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Biomedical subjects

M De Bandt

Publications and source records attributed to M De Bandt.

At least 37 records · Page 2Linked to original sources

Antibodies to proteinase-3 mediate expression of intercellular adhesion molecule-1 (ICAM-1, CD 54).

We investigated the role of intercellular adhesion molecule-1 (ICAM-1) in the adhesion of polymorphonuclear neutrophils (PMN) to classic antineutrophil cytoplasmic antibody (C-ANCA)-treated endothelial cells, independently of cytokines. Human umbilical vein endothelial cells (HUVEC) grown to confluence in cytokine-free conditions were stimulated with C-ANCA sera and affinity-purified anti-proteinase 3 antibodies (PR3) from Wegener's granulomatosis (WG) patients. Non-activated PMN were added to treated HUVEC and adhesion was measured. In parallel experiments, treated HUVEC were fixed and ICAM-1 and E-selectin were assayed by cyto-ELISA; in other experiments anti-ELAM-1 and anti-ICAM-1 antibodies were assessed. In this in vitro model, adhesion of non-activated PMN to anti-PR3-stimulated HUVEC was enhanced. Adhesion was greater with anti-PR3 antibodies than with control and normal immunoglobulins, and correlated with the level of anti-PR3 antibodies. Neutralization of anti-PR3 antibodies by neutrophil azurophilic granule proteins abolished adhesion. This adhesion increased at the fourth hour after simulation, peaked at the twelfth hour and then decreased. This phenomenon occurred mainly through endothelial expression of ICAM-1 (the main counter-receptor for integrins, involved in firm PMN adhesion and migration) and E-selectin on HUVEC membranes. Anti-adhesion molecule antibodies inhibited this adhesion. This work supports the hypothesis of a direct effect of C-ANCA in endothelial stimulation, namely, on endothelium-PMN adhesion, and strengthens the major role of ICAM-1, directly involved in firm sticking of PMN to HUVEC, besides E-selectin. C-ANCA upregulate endothelial adhesiveness and thus participate in inflammatory reactions by providing endothelial adhesive structures for neutrophils. This might be one of the first steps leading to clinical expression of the disease. These results provide new insights into the pathogenesis of C-ANCA-related diseases.

Antibodies↗

Serum sickness after wasp venom immunotherapy: clinical and biological study.

We describe a case of a man who developed serum sickness during wasp venom immunotherapy. Remarkable features were unusually severe neurological symptoms, multiple relapses in the absence of rechallenge, parallel course between clinical symptoms, serum levels of specific reagins and their antibodies, and a dramatic response to plasma exchange therapy. Desensitization is widely used and can cause a wide range of adverse effects; however, systemic vasculitis is a very rare complication and we are not aware of any case similar to ours, with serum sickness after injection of highly purified hymenoptera antigen. Clinicians should be aware of such a possibility.

Aged↗

[ANCA (antineutrophil cytoplasmic antibodies). Contribution of ANCA to the understanding of pathogenic mechanisms of systemic vasculitis].

ANCA (antineutrophil cytoplasmic antibodies) are a growing class of interest; these antibodies target lysosomial enzymes from polymorphonuclear cells. They represent diagnosis tools and prognosis markers in systemic vasculitis (Wegener's granulomatosis and microscopic polyangiitis). They are also detected in other chronic inflammatory diseases as lupus and rheumatoid arthritis. Their description, ten years ago, led to a huge amount of clinical and experimental work. The role of ANCA as pathogenic auto-antibodies in the disease is supported in numerous studies giving new insights into the pathogenesis of systemic vasculitis.

Antibodies, Antineutrophil Cytoplasmic↗

Angiotropic large cell lymphoma with mononeuritis multiplex mimicking systemic vasculitis.

Angiotropic large cell lymphoma (ALCL), the so-called malignant angioendotheliomatosis, is characterised by proliferation of tumorous cells within small vessels. Manifestations in the CNS and cutaneous lesions prevail in the clinical presentation, although any organ can be involved. The recent classification of this lymphoma as part of the large cell lymphomas has modified the therapeutic approaches employed. This should improve the prognosis of this usually fatal disease. An unusual case presenting with fever, mononeuritis multiplex, and cutaneous lesions is reported. Peripheral neuropathy without other neurological symptoms is uncommon, and, to our knowledge, such isolated mononeuritis multiplex with nerve lesions has not been previously reported in ALCL. The clinical diagnosis was a systemic necrotising vasculitis and it is considered that its differential diagnosis must include angiotropic large cell lymphoma.

Aged↗

Familial multiple myeloma. Report of a case in a father and daughter. Review of the literature.

We report a case of familial multiple myeloma in a father and daughter. The same lambda chain was detectable in both patients. Symptom onset occurred three years earlier in the daughter than in the father. We found 52 previously published cases of familial myeloma, of which only 13 occurred in a parent and child. Given the incidence of myeloma in the general population, the occurrence of several cases in the same family is probably not due to chance. The few available data on karyotypes and oncogenes are discordant. The respective role of genetic factors and environmental factors is discussed.

Animals↗

Ossification of the posterior longitudinal ligament, diffuse, idiopathic skeletal hyperostosis, abnormal retinol and retinol binding protein: a familial observation.

We describe a 52-year-old man who presented with diffuse idiopathic skeletal hyperostosis, ossification of the posterior longitudinal ligament, and abnormal levels of retinol and retinol binding protein (RBP). The molar retinol/retinol binding protein ratio was high, suggesting congenital functional RBP deficiency. His two sons, aged 23 and 27 years, shared the same biological abnormality without clinical symptoms. To our knowledge, this is the first case report of such a familial association.

Humans↗

Interleukin 6 secretion by monocytes and alveolar macrophages in systemic sclerosis with lung involvement.

Interleukin-6 (IL-6) has been involved in the pathogenesis of inflammatory and/or autoimmune diseases. We characterized the production of IL-6 by blood monocytes and alveolar macrophages (AM) in 11 patients with definite systemic sclerosis (SSc) with lung involvement and in eight normal control subjects. IL-6 levels were determined in serum, bronchoalveolar lavage (BAL) supernatants, monocytes, and AM cell culture supernatants using an ELISA kit. BAL cell analysis evidenced an alveolitis with hypercellularity and increased neutrophils and mast cells absolute counts. Serum and BAL IL-6 levels were low and similar in SSc and control groups. The monocytes of the group with SSc secreted more IL-6 than did the control group, both spontaneously (p = 0.01) and after LPS stimulation (p = 0.0007). Spontaneous secretion of IL-6 by AM tended to be higher in the SSc group than in the control group (p = 0.22). LPS-induced IL-6 secretion by AM was similar in both groups. Our study demonstrates that during SSc lung disease, spontaneous and stimulated IL-6 secretion by blood monocytes is increased, compared with secretion by healthy control subjects. By contrast, IL-6 secretion by AM is normal despite evidence of mild alveolitis.

Bronchoalveolar Lavage Fluid↗

[Salazosulfapyridine-induced eruption. Pustular erythroderma and pseudo-lymphomatous syndrome: 2 cases].

We report two observations of drug eruption due to salazosulfapyridine which occurred 15 to 21 days after introducing the molecule. The two patients were of Carabian origin and presented with fever, erythrodermia which became pustular and a pseudolymphomatous syndrome. Favourable outcome was observed 4 to 6 weeks later. The diagnosis of toxidermia was made based on the strong imputability of salazosulfapyridine in both cases. Severe immunoallergic reactions to this molecule have been described, including fatal cytolytic hepatitis and Lyell syndrome.

Adult↗

[Treatment of acute systemic lupus erythematosus with intravenous infusions of cyclophosphamide. Value and limitations].

Monthly intravenous cyclophosphamide (IVCY) has been a recommended therapy for severe lupus nephritis or neurological flare-ups in lupus patients. But the optimal treatment regimen and duration remains unknown. We report our experience in an open study of 37 patients treated with monthly IVCY. Thirty-four women and 3 men, mean age 35.5 with a mean disease duration of 59 months, with a mean 5.7 ACR criteria for SLE were analysed. 27 (group I) had lupus nephritis (OMS Class III or IV) and 10 had neurological involvement (group II). In group I, after six months of IVCY, a significant improvement was noticed in the UCH-Middlesex clinical index (2.9 pts vs 7.8), the proteinuria (3.12 g/d vs 5.4), complement and split fractions (CH50 98.4 vs 48.9%; C3 877 vs 600 mg/l; C4 177 vs 128 mg/l), the level of anti-DNA antibodies (67.5 vs 775 UI/ml) and the daily dose of steroids (22 vs 44 mg/d). Kidney biopsies showed a reduction of the activity index despite a slight increase of the chronicity index (4.1 vs 6.3 pts and 5.5 vs 3.6 pts). Those results were not maintained at medium and long term. Moreover five patients presented with worsening of renal function during IVCY treatment and two patients relapsed after the end of the treatment. In group II significant improvement was noticed at six months concerning the clinical index (1.77 pts vs 7.17) and the daily dose of steroids, 3 patients died because of cerebral vasculitis refractory to IVCY. Adverse effects are frequent: infectious (25 among 20 patients), hemorrhagic cystitis (2 events in 1 patient), gastrointestinal side effects were common (12/37 patients). Were also noticed: neutropenia (5/37), transient amenorrhea (4/28), drug induced menopausis (2/28). Overall mortality is important (7/37), uneffectiveness of IVCY was noticed in 5 patients, flares occurred in 8 patient during or after stopping treatment. IVCY seems efficacious if given at the very beginning of the flare. Its usefulness is obvious at six months among clinical and biological data in patients with severe lupus nephritis or neurological flare. It seems that long term outcome on the renal function is not modified.

Acute Disease↗

Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases.

OBJECTIVE: To determine the circumstances, the clinical features and the outcome of Pneumocystis carinii pneumonia (PCP) in human immunodeficiency virus (HIV)-free patients with connective tissue diseases (CTD). METHODS: Retrospective analysis of all cases referred 10 medical units in the last 10 years. RESULTS: A total of 34 cases of PCP in patients with CTD were studied (Wegener's granulomatosis, n = 12; systemic lupus erythematosus, n = 6; polyarteritis nodosa, n = 4; poly/dermatomyositis, n = 5; others, n = 7). The majority of patients (25/34 patients; 74%) presented PCP during the first 8 months following the diagnosis of CTD. At the time of diagnosis of PCP, most patients (32/34; 94%) were receiving corticosteroids (mean prednisone equivalent dose: 1.2 mg/kg/day) associated in 24 cases with cytotoxic agents (cyclophosphamide, n = 19; methotrexate, n = 5). Most patients were lymphocytopenic at the onset of PCP: 91% (31/34) of patients had fewer than 1.5 x 10(9)/l circulating lymphocytes and 76% (26/34) had fewer than 0.8 x 10(9)/l. The mean duration of prodromal symptoms was 6 days: this is much shorter than for AIDS associated PCP. Half the patients required intensive care for respiratory failure. Mortality was high (11/34 patients; 32%) although deaths were partly due to infections acquired in intensive care units. Among the 23 survivors, 10 (43%) received secondary prophylaxis for PCP and 13 (57%), received the usual therapeutic regimen. No relapse has been observed in either group with a mean followup of 22 months. CONCLUSION: Although rare, PCP must be considered in patients with any type of CTD and receiving cytotoxic agents and corticosteroids, particularly if they are lymphocytopenic. Thus, bronchoalveolar lavage must be rapidly performed in patients with CTD presenting with fever, pulmonary infiltrates, hypoxemia and lymphopenia.

Adult↗

[Failure of intravenous immunoglobulins in certain systemic diseases. 5 cases].

Human polyvalent immunoglobulins administered intravenously have shown to be effective in some immune diseases. We administered high dose-IV Ig (2g/kg/session) in five patients with severe chronic systemic diseases. Their condition did not improve, except in one case where a transient response was noticed. Considering the inconsistent results and high cost of IV Ig, double blind studies are required to determine the conditions in which high dose IV Ig may be more effective than conventional treatments.

Adult↗

[Still disease in the adult and in pregnancy].

The interactions between pregnancy and adult-onset Still's disease are unclear. Nine pregnancies which occurred from 1983 through 1991 in seven women (mean age 27.7 years; range 23-40) were studied retrospectively. Five patients had one pregnancy and two had two pregnancies. Onset of the joint disease occurred before or during pregnancy. The joint disease occurred at the 5th or 6th month of the first pregnancy in two patients; one of these patients had a second pregnancy which was not associated with a flare. In five patients, onset of the joint disease occurred 4, 15, 42, 44 and 58 months before the first pregnancy, respectively; any effects of the joint disease on the pregnancy were extremely variable. 6 infants were full-term and two were premature (35 weeks); the remaining pregnancy was electively terminated; Apgar scores were normal in all eight infants, who were all male. Adult-onset Still's disease did not influence the outcome of the pregnancy in our patients; no clear-cut effects of pregnancy on adult-onset Still's disease were seen.

Adult↗

Hypocomplementemic urticarial vasculitis syndrome, Jaccoud's syndrome, valvulopathy: a new syndromic combination.

We describe 3 cases of hypocomplementemic urticarial vasculitis syndrome (HUVS) with Jaccoud's hands deformity and cardiac valve disease (aortic regurgitation, mitral regurgitation, mitral disease). In one case, the valve lesions required valve replacement and later a heart transplant. Valve disease and articular deformities developed 2 and 4 years, respectively, after the onset of HUVS. This as yet undescribed combination of diseases suggests a new syndrome. Pathogenesis of periarticular and cardiac lesions is unknown. The role of Clq and anti-Clq antibody is discussed.

Adult↗

[3 cases of polyarthritis treated with recombinant alfa interferon].

Bilateral symmetrical polyarthritis occurred in three patients (2 males and 1 female), with no previous history of inflammatory rheumatologic disease, given alpha-interferon for 1 1/2, 7, and 10 months as treatment of chronic non A-non B hepatitis, myelofibrosis, and thrombocytopenia with myeloproliferative disorder, respectively. Joint manifestations developed 1 1/2, 3, and 10 months after initiation of alpha-interferon in a dosage of 3.10(6) U three times a week, 4.5.10(6) U per day, and 8.10(6) U three times a week. Polyarthritis persisted following withdrawal of alpha-interferon in the two last patients of whom one had rheumatoid nodules and positive rheumatoid serology and the other had scleritis, exanthema, and negative rheumatoid serology. Erosive rheumatoid arthritis was diagnosed after 28 months and 12 months, respectively, in two patients who required systemic corticosteroids with antimalarials (1 case) or azathioprine after failure of methotrexate (one case). Follow-up in the third case (12 months) is too short to allow differentiation of systemic lupus erythematosus (ANA: 1/1500 H with anti-DNA antibodies 58 U/ml) and chronic autoimmune hepatitis. Reports of chronic inflammatory rheumatologic disease during alpha interferon therapy are exceedingly few in number. In the cases reported herein, alpha-interferon may have either triggered or revealed the joint disease. To prevent occurrence of this complication, exclusion from alpha-interferon therapy of patients with autoantibodies or a positive history for clinical evidence of immune dysfunction may be considered.

Adult↗