The dilemma of diagnosing fever of unknown origin: large arteries vasculitis revealed by 18F-FDG PET/CT imaging. A case report.

Fever of unknown origin (FUO) is an uncommon disease, and its underlying etiology may include a number causes, i.e., infections, malignancies, autoimmune conditions. Diagnosis is often a difficult task, and usually physician spend time and money in order to define the etiology of FUO. We report a case of patient who presented with FUO and headache, and positron emission tomography (PET) with 2-deoxy-2-[fluorine-18] fluoro-D-glucose (18F-FDG) allowed to reveal the presence of a large vessel vasculitis. 18F-FDG PET may represent an useful tool in patients with FUO, since it can early depict an hypermetabolic activity due to inflammation and so help to achieve a final diagnosis in some cases of FUO.

[Hyper-IgD syndrome and other hereditary periodic fever syndromes]. / La sindrome da Iper-IgD e altre sindromi febbrili periodiche ereditarie.

Hereditary periodic fever syndromes are a group of systemic disorders characterized by recurrent attacks of systemic inflammation (autoinflammation) without infectious or autoimmune cause. The hyper-IgD syndrome (HIDS) is a rare autosomal recessive inflammatory disorder characterized by recurrent fever, increased serum IgD (normal value < 100 U/ml) and generalized inflammation (lymphadenopathy, arthralgias/arthritis, abdominal complaints, skin rash, and headache). The attacks persist during the entire life although frequency and severity tend to diminish with age. HIDS is caused by specific mutations in the gene encoding mevalonate kinase, resulting in depressed enzymatic activity. At present the therapy for the syndrome is only supportive. Other than HIDS, other hereditary systemic inflammatory disorders have been described: the Familial Mediterranean Fever, the tumour necrosis factor receptor associated periodic syndrome (TRAPS), a disease related to the mutations of one of the TNF receptors, the Familial Cold Urticaria and the Muckle-Wells syndrome. The differential diagnosis with other causes of periodic fever is crucial for assessing appropriate management and treatment.

[Pancreatic duct antibodies and subclinical insufficiency of the exocrine pancreas in Sjögren's syndrome]. / Gli anticorpi anti-duttuli e l'insufficienza subclinica del pancreas esocrino nella sindrome di Sjögren.

In previous studies we reported evidence of subclinical exocrine pancreatic insufficiency in primary or secondary Sjögren's syndrome (SSI, SSII) and rheumatoid arthritis (RA). In present study we evaluated the occurrence of pancreatic duct antibodies (PDA), and their relationship to exocrine pancreatic function in 36 women. Of these patients, 12 were classified as SSI, 12 as SSII, and 12 as RA. Exocrine pancreatic function was evaluated using the Secretin-Caerulein test (S.Cae test). The indirect immunofluorescent technique was used to evaluate patient sera for PDA. S.Cae test results, compared to controls, showed a statistically significant decrease in duodenal juice volumes, bicarbonates and enzymes in 58.3% of SSI, in 58.3% of SSI and in 30% of RA, according to our previous trials. PDA were found in two patients, the former with SSI and the latter with SSII, both asymptomatic with regard to pancreatic diseases but with impaired exocrine pancreatic function as evaluated by S.Cae test. We discuss the possible causes of these results.

Adverse food reactions in patients with grass pollen allergic respiratory disease.

The purpose of this study was to assess the frequency of reported food-induced symptoms, and of food allergy or intolerance in 169 allergic patients monosensitized to grass pollen and in a control group of 50 patients who were monosensitized to Dermatophagoides. This study clearly demonstrates that patients with grass pollen-allergic respiratory disease report adverse food reactions more frequently than patients allergic to Dermatophagoides. This increased incidence is due to a high percentage of adverse reactions to some vegetable foods, especially peanut, garlic, tomato, onion; and fruits, such as peach; and animal foods, such as egg (white) and pork. By separating the food-allergic patients from the food-intolerant patients, the number of subjects with food intolerance was higher than that of the patients with food allergy. Crossreactivity between pollen allergens and fruits and vegetable allergens may explain the association between pollen allergy and food allergy, but not the higher incidence of food intolerance. An increased intestinal permeability to macromolecules may be hypothesized as part of a primary defect in permeability in "atopic (pollen allergic?) constitution."

T-cell phenotypic profiles in the cerebrospinal fluid and peripheral blood of multiple sclerosis patients.

Thirty-nine patients with clinically definite multiple sclerosis (MS) entered the study. Of 28 subjects with a relapsing-remitting course, 19 were classified in acute relapse, 9 in remission; 11 patients had a progressive course without remissions. Furthermore, 6 subjects with inflammatory neurological disease (IND), and 10 with non-inflammatory and non-neoplastic neurological disease (NIND) were investigated. We simultaneously studied cerebrospinal fluid (CSF) and peripheral blood (PB) T-, B- and NK-cell subsets, as defined by following monoclonal antibodies: anti-CD3, -CD4, -CD8, -CD19, -CD16, -HLA-DR and -IL-2-R. We found a significant increase of CD4+ T-cells compared with controls in CSF, with respect to PB, of MS patients, particularly in acute relapse. An increase of HLA-DR+ cell percentages in the CSF than in the PB in all MS groups, especially in attacks of MS but also in remission, was also observed, with a positive correlation between CD4+ T-cell and DR+ cell percentages both in the CSF as well as in the PB of relapsing MS patients. These findings, together with the increase of IL-2-R+ cells in the PB, particularly in relapsing MS, give further support for the presence of a systemic T-cell activation in MS.

Comparative evaluation of RAST and MAST-CLA for six allergens for the diagnosis of inhalant allergic disease in 232 patients.

The aim of this study was to compare a recent multiple allergosorbent chemiluminescent assay (MAST-CLA) with the RAST for the diagnosis of inhalant allergic disease in 232 patients with rhinitis and/or bronchial asthma. As judged by concordance of clinical history, skin prick tests to a range of six allergens common to our geographic area, and by nasal provocation tests, 70 patients were non-allergic and 162 allergic: 70 to grasses, 46 to mites, four to mugwort, eight to pellitory, and 34 were sensitive to several allergens. In our patient sample that, among other things, comprises subjects with fairly rare monoallergies, MAST-CLA testing gave results which closely corresponded to positive RAST for the allergens studied, and demonstrated a close correlation with the diagnosis of inhalant-specific allergy. Our results showed that, for overall allergens, MAST-CLA was lightly less sensitive and more specific than RAST (the two in vitro tests gave an identical overall efficiency).

Does intravenous streptokinase therapy facilitate the formation of anti-heart antibodies in acute myocardial infarction?

Streptokinase (SK), a nonenzymatic protein produced by group C beta haemolytic streptococci, is a potent antigen. It is used worldwide as a thrombolytic agent in the treatment of acute myocardial infarction (AMI). Specific antiheart antibodies (AHA) have been found with a significantly high incidence in patients with AMI, and after streptococcal infection as a result of stimulation by constituents of the group A streptococci antigenically cross-reactive with sarcolemmal portion of the muscle fiber of the heart. Since there may be partial antigenic identity of group C streptococcal membranes with membranes isolated from group A streptococci, we have designed a prospective study to evaluate the incidence of serum AHA (and of other organ-specific and non-organ-specific antibodies) in 36 patients with AMI, 14 of whom treated with SK. AHA, of IgG class, were of the sarcolemmal-subsarcolemmal type, and did not fix complement. They were found in 4/36 patients already on admission; of the 32 patients negative, none developed AHA later, on days 7, 15 and 21 of hospitalization, also after treatment with SK (in 14 cases). There was no significant difference either within or between the two SK-treated and non-SK-treated groups also with regard to the incidence of organ-specific and non-organ-specific autoantibodies. These findings do suggest that the intravenous SK therapy does not facilitate the formation of AHA in AMI.

Correlation of MAST chemiluminescent assay (CLA) with RAST and skin prick tests for diagnosis of inhalant allergic disease.

The purpose of this study was to compare the new multiple allergosorbent chemiluminescent assay (MAST CLA) system with the RAST and skin prick tests as an adjunct to the diagnosis of inhalant allergies. In this report, MAST CLA and RAST have given similar results, but no technique is as sensitive as skin tests for allergen-specific diagnosis of inhalant allergic disease.

[Serum immunoconglutinin in patients with a respiratory allergy syndrome, before and after specific immunotherapy]. / L'immunoconglutinina serica in pazienti con sindrome allergica respiratoria, prima e dopo immunoterapia specifica.

Immunoconglutinins (Ic) are a group of predominantly IgM antibodies formed towards antigenic determinants exposed in fixed complement components (C3b and C4). Ic production is stimulated by bacterial and viral infections; elevated titers were also found in a number of other diseases involving completement-fixing reactions in vivo. High titers have been associated with the infectious morbidity within a particular population. The object of this investigation was to determine the correlations of autostimulated Ic titre with several parameters in 100 atopic nondesensitized subjects with respiratory allergies and in 140 non atopic, healthy, individuals. Eighty of them had rhinitis and/or conjunctivitis, 9 asthma and 11 rhinitis and asthma. All the atopics were studied also after at least 6 months of specific immunotherapy. None of the subjects received any other therapy at the time of study. Atopic subjects with high titers of Ic are significantly more numerous than controls, without significant correlation with the kind and beginning of atopic syndrome, and with immunotherapy. These results might be attributed to a higher incidence of infections in the atopic population. The Ic might play an important role in the immunoregulation involving complement system.

[Plasma cell leukemia. Bibliographic review and ultrastructural study (by transmission and scanning electron microscopy) in 2 cases]. / La leucemia plasmacellulare. Revisione bibliografica e studio ultrastrutturale (al M.E.T. ed al M.E.S.) in due casi.

After a brief examination of the recent literature on plasma cell leukemia, the Authors describe the clinical features and the ultrastructural findings of the peripheral blood plasma cells, examined with transmission (T.E.M.) and scanning (S.E.M.) electron microscopes, of two patients with acute plasma cell leukemia. Both of them had a previous history of myeloma. T.E.M. confirmed the diagnostic value of the asynchronous plasma cells, and S.E.M. showed the characteristic microvilli and blebs, previously observed.

[Non-secretory plasmacytoma. Bibliographic review and immunohistochemical study of a case]. / Plasmocitoma non secernente. Revisione bibliografica e studio immunoistochimico di un caso.

After a brief examination of the recent literature on non-secretory multiple myeloma, the Authors describe the immunohistologic study (peroxidase-antiperoxidase method) of a case of truly non producing plasmacytoma, interesting because of the presence of a small polyclonal plasma cell population within the neoplastic clone. Several possible explanations are considered.

[Autoantibodies and respiratory allergy: immunologic case study]. / Autoanticorpi e allergie respiratorie: studio casistico immunologico.

Sera obtained from 212 patients with rhinopathy and/or asthma of allergic origin (pollens and/or mites) and from 87 apparently healthy controls were studied for the presence of organ and non organ specific autoantibodies. These were determined by indirect immunofluorescence techniques (5) using test tissue from monkeys (thyroid) and rats (stomach, liver and kidney). Two groups were matched for age and sex and the tests were performed using the double-blind method. The incidence observed was not dissimilar to that detected in healthy subjects, with the exclusion of smooth-muscle antibodies. These were present to a lesser extent in patients with respiratory allergies (0.94%) in contrast with the incidence of 5.73% in normal subjects (P less than 0.01). On the basis of our findings, it seems possible to hypothesize that allergic patients have a lesser tendency to autoimmune diseases because the immune system is committed to the IgE-mediated immunoreactions. In conclusion, autoantibodies do not seem to play a role in the pathogenesis of respiratory allergies to inhaled allergens.