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This cross-sectional investigation examined the prevalence and severity of dysphonia, globus pharyngeus, and dysphagia in patients affected by immunomediated (IM) diseases. Seventy subjects were administered the Voice Handicap Index (VHI) (scale 0-4), Glasgow-Edinburgh Throat Scale (GETS) (scale from 0 to 7) for globus pharyngeus assessment, and modified Swallowing Outcomes After Revised Laryngectomy (SOAL) (scale 0, 1, 2) to test swallowing symptoms. VHI: the mean percentage of answers with a score greater than 1 (corresponding to a frequency of situation's occurrence "sometimes," "almost always," or "always") was 25.7, 26.7, and 44.1% for functional, emotional, and physical groups of sub-items respectively. GETS: the mean percentage of answers with a score ≥ 3 was 60.85%, significantly higher if compared with that of answers with a score < 3 (40.14%). The mean percentages of answers with a score 0-2, 3-4, and 5-7 were 40.1, 16.7, and 43.7% respectively. SOAL: a mean of 57.9% of answers gained a symptomatic score (1 ["a little"] or 2 ["a lot"]) and 41.9%, the score 0. The difference was statistically significant (p < 0.05). The first two most recurrent items with a score 2 ("a lot") were "Do you have a problem swallowing dry food?" (46%) and "Do you have a problem swallowing solid food?" (36%). The study represents the first to describe the globus pharyngeus symptoms in IM population. Moreover, it allows to confirm the recurrence of dysphonia and dysphagia in this type of patients. Particularly, it has been demonstrated that the alteration of swallowing function is related to solid and dry food. The self-assessment questionnaires proved as a useful tool to early detection of dysfunctions in order to avoid further deterioration of quality of life and to prevent serious life-threatening complications.

Purpose: The purpose of this study was to explore the presence of autoimmune manifestations and characterize the autoantibody production in a cohort of patients with Aicardi-Goutières syndrome (AGS)., Methods: Seventeen patients with a genetically-confirmed diagnosis of AGS were recruited. At the time of enrollment, past medical and family history was reviewed, looking for possible signs or symptoms of autoimmune disorders. Blood samples were taken, for the detection of a panel of autoantibodies: anti-nuclear, anti-double-stranded-DNA, anti-nucleosome, anti-extractable nuclear antigens, anti-cardiolipin IgG/IgM, anti-β2glycoprotein I IgG/IgM, and anti-neutrophil cytoplasmic. We also measured complement levels determined as C3 and C4 quantification and total complement activity, measured as CH50., Results: Nine of seventeen patients presented with at least one first- or second-degree relative with a history of autoimmune diseases (the childrens' mother or grand-mother in the majority of cases). A specific autoimmune disease was present in only one AGS patient, namely an autoimmune thyroiditis. Autoantibodies were present in 9/17 patients, with different patterns of positivity. Complement levels were normal in all the patients. There was no correlation between auto-antibody production and personal or family history of autoimmune diseases., Conclusions: Definite autoimmune diseases are not common in patients with AGS. Autoantibodies are mainly directed towards nucleic acids-containing elements but seem not to be pathogenic and, rather, may represent an epiphenomenon of the enhanced interferon production.

Growing data have provided encouraging results on the use of interleukin (IL)-1 inhibitors in Behçet's disease (BD). This study was aimed at reporting the largest experience with anti-IL-1 agents in BD patients. We evaluated 30 BD patients receiving treatment with anti-IL-1 agents. The primary aims of the study were to evaluate the efficacy of anakinra (ANA) and canakinumab (CAN) in a cohort of BD. The secondary aims were to evaluate the overall safety profile of the treatments, explore the timing of response to therapy and any adjustment of dosage and frequency of drugs studied, and investigate predictive factors of response to therapy. The frequency of first line therapy was 90 % with ANA and 10 % with CAN. The overall number of subjects in complete remission after 12 months of therapy with anti-IL-1 drugs was 13: 6 maintained the initial therapy regimen, 1 maintained the same initial anti-IL-1 drug with further therapeutic adjustments, and the remaining 6 shifted from ANA to CAN. Among them, 3 used CAN for at least 12 months without therapeutic adjustments, 1 had therapeutic adjustments, and 3 had an overall history of a 12-month complete remission. Adverse events (AEs) were reported in 15 % patients who received ANA, represented in all cases by local cutaneous reactions, while no AE were observed in patients who received CAN; we did not observe any serious AEs (SAEs) during the follow-up period. Our data have confirmed that the use of anti-IL-1β drugs is efficacious and safe with an overall acceptable retention on treatment.

The objective of this study is to assess the effectiveness and safety of TNF-α blockers in a group of early psoriatic arthritis (PsA) patients with an unsatisfactory response to previous conventional treatment consecutively enrolled in five Italian centres. A 24-week open-label trial was carried out in consecutive early PsA patients classified according to the CASPAR criteria, with unsatisfactory response to previous treatments and with a DAS28 threshold as ≥3.2, seen at the outpatient clinics of each centre. Exclusion criteria were previous usage of TNF-α blockers and a disease duration >12 months. The choice of any of the three TNF-α blockers was decided by the expert's opinion, without any restriction. Effectiveness was considered as an improvement of DAS28 at 12 and 24 weeks of treatment. Secondary endpoints were an improvement of TJC, SWJ, HAQ score and PASI score. Changes from baseline to the 12- and 24-week follow-up assessments were analysed using the Wilcoxon paired sign rank test. Twenty-nine patients (14 males, 15 females, median age 37 years, range 20-65 years) were enrolled. A statistical improvement of the DAS28 was observed at 12 and 24 weeks from baseline (p<0.001). Secondary endpoints also confirmed the effectiveness of the TNF-α blockers in the treatment of early PsA. No severe adverse events were observed during the treatment period, and no patient withdrew from the medications. This study suggests that the TNF-α blockers can be effective in the management of early PsA. Further controlled studies will provide more data on this challenging topic.

We aimed to establish by using fast spin echo (FSE)-T2-weighted sequences with fat saturation if flexor tendon enthesitis is the primary lesion in spondyloarthritis (SpA) toe dactylitis. Consecutive patients showing toe dactylitis and meeting Amor criteria for the classification of SpA were enrolled. Dactylitic toes and their corresponding normal contralateral digits were studied by FSE-T2-weighted sequences with fat saturation. Twelve dactylitic toes belonging to ten SpA patients were studied. All dactylitic toes showed mild-to-moderate fluid collection in the synovial sheaths of flexor digitorum brevis and longus. Involvement of joint cavity was simultaneously seen in at least one joint of eight (66.6%) out of the 12 toes. A mild-to-severe peritendinous soft tissue edema was observed in all but one of the affected toes. In no dactylitic toe was bone edema observed either near the insertions of the flexor digitorum brevis and longus tendons or in other sites of the phalanges. No lesions were observed in the 12 contralateral clinically normal toes. In SpA toe dactylitis there is no evidence of enthesitis of the flexor digitorum brevis and longus tendons and joint capsules.

We evaluated the prevalence of hepatitis C virus (HCV) infection in Italian patients suffering from fibromyalgia (FM), in comparison with patients affected by non-HCV related rheumatic degenerative disorders. Consecutive patients with FM and a statistically comparable group of patients suffering from peripheral osteoarthritis (OA) or sciatica due to L4-L5 or L5-S1 herniated disc were tested for HCV infection with a third-generation microparticle enzyme immunoassay (MEIA). In the positive cases, a third-generation recombinant immunoblot assay (RIBA) confirmatory test and serum HCV-RNA test were performed. Fisher's exact test was performed to compare the prevalence of HCV infection (MEIA- and RIBA-positive results) obtained in the two enrolled groups. Enrolled were 152 subjects suffering from FM and 152 patients with peripheral OA or sciatica. Anti-HCV antibodies were found in 7/152 (4.6%) patients suffering from FM and in 5/152 (3.3%) of control subjects. No statistically significant differences in HCV prevalence were detected between cases and controls. Our present report does not confirm previous data indicating an increased prevalence of HCV in FM patients and does not seem to support a significant pathogenetic role of HCV under this condition.

Hereditary angioedema (HAE) is an autosomal dominant disease that causes recurrent attacks of non-pitting edema of soft tissues, without pruritus. This disorder can also affect internal organs. The cause of HAE consists in quantitative or qualitative defective production of C1 inhibitor (C1-INH). Many autoimmune diseases such as systemic lupus erythematosus (SLE) (or SLE-like syndromes), Sjögren's syndrome, scleroderma, thyroiditis, glomerulonephritis, and inflammatory bowel disease have been described in patients suffering from HAE. A concomitance with pure arthritis was previously reported only in two adult patients. Here, we describe for the first time the association between HAE and a non-rheumatoid erosive oligoarthritis involving hips and wrists.

A patient suffering from Behçet's syndrome and undifferentiated spondyloarthritis developed dactylitis and psoriasis over the next three years.

The case of a man suffering from undifferentiated seronegative spondyloarthropathy (uSpA) without spine involvement and with iliolumbar ligament ossification is reported. Unlike a similar previously published case showing ossification only of the middle part of the left ligament, our patient had ossification of the whole course of both ligaments.

The case of a patient with seronegative spondyloarthropathy showing oligoarthritis of the hand together with large pitting oedema is reported. Unlike the patients with late onset peripheral spondyloarthropathy described by Dubost and Sauvezie who show minimal involvement of the axial skeleton, the patient has been suffering from AS for about twenty years.

We have investigated the relationship between phenotypic and genetic correlations among a large number of quantitative traits (36) in three different environments in order to determine their degree of disparity and whether phenotypic correlations could be substituted for their genetic counterparts whatever the environment. We also studied the influence of the environment on genetic and phenotypic correlations. Twenty accessions (full-sib families) ofMedicago luPulina were grown in three environments. In two of these two levels of environmental stress were generated by harvesting plants at flowering and by growing plants in competition with barley, respectively. A third environment, with no treatment, was used as a control with no stress. Average values of pod and shoot weight indicate that competition induces the highest level of stress. The genetic and phenotypic correlations among the 36 traits were compared. Significant phenotypic correlations were obtained easily, while there was no genetic variation for 1 or the 2 characters being correlated. The large positive correlation between the genetic and phenotypic correlation matrices indicated a good proportionality between genetic and phenotypic correlations matrices but not their similarity. In a given environment, when only those traits with a significant genetic variance were taken into account, there were still differences between genetic and phenotypic correlations, even when levels of significance for phenotypic correlations were lowered. Consequently, it is dangerous to substitute phenotypic correlations for genetic correlations. The number of traits that showed genetic variability increased with increasing environmental stress, consequently the number of significant genetic correlations also increased with increasing environmental stress. In contrast, the number of significant phenotypic correlations was not influnced by the environment. The structures of both phenotypic and genetic matrices, however, depended on the environment, and not in the same way for both matrices.

A pair of identical twins suffering from ankylosing spondylitis is reported. One brother developed an earlier-onset disease and showed ossification of the posterior longitudinal ligament and the flavum ligament in his cervical spine.

The case of a 49-year-old man affected by Behçet's syndrome (BS) without any clinical or radiological evidence of ankylosing spondylitis, exhibiting a peripheral enthesitis typical of seronegative spondyloarthropathy (SpA) is reported. The diagnosis of SpA is supported by computed tomographic evidence of sacroiliitis. This case confirms our hypothesis that patients with BS may have other forms of SpA than AS.

The case of a 59-year-old man with definite HLA B27 negative ankylosing spondylitis (AS) and Paget's disease involving the skull, the right side of the pelvis and the proximal femur is reported. Fifteen cases with this coexistence have previously been described. Some of these, however, are patients with Paget's disease mimicking AS. It is emphasized again that attention must be paid to diagnosing AS in patients suffering from Paget's disease.

Carduus pycnocephalus and C. tenuiflorus are two important weed species in mediterranean-type ecosystems. They were studied in their native habitat in southern France from April to December 1981.These species both produce two types of seed (achenes) in each capitulum. Those in the center are not dormant and are easily dispersed, while the outer ones can be dormant and have no apparent means of dispersal. Our observations on pollen transfer and self fertilization show that the dispersable, non dormant seeds have a low probability of inbreeding, while the potentially dormant, non dispersed seeds have a high probability of inbreeding. The significance of this dimorphism is discussed.

The interaction of host plant phenology and microclimatic heterogeneity was examined to determine its role in the population dynamics of checkerspot butterflies, Euphydryas editha, inhabiting serpentine grassland in California's outer Coast Range.Within the 2-3 hectares inhabited by a population of E. editha (Jasper Ridge Area H), microclimatic differences resulting from topographic heterogeneity largely determine the temporal and spatial pattern of senescence of the larval host plants, Plantago erecta and Orthocarpus densiflorus. Survival of larvae from hatching to diapause is extremely low as a result of unpredictable variation in the timing of larval development relative to the timing of host plant senescence, both of which are mediated by microclimatic patterns. During this study, population H declined to near extinction as a result of two consecutive years of record rainfall that apparently disrupted the tenuous temporal relationship between larval development and plant senescence. Retarded development of post-diapause larvae led to a late and extended flight season and delayed egg production; this in turn resulted in massive mortality of pre-diapause larvae due to starvation because host plant senescence occurred before larvae became large enough to enter diapause. Adult population size the following spring was the smallest in 25 years of study. This work emphasizes the importance of microclimatic heterogeneity for understanding population-level processes in small ectothermic animals and underlines the potential importance of such heterogeneity in the establishment of reserves designed to protect such animals.

A case of erosive sterile arthritis following meningococcal meningitis is described. High levels of immune complexes were detected in serum and synovial fluid. This is the first case in literature in which destruction of subchondral bone is documented. The erosion showed a progressive remineralization in the six months following clinical recovery.