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N Sepp

Publications and source records attributed to N Sepp.

At least 19 recordsLinked to original sources
PubMed2003

Linear scleroderma "en coup de sabre" coexisting with plaque-morphea: neuroradiological manifestation and response to corticosteroids.

A 24 year old woman in the 33rd week of pregnancy developed progressive neurological complications with right sided hemiparesis in association with the occurrence of linear scleroderma "en coup de sabre" (LSCS) and pre-existing plaque-morphea, already being treated by balneophototherapy. Further progression of neurological symptoms led to a caesarean section with the delivery of a healthy child. Brain magnetic resonance imaging (MRI) showed focal T2 signal increases in the left frontoparietal region directly adjacent to the area of LSCS. Cerebrospinal fluid analysis revealed oligoclonal bands, suggesting an intracerebral inflammatory process. Subsequent pulsed corticosteroid treatment led to a remission of neurological symptoms and to a marked resolution of the MRI lesions.

Adult↗
PubMed2002

Extravascular necrotizing palisaded granulomas as the presenting skin sign of systemic lupus erythematosus.

We recently examined a patient who presented clinically with tender, erythematous papules associated with a febrile illness and polyarthralgias. Histopathological examination revealed extravascular palisaded neutrophilic granulomas, a skin reaction pattern originally described in Churg-Strauss disease but subsequently observed in a variety of other systemic disorders characterized by immune complex generation. A diagnosis of systemic lupus erythematosus (SLE) was established. To our knowledge, this is the first report of extravascular necrotizing palisaded granulomas as the presenting skin sign of SLE.

Adult↗
PubMed2002

Aseptic meningo-encephalitis related to dexibuprofen use in a patient with systemic lupus erythematosus: a case report with MR findings.

We report of a 22-year-old woman with systemic lupus erythematosus (SLE) who was admitted to the intensive care unit (ICU) because of obtundation and a febrile illness. These symptoms had occurred after ingestion of 16 tablets of dexibuprofen. Cerebral magnetic resonance imaging (MRI) disclosed multiple hyperintense white matter abnormalities without gadolinium enhancement. No infectious origin or signs of a lupus flare were found. Our report is the first description of MR findings in dexibuprofen-induced aseptic meningitis, which here is actually a case of meningo-encephalitis. Patients suffering from SLE show increased susceptibility to non steroidal anti-inflammatory drug (NSAID)-induced aseptic meningitis/encephalitis, an important differential diagnosis to be considered at the work-up of altered mental status.

Adult↗
PubMed2001

Lymphocytic infiltration of the skin is a photosensitive variant of lupus erythematosus: evidence by phototesting.

BACKGROUND: Lymphocytic infiltration of the skin (LIS) is a disorder in which photosensitivity has been suspected but never proven. OBJECTIVES: To carry out a systematic photobiological investigation in patients with LIS and to compare the photobiological features of LIS with those of other photosensitive disorders. METHODS: We performed provocative phototesting with ultraviolet (UV) A and UVB in 10 patients with active LIS. RESULTS: In all patients, UVA and/or UVB elicited abnormal papular phototest reactions resembling lesions of LIS both clinically and histologically. Lesions typically developed 3--6 days (mean +/- SD 100.8 +/- 20.9 h) after the first UV exposure. CONCLUSIONS: This characteristic latency interval together with certain clinical features, i.e. onset in summer, predilection for the face and persistence of the lesions, indicate that LIS is a photosensitive disorder distinct from polymorphic light eruption but indistinct from lupus erythematosus (LE). Both our photobiological findings and the effective treatment with hydroxychloroquine in half of our patients strengthen the proposal that the two entities LIS and LE tumidus are identical. As diagnosis cannot be made by histological, immunofluorescence or laboratory criteria, provocative phototesting may be a diagnostic aid in this disorder.

Adult↗
PubMed2001

Interaction of HSV-1 infected peripheral blood mononuclear cells with cultured dermal microvascular endothelial cells: a potential model for the pathogenesis of HSV-1 induced erythema multiforme.

The effect of herpes virus infection on human dermal microvascular endothelial cells and herpes-virus-1-infected peripheral blood mononuclear cells on human dermal microvascular endothelial cells was studied as a model of herpes-associated erythema multiforme. After infection of human dermal microvascular endothelial cells with native herpes virus and overnight culture, 60%--90% of human dermal microvascular endothelial cells showed cytopathic effects. HLA class I molecules and CD31 (PECAM-1) surface expression in herpes-virus-infected endothelial cells were substantially downregulated, whereas CD54 (ICAM-1) remained unchanged. Cocultivation with herpes-virus-1-infected peripheral blood mononuclear cells left characteristic plaques on the human dermal microvascular endothelial cell monolayer; however, very few human dermal microvascular endothelial cells (1%--3%) were infected. Adhesion molecule expression of human dermal microvascular endothelial cells cocultivated with herpes-virus-infected peripheral blood mononuclear cells demonstrated a 5-fold increase in CD54 expression, a 2-fold increase in HLA class I expression, but no change of CD31 by fluorescence-activated cell sorter analysis. Incubation of human dermal microvascular endothelial cells with ultraviolet-C irradiated herpes-virus-infected peripheral blood mononuclear cells had no effect on morphology or adhesion molecule expression levels. Changes of adhesion molecule expression by direct infection or cocultivation with peripheral blood mononuclear cells (with native and ultraviolet-C inactivated herpes virus infection) were also documented at the mRNA level. Adhesion assays demonstrated an increased binding of herpes-virus-infected peripheral blood mononuclear cells versus noninfected peripheral blood mononuclear cells to noninfected human dermal microvascular endothelial cells. Our results suggest that incubation of herpes-virus-infected peripheral blood mononuclear cells with human dermal microvascular endothelial cells induces significant upregulation of CD54 and major histocompatibility complex class I molecules in the surrounding noninfected human dermal microvascular endothelial cells, which is associated with an increased binding of peripheral blood mononuclear cells. Our in vitro findings may serve as a model for herpes-associated erythema multiforme possibly explaining the dermal inflammatory reaction seen in that condition.

Antigens, Surface↗
PubMed2001

Detection of myocardial involvement in systemic lupus erythematosus: mismatch between normal perfusion scans with 201Thallium and pathological 18FDG uptake.

BACKGROUND: Systemic lupus erythematosus (SLE) patients can frequently present cardiac symptoms, however its etiology is not well known. METHODS EXPERIMENTAL DESIGN: prospective study. SETTINGS: specialized out-patient unit for SLE patients at an university hospital. PATIENTS: 15 SLE patients (13 females, 2 males; age range 18-64 years). INTERVENTIONS: metabolic studies of the heart were done using 18F-deoxy-glucose (18FDG, 296-333 MBq on a 2-head hybrid system) as well as heart perfusion studies (111MBq 201Tl). Additional studies: resting ECG, echocardiography, stress ECG, immunological activity parameters, antibody analyses (ANA, ENA, anti-cardiolipin antibodies), CPK, troponin-T, and lipid profiles. MEASURES: degree of correlation between conventional diagnostics and the imaging techniques. RESULTS: Abnormal ECG in 10 cases, pericardial involvement in 11 cases, elevated CPK in 1 case. ANTIBODY PROFILES: anti-cardiolipin in 10/15, ENA in 9/15, ANA in 14/15. None of these changes were associated with parameters of immune activation. In the majority of cases (10/15) the 18FDG scan showed a speckled, inhomogeneous pattern of distribution, which contrasted sharply with a normal 201Tl scan. A similar pattern was observed in the patients with ocular mitochondrial myopathy, the anti-phospholipid syndrome as well as in dermatomyositis. CONCLUSIONS: Our preliminary results suggest that SLE patients with cardiac symptoms may have an abnormal glucose metabolism of the myocardium as shown by a pathological 18FDG scan, whereas perfusion appears to be normal (reversed mismatch). The lack of correlation with acute elevation of cardiac enzymes or with ECG changes suggest a chronic process.

Adult↗
PubMed2000

Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95.

OBJECTIVE: Apoptosis of endothelial cells is a key event in the pathogenesis of systemic sclerosis (SSc). The aim of the present study was to analyze in vitro the mechanism causing endothelial cell apoptosis in SSc. METHODS: Human dermal microvascular endothelial cells (HDMEC) or human umbilical vein endothelial cells (HUVEC) were cultured with native or heat-inactivated serum from SSc patients or controls with or without interleukin-2-activated natural killer (NK) cells or peripheral blood mononuclear cells. SSc and control sera were tested for the presence or absence, respectively, of anti-endothelial cell antibodies (AECA) by indirect immunofluorescence. Apoptosis was detected by the TUNEL technique. RESULTS: Native sera alone had no effect. Apoptosis induction was observed on HDMEC, but not on HUVEC, in the presence of AECA-positive SSc sera and activated NK cells, and could be inhibited by an anti-Fas ligand antibody. Inhibition of the perforin/granzyme pathway with concanamycin A had no effect on apoptosis induction in this in vitro model. Immunofluorescence analysis of cryosections from SSc skin showed Fas (CD95) expression by endothelial cells, supporting the in vitro findings. CONCLUSION: The results suggest that endothelial cell apoptosis in SSc is induced by antibody-dependent cell-mediated cytotoxicity via the Fas pathway. These data not only provide insight into the pathogenesis of SSc, but also may open new ways to rational therapy for this disease.

Adult↗
PubMed2000

Enhanced tryptophan degradation in systemic lupus erythematosus.

In vitro and in vivo, tryptophan degradation was found to be associated with T cell functional loss and tolerance induction. In systemic lupus erythematosus (SLE) besides the Th2-type cytokine interleukin-10, Th1-type cytokines including interferon-gamma (IFN-gamma) are expressed especially during exacerbation of the disease. IFN-gamma stimulates the enzyme indoleamine (2,3)-dioxygenase (IDO) converting tryptophan to the metabolite kynurenine which in macrophages is subsequently degraded to other, partly neurotoxic compounds like quinolinic acid, and finally to nicrotinamides. We measured kynurenine and tryptophan concentrations in the sera of 55 SLE patients. In these patients, the concentrations of tryptophan (median, interquartile range: 53.9, 45.7-64.1 microM) were lower (p < 0.0001), and the kynurenine concentrations (2.45, 1.75-3.40 microM) were increased (p < 0.0005) compared to healthy blood donors (70.0, 63.8-80.6; 1.80, 1.45-2.27 microM, respectively). Also the kynurenine per tryptophan quotients (K/T), which allow to estimate IDO activity, were significantly higher in patients than in normals (0.043, 0.033-0.062 vs. 0.027, 0.021-0.030; p < 0.0001), indicating enhanced IDO-induced tryptophan degradation in SLE. There was no significant relationship between tryptophan, kynurenine and the SLEDAI, and also the correlation of K/T with SLEDAI was rather weak (rs = 0.243, p < 0.05). Higher K/T was found in patients presenting with serositis (p = 0.01), decrease of complement (c3, c4; p < 0.01) and blood count change (anemia, leucopenia, lymphopenia; p = 0.032) than in patients without such disease manifestations. The significant correlation found between K/T and neopterin (rs = 0.808, p < 0.001), a marker of immune activation, points to a role of immune activation to be responsible for tryptophan degradation in SLE patients.

Adult↗
PubMed2000

Anaphylaxis to camomile: clinical features and allergen cross-reactivity.

BACKGROUND: Medicinal remedies of plant origin became very popular in recent years, and allergic reactions to these are on the rise, accordingly. Camomile has been reported as a potential trigger of severe anaphylaxis. The allergens responsible for camomile allergy have not been characterized as yet. OBJECTIVE: The present study aims at reviewing the clinical symptomatology of immediate-type reactions in a series of patients sensitized to camomile and at characterizing the responsible allergens. METHODS: Fourteen patients with a history of allergy either to camomile or to spices or weeds, and a positive skin prick test/RAST to camomile were investigated for related allergic reactions to food, pollen and others. IgE-binding patterns were determined by immunoblotting, inhibition tests and deglycosylation experiments. RESULTS: Ten of 14 patients had a clinical history of immediate-type reactions to camomile, in some cases life threatening. Eleven subjects were also sensitized to mugwort in prick or RAST, eight to birch tree pollen. Using a polyclonal rabbit anti-Bet v 1 antibody, a homologue of the major birch pollen allergen Bet v 1 was detected in two camomile blots. In four cases a group of higher molecular weight allergens (23-50 kDa) showed IgE-binding to camomile. All allergens proved heat stable. Binding was inhibited in variable degrees by extracts from celery roots, anize seeds and pollen from mugwort, birch and timothy grass. Deglycosylation experiments proved the presence of carbohydrate determinants in camomile which were not responsible for IgE-binding, though. Profilins (Bet v 2) were not detected in our camomile extracts. CONCLUSION: Incidence and risk of type I allergy to camomile may be underestimated. Concurrent sensitization to mugwort and birch pollen is not infrequent. Bet v 1 and noncarbohydrate higher molecular weight proteins were found to be eliciting allergens and are responsible for cross-reactivity with other foods and pollen.

Adolescent↗
PubMed2000

Acute respiratory failure associated with catastrophic antiphospholipid syndrome.

We present a case of multiple organ dysfunction syndrome with acute respiratory failure due to alveolar haemorrhage associated with antiphospholipid antibodies in a 42-year-old woman with a medical history of antinuclear antibody-negative systemic lupus erythematosus and antiphospholipid syndrome. Severe respiratory failure, circulatory shock and acute renal failure necessitated artificial ventilation, inotropic and vasopressor therapy, and continuous venovenous haemofiltration. A tentative diagnosis of haemorrhagic lupus pneumonitis or pulmonary manifestation of antiphospholipid syndrome was made. Lupus anticoagulant, IgG anticardiolipin and anti-beta2-glycoprotein I antibodies were positive. High-dose glucocorticoid, anticoagulation with heparin, plasmapheresis and cyclophosphamide improved her clinical condition. Despite this, the patient died several days later of spontaneous intracranial haemorrhage. This case illustrates the uncommon manifestation of acute respiratory failure associated with antiphospholipid syndrome.

Acute Disease↗
PubMed1999

Degradation of tryptophan in patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE), a progressive autoimmune disorder, is associated with chronic stimulation of various components of the immune system. Since cell-mediated immunity is also activated, we were interested to test for abnormalities in tryptophan metabolism in SLE which may result from activation of indoleamine 2,3-dioxygenase by cytokines released during the immune response. We measured serum tryptophan and kynurenine concentrations in 52 patients with SLE as well as serum neopterin as an indicator for the degree of immune activation. Compared to controls, we found significantly decreased tryptophan and increased kynurenine concentrations in SLE. The extent of tryptophan catabolism correlates with neopterin concentrations or with the disease activity index. Tryptophan depletion may be associated with neurologic/psychiatric disturbances in patients suffering from SLE.

Depression↗