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Biomedical subjects

P Guilpain

Publications and source records attributed to P Guilpain.

11 recordsLinked to original sources
PubMed2006

Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension.

The aim of the present study was to investigate the presence of anti-fibroblast antibodies in patients with idiopathic or scleroderma-associated pulmonary arterial hypertension (PAH) and healthy controls. PAH was documented by right-heart catheterisation (mean pulmonary artery pressure at rest >25 mmHg). Serum immunoglobulin (Ig)G and IgM reactivities of patients with idiopathic PAH (n = 35), scleroderma-associated PAH (n = 10), diffuse (n = 10) or limited cutaneous (n = 10) scleroderma without PAH and age- and sex-matched healthy individuals (n = 65) were analysed by cell-based ELISA and immunoblotting on normal human fibroblasts. As assessed by ELISA, 14 out of 35 (40%) patients with idiopathic PAH and three out of 10 (30%) patients with scleroderma-associated PAH expressed anti-fibroblast IgG antibodies. IgG from all individuals bound to one major 40-kDa protein band. IgG from patients with idiopathic PAH bound to two 25- and 60-kDa bands with a higher intensity than IgG from other individuals. In conclusion, immunoglobulin G anti-fibroblast antibodies are present in the serum of patients with pulmonary arterial hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens.

Adult↗
PubMed2006

[Eosinophil granule cationic proteins: eosinophil activation markers].

INTRODUCTION: Eosinophils play a central role in the pathogenesis of allergic diseases and host protection against parasites, especially against helminths. EXEGESIS: Eosinophil granules contain cytotoxic proteins, including cationic proteins: major basic protein (MBP), eosinophil cationic protein (ECP), eosinophil peroxidase (EPO) and eosinophil derived neurotoxin (EDN). CONCLUSION: Eosinophil granule proteins are implicated in the occurrence of allergic diseases and Churg-Strauss syndrome. These proteins are activation markers of eosinophils and may be useful in clinical practice.

Biomarkers↗
PubMed2005

[Pathogenesis of primary systemic vasculitides (I): ANCA-positive vasculitides].

The pathogenesis of different types of systemic vasculitis positive for antineutrophil cytoplasmic antibodies (ANCA) remains incompletely understood. ANCA constitute a heterogeneous group of antibodies that are associated with different types of small-vessel vasculitis, including Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and Churg-Strauss syndrome (CSS). Anti-proteinase 3 ANCA are present in more than 90% of patients with systemic WG, and anti-myeloperoxidase (MPO) ANCA in 50-75% of those with MPA and 40-60 % of those with CSS. The pathogenic role of ANCA has been well documented in vivo: passive transfer of anti-MPO ANCA in an MPO knockout mouse model immunized with MPO is sufficient to induce the disease. In vitro, mouse and human anti-proteinase 3 ANCA can activate neutrophils primed with TNF-a and contribute to vasculitic lesions. T-cells are also involved: type 1 helper cytokines have been detected in tissue lesions of limited forms of WG, while type 2 helper cytokines have been identified in its systemic forms. Eosinophils may play a key role in the development of vasculitic lesions in CSS, although this remains to be proved.

Adoptive Transfer↗
PubMed2005

[Pathogenesis of primary systemic vasculitides (II): ANCA-negative vasculitides].

The pathogenesis of different types of systemic vasculitis negative for antineutrophil cytoplasm antibodies (ANCA) and involving small or medium-sized vessels is not very well documented. During polyarteritis nodosa (PAN), which is related to hepatitis B virus (HBV) infection, as well as during cryoglobulinemic vasculitides, associated with hepatitis C virus (HCV), and probably during Henoch Schönlein purpura, histological lesions may result from the deposition of immune complexes formed from viral antigens and from antibodies responsible for the activation of the classic complement pathway and for recruitment of polymorphonuclear neutrophils. Two other mechanisms are discussed for other types of ANCA-negative systemic vasculitis: immune complex deposition and sheer stress at arterial bifurcation points. A bacterial superantigen is suspected in Kawasaki disease but remains unproved.

Animals↗
PubMed2005

Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension.

BACKGROUND: It has previously been shown that IgG antibodies from patients with limited cutaneous systemic sclerosis (SSc) bind to specific microvascular endothelial cell antigens. Since patients with limited cutaneous SSc are prone to develop pulmonary arterial hypertension (PAH), and since endothelial cell activation is involved in the pathogenesis of idiopathic PAH (IPAH), a study was undertaken to examine the presence of anti-endothelial cell antibodies in patients with idiopathic or SSc associated PAH. METHODS: PAH was confirmed by right heart catheterisation (mean pulmonary artery pressure at rest >25 mm Hg). Serum IgG and IgM reactivities were analysed by immunoblotting on human macrovascular and microvascular lung and dermal endothelial cells from patients with IPAH (n = 35), patients with PAH associated with SSc (n = 10), patients with diffuse (n = 10) or limited cutaneous (n = 10) SSc without PAH, and 65 age and sex matched healthy individuals. RESULTS: IgG antibodies from patients with IPAH bound to a 36 kDa band in macrovascular endothelial cell extracts with a higher intensity than IgG from other patient groups and controls. IgG antibodies from patients with IPAH bound more strongly to a 58 kDa band in microvascular dermal endothelial cells and to a 53 kDa band in microvascular lung endothelial cells than IgG antibodies from other patients and controls. IgG antibodies from patients with limited cutaneous SSc with or without PAH, but not from other groups or from healthy controls, bound to two major bands (75 kDa and 85 kDa) in microvascular endothelial cells. CONCLUSION: IgG antibodies from patients with idiopathic or SSc associated PAH express distinct reactivity profiles with macrovascular and microvascular endothelial cell antigens.

Adult↗
PubMed2004

[Bilateral optic neuropathy revealing systemic sarcoidosis].

INTRODUCTION: Although ocular involvement concerns about 25 to 60% of patients with systemic sarcoidosis, papilledema is a rare event. EXEGESIS: A 43-year-old man presented with left visual loss related to papillitis. Despite high-dose corticosteroids, visual loss became rapidly bilateral. Thoracic CT-scan showed mediastinal and parenchymal involvement. Histological study of lung, bronchial and lymphadenopathy biopsies displayed giant-cells granuloma without necrosis. Diagnosis of sarcoidosis complicated with optic neuropathy was made. Therapy combining prednisone, methotrexate and infliximab was started without significant improvement of visual acuity. CONCLUSION: This report underlines the potential severity of optic neuropathy in sarcoidosis.

Adult↗
PubMed2003

[Compression of the spinal cord revealing a seronegative rheumatoid arthritis].

INTRODUCTION: Craniocervical junction damages may result in a compression of the spinal cord. They may be caused by infectious, tumoral or inflammatory processes. Rheumatoid arthritis is probably among rheumatic diseases the most frequent cause of atlantoaxial arthritis. Nevertheless involvement of the craniocervical junction as the presenting symptom of rheumatoid arthritis is a very rare feature. EXEGESIS: We report the case of a 61 years old woman who presented with atlantoaxial involvement and spinal cord compression one year before the diagnosis of a seronegative rheumatoid arthritis. CONCLUSION: Symptomatic craniocervical junction damages may appear. Patients with damages of the craniocervical junction and negative investigations should be followed long-term; an underlying inflammatory disease may become evident after significant delay.

Arthritis, Rheumatoid↗
PubMed2002

Churg-Strauss syndrome in two patients receiving montelukast.

OBJECTIVE: Churg-Strauss syndrome (CSS) has been described in association with the treatment of asthmatic patients with leukotriene receptor antagonist. The main mechanism proposed to explain this condition is the unmasking of CSS after the leukotriene receptor antagonist has allowed corticosteroid tapering. Other hypotheses might be proposed. METHODS: We describe two patients who developed CSS after starting treatment with montelukast, a new antileukotriene drug. RESULTS: Both patients presented with CSS after 4-5 months of treatment with montelukast. Neither patient received long-term systemic steroids for asthma, but both were on inhaled steroids. One patient had a myocardial involvement and experienced a stroke. Our two patients were treated with systemic steroids and cyclophosphamide. CONCLUSIONS: CSS does not appear to relate to steroid tapering in our patients. The other hypotheses are a coincidence or a direct adverse effect of the antileukotriene. Long-term data on these drugs are lacking and leukotriene's role in vasculitis remains to be elucidated.

Acetates↗
PubMed2001

[An index based on the age and free thyroxine rate predicts the likelihood of Graves' disease in hyperthyroidism].

PURPOSE: Graves' disease patients are generally younger and have more severe symptoms than other thyrotoxic patients. We established an index based on the normalized free thyroxine rate and age, capable of predicting Graves' disease in thyrotoxic patients. METHODS: The predictive index was established from a discriminant analysis from a retrospective population of 114 thyrotoxic patients and its predictivity was confirmed by cross-validation on the same population. RESULTS: The index IGD = 41.38 - age + 37.05 x in (normalized free T4) classifies accurately 80.7% of the patients (CI 95%: 72.2-87.5). Sensitivity is 78.5% (CI 95%: 66.5-87.7). Specificity is 83.7% (CI 95%: 70.3-92.7). Predictive positive value is 86.4% (CI 95%: 75.0-94.0). Negative predictive value is 74.5% (CI 95%: 61.0-85.3). Likelihood ratio of Graves' disease in case of prediction by the index is 4.81 (CI 95%: 2.66-9.32). Likelihood ratio of Graves' disease in case of non-prediction by the index is 0.26 (IC 95%: 0.16-0.40). CONCLUSION: A simple index based on the normalized free thyroxine rate and age provides an early diagnosis orientation toward Graves' disease in thyrotoxic patients who are waiting for complementary investigations.

Adult↗