Maria Paola Loria

Reactivity to autologous serum skintest and clinical features in chronic idiopathic urticaria

Summary The autologous‐serum skin test (ASST) can cause a wheal‐and‐flare response in some cases of chronic idiopathic urticaria. We subjected 102 patients affected by chronic idiopathic urticaria to this test and studied some clinical parameters to detect any significant differences between ASST‐positive and ASST‐negative patients. The only significant difference we noted between the two groups was the incidence of angioedema (P = 0.01). We suggest that the ASST cannot be used alone either to predict the severity of urticaria or to define it as ‘autoimmune’.

Patients with Hereditary Hemorrhagic Telangectasia (HHT) Exhibit a Deficit of Polymorphonuclear Cell and Monocyte Oxidative Burst and Phagocytosis: A Possible Correlation with Altered Adaptive Immune Responsiveness in HHT

Hereditary Hemorrhagic Telangiectasia (HHT) is a rare genetic disease characterized by mutations occurring in the endoglin and ALK-1, two receptors of transforming growth factor-beta1. From a pathogenic point of view, a possible involvement of the immune system in HHT has been suggested since a mononuclear cell infiltrate was found around the area of telangiectases. Up until now, no information has been available about the role played by leukocytes in HHT and the mechanisms elicited by secretion of their mediators. However, the fact that a deficit of adaptive immunity in HHT has been reported in a companion paper in this issue will represent a great contribution to the understanding of HHT pathogenesis. The purpose of this study was to evaluate whether patients with HHT manifest also alterations in the innate immune response. Therefore, the phenotype of T, B and natural killer lymphocytes, serum immunoglobulin levels, phagocytosis and oxidative burst activity exerted by polymorphonuclear cells (PMN) and monocytes (MO) were analyzed in 22 patients. Twenty individuals demonstrated single or multiple deficits of PMN and MO functions, while the immunophenotype of lymphocytes and serum concentrations of immunoglobulins were normal. To the best of our knowledge, this is the first demonstration of a reduction in PMN and MO functions in HHT, thus suggesting a higher susceptibility to infectious complications in these patients. The relationship between innate immune deficits and T helper 1 and monocyte-derived cytokine dysfunction in HHT, as previously reported, is discussed.

CYCLOSPORIN A IN PATIENTS AFFECTED BY CHRONIC IDIOPATHIC URTICARIA: A THERAPEUTIC ALTERNATIVE

Chronic Idiopathic Urticaria (CIU) is a cutaneous disorder for which there is no identifiable specific etiologic agent. Some recent evidences suggest that CIU might be an autoimmune disease. We analyzed immunological features occurring in CIU and evaluated effectiveness and tolerance of Cyclosporin A (CsA) treatment in patients unresponsive to antihistaminic treatment. Twenty patients with CIU were recruited after a selective diagnostic protocol and were divided into two groups. CsA was prescribed for group 1 and Prednisone for group 2 as control, for 8 weeks. Before and after the therapy we performed on all patients immunological studies. For all patients symptoms disappeared after a few days of therapy. Before therapy all patients showed activated B cells (CD19+CD23+ cells) and among B CD19+ cells, about 20% were CD5+ (cells that synthesize natural autoantibodies). After treatment with Prednisone in group 2, a significant reduction of CD4+ lymphocytes (p + 0,01) was observed. Our findings might support the CIU autoimmune pathogenetic hypothesis. The clinical remission in the CsA-treated group confirmed the therapeutic effectiveness of this therapy in antihistaminic unresponsive CIU and, at dosage used, side effects were rare, mild and reversible. Thus, CsA might be a good therapeutic alternative in CIU patients unresponsive to conventional treatments.

Acquired C1-inhibitor deficiency in a patient with systemic lupus erythematosus: a case report and review of the literature

The C1‐inhibitor (C1‐INH) is an important member of the serpin family which inhibits the first component of the human complement system and controls contact activation of the coagulation and kinin system. An acquired form of C1‐INH deficiency was recognized and classified as type I, which is characterized by accelerated catabolism of C1‐INH, whereas type II is defined by the presence of an autoantibody directed against the C1 inhibitor molecule. This study reports the case of a 32‐year‐old woman with systemic lupus erythematosus (SLE) who experienced recurrent angioedema because of an acquired C1‐INH deficiency. The relevant literature is reviewed.

Type I allergy to natural rubber latex and Type IV allergy to rubber chemicals in children with risk factors.

Keywords: natural rubber latex; allergic contact dermatitis; rubber chemicals; Type I hypersensitivity; Type IV hypersensitivity; thiuram mix; tetramethylthiuram monosulfide; carba mix; 1,3-diphenylguanidine; hexamethylenetetramine

Effects of Oral Bacterial Immunotherapy in Children with Atopic Eczema/Dermatitis Syndrome

BackgroundAtopic eczema/dermatitis syndrome (AEDS) is a chronic inflammatory skin disease, affecting 10–20% of children and 1–3% of adults. The purpose of this pilot study was to assess the clinical and anti-inflammatory effect of bacterial and ribosomal immunotherapy with Immucytal® (Pierre Fabre Médicament, France) in children with AEDS.MethodsSeventeen children with allergic and non-allergic forms of AEDS (AAEDS and NAAEDS, respectively), graded moderate to severe (Severity Scoring of Atopic Dermatitis [SCORAD] index of >25), received ribosomal immunotherapy (Immucytal®) once daily according to the standard treatment regimen (4 consecutive days a week for 3 weeks, and then 4 consecutive days a month for 4 months). We assessed the clinical status of AEDS using the SCORAD index at baseline, and after 8 and 20 weeks of treatment. Furthermore, peripheral blood from patients was examined for the frequencies of CD4+ cells expressing interferon (IFN)-ψ and interleukin (IL)-4 using flow cytometry.ResultsThere was a progressive and significant clinical improvement of AEDS, confirmed by a reduction of the SCORAD index over time in both AEDS forms (p < 0.01). Pooled data from the two groups showed that the mean baseline index of 43 was reduced to 17 after treatment. Overall, these data indicate a marked improvement in total clinical severity of AEDS (−62%). Flow cytometry analysis showed that frequencies of the two CD4+ T cell subsets did not differ significantly from the beginning to the end of the study in both forms of AEDS. However, the percentage of CD4+ cells expressing IL-4 in children with AAEDS tended to decrease by the end of treatment with ribosomal immunotherapy. Clinical and laboratory data confirmed that immunotherapy was well tolerated.ConclusionsThe results of this pilot investigation suggest that ribosomal immunotherapy may be beneficial in the management of AEDS in children, and that this could be at least partially explained by a role in restoring the type 2 helper-T cell imbalance seen in allergic patients. Placebo-controlled, randomized clinical trials are recommended in order to confirm these findings.

Adhesion molecules in gonarthrosis and knee prosthesis aseptic loosening follow-up: possible therapeutic implications.

The involvement of the synovium is common in phlogistic processes of various joint diseases. A part from synoviocytes and the other cells in the synovial tissue, circulating cells recruited from peripheral blood also participate in the phlogistic process. The increased expression of adhesion molecules on both circulating and endothelial cell surface may further this recruitment. We studied 15 patients affected by serious gonarthrosis requiring a prosthetic implant (GPI) and 7 with knee prosthesis aseptic loosening (KPL) to evaluate adhesion molecule expression and phlogistic infiltration in the synovium using immunohistochemistry and microscopic analysis. As control we studied 10 subjects affected by degenerative meniscopathies undergoing a selective arthroscopic surgical meniscectomy. Analysis with Kruskal-Wallis test showed no statistical significant differences in the expression of CD54, CD11a, CD11b and CD18 in three groups examined. The model of variance analysis (Friedman test), showed that CD54 expression is greater in patients with GPI and KPL in comparison with the other molecules. Adhesion molecules and their functions are important in arthropathies not only because their evaluation can allow us to identify the degree of inflammation and to predict its evolution, but also because pharmacological control of their expression could have important therapeutic implications.