade of events described during this disease.

Aneurysm↗
PubMed2004

[Liver involvement revealing systemic mastocytosis: report of two cases].

Systemic mastocytosis is a disease defined by an abnormal infiltration of mast cells involving several extra-cutaneous organs. Hepatic involvement is frequent, however it rarely reveals the disease. We report two cases of systemic mastocytosis revealed by hepatic symptoms: liver failure in one case and jaundice in the second case. The diagnosis is often difficult. Mast cell tissular infiltration can be identified on paraffin sections by tryptase or CD117 (c-kit) immuno-staining.

Aged↗
PubMed2003

RNase L levels in peripheral blood mononuclear cells: 37-kilodalton/83-kilodalton isoform ratio is a potential test for chronic fatigue syndrome.

Chronic fatigue syndrome (CFS) is a disorder characterized by debilitating fatigue associated with immunological abnormalities. The etiology remains unclear. A low-molecular-mass (37 kDa) isoform of RNase L has been described in peripheral blood mononuclear cell (PBMC) extracts, and the ratio of two isoforms of RNase L (37 kDa/83 kDa) has been proposed as a potential biochemical marker of CFS. In a prospective case-control study, we tested whether the RNase L 37-kDa/83-kDa ratio could discriminate a SFC population. We compared the ratio of RNase L isoforms in PBMCs from 11 patients with CFS (6 women and 5 men; mean age +/- standard deviation, 43.2 +/- 13.8 years) and PBMCs from 14 healthy well-matched volunteers (10 women and 4 men; age, 39.1 +/- 11.6 years). A ratio of RNase L of 0.4 used as a threshold allowed diagnosis of CFS with high sensitivity (91%; 95% confidence interval [CI], 57 to 99%) and specificity (71%; 95% CI, 41 to 90%). The positive and negative prognostic values were 71% (95% CI, 41 to 90%) and 91% (95% CI, 57 to 99%), respectively. In the absence of acute infection or chronic inflammation, a high RNase L ratio could distinguish CFS patients from healthy volunteers. Additional large studies and follow-up studies are required to confirm the stability of this high ratio of RNase L isoforms in a CFS group.

Adult↗
PubMed2002

[Living with scleroderma].

To live with scleroderma is a very difficult challenge, owing to the various and sometimes painful or incapacitating consequences of the disease. The lengthy evolution, the uncertain prognosis and the functional handicap yield a complex and heavy burden on the patient and his family. Numerous means exist to counter the sequels of scleroderma, but the problem is to combine them correctly to obtain the best possible result in terms of comfort and quality of life. The various life aids, the patients association, and the non pharmaceutical treatments have an important role to play besides the intervention of the medical specialists of the organs involved by scleroderma.

Humans↗
PubMed2002

Autologous bone marrow transplantation in the treatment of refractory systemic sclerosis: early results from a French multicentre phase I-II study.

Haematopoietic stem cell transplantation (HSCT) has been proposed for refractory autoimmune diseases, including systemic sclerosis (SSc). A sequential Bayesian phase I-II clinical trial was conducted in SSc patients to assess the feasibility, the tolerance and the efficacy of autologous HSCT. Peripheral blood stem cells (PBSC) were collected using cyclophosphamide (4 g/m2) and recombinant human granulocyte colony-stimulating factor (5 micro g/kg/d) and reinfused after positive CD34+ selection. Conditioning used cyclophosphamide (200 mg/kg) or melphalan (140 mg/m2) according to cardiac function. The main end-point was the failure of the procedure, defined by failure of either PBSC mobilization, CD34+ selection or intensification procedure, or by procedure-related death. Among the 12 enrolled patients, three failures occurred: one PBSC mobilization, one CD34+ selection and one CD34+ intensification. Probability of graft failure was estimated at 0.286 (95% confidence interval: 0.095-0.54). Autologous PBSC (n = 10) or bone marrow (n = 1) transplantation was actually performed in 11 patients with one procedure-related death. Median time to neutrophil (> 0.5 x 10(9)/l) and platelet (> 25 x 10(9)/l) haematopoietic reconstitution was 12 and 10 d respectively. After 18 months (range 1-26), eight out of 11 patients have shown major or partial response. Non-myeloablative conditioning, followed by a T cell-depleted autologous PBSC or bone marrow transplantation, appears feasible with low toxicity in severe SSc with short-term clinical benefits.

Adolescent↗
PubMed2002

[Scleroderma and pregnancy].

PURPOSE: - The frequency of concurrent scleroderma and pregnancy is low because scleroderma is a rare connective-tissue disease and the mean age of symptom onset is in the early 40s. The reciprocal influence of pregnancy and scleroderma and management of pregnancy are the purpose of this review. Current knowledge and key points. - There is no increase in infertility in women with scleroderma. The effect of pregnancy on scleroderma is a greater risk of renal crisis, especially when scleroderma is evolutive, diffuse and recent. Conversely, pregnancy is characterized by prematurity and small full-term infants because scleroderma induce placentar vascular abnormalities. There is no increase in miscarriages and spontaneous abortions. The pregnant scleroderma patient is a potential anaesthetic challenge because of physical difficulties and visceral involvement. Future and projects. - Planning of pregnancy and follow-up by a multidisciplinary experienced team in high risk pregnancies are guarantees for successful pregnancies. Future research will try to determine the role of microchimerism in scleroderma physiopathology and specify placental findings in order to ameliorate the obstetrical prognosis.

Acute Kidney Injury↗