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Background: Ear involvement is not unusual in autoimmune diseases, but few data on this problem in the Sjögren syndrome are available.Objectives: To determine whether the incidence of hearing loss is increased in patients with the Sjögren syndrome and to determine what factors might be involved in the pathogenesis of Sjögren syndrome-related hearing loss.Design: Cross-sectional study.Setting: Secondary referral center in Italy.Patients: 30 women with the Sjögren syndrome were evaluated for evidence of audiovestibular disorder. Their results were compared with those of 40 age-matched healthy women.Measurements: Evaluation techniques included pure tone audiometry thresholds at octave frequencies of 125 Hz to 8000 Hz, tympanometry, and the stapedial reflex test in both ears. The presence of vasculitis, the Raynaud phenomenon, nervous system involvement, and serum anticardiolipin antibodies was recorded.Results: 14 patients with the Sjögren syndrome (46% [95% CI, 28% to 66%]) had sensorineural hearing loss. Only one control (2.5% [CI, 0.06% to 13%]) had a similar hearing impairment (P < 0.001). Nine of the 14 patients who had the Sjögren syndrome and sensorineural hearing loss (64% [CI, 36% to 87%]) had anticardiolipin antibodies compared with only 3 controls (18% [CI, 4% to 45%]) (P = 0.02).Conclusion: The high prevalence of hearing loss in the Sjögren syndrome supports the value of doing audiometric studies before excluding cranial nerve involvement in this disease. The correlation of sensorineural hearing loss with anticardiolipin antibodies must be investigated further. [ABSTRACT FROM AUTHOR]

Objectives: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV., Methods: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV. We enrolled patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), the latter with poor-prognosis factors and/or peripheral neuropathy. Remission was induced with cyclophosphamide. At remission, the patients were randomised to receive methotrexate or to continue with cyclophosphamide for 12 months; after treatment, they were followed for another 12 months. The primary end-point was relapse; secondary end-points included renal outcomes and treatment-related toxicity., Results: Of the 94 enrolled patients, 23 were excluded during remission-induction or did not achieve remission; the remaining 71 were randomised to cyclophosphamide (n = 33) or methotrexate (n = 38). Relapse frequencies at months 12 and 24 after randomisation were not different between the two groups (p = 1.00 and 1.00). Relapse-free survival was also comparable (log-rank test p = 0.99). No differences in relapses were detected between the two treatments when GPA+MPA and EGPA were analysed separately. There were no differences in eGFR at months 12 and 24; proteinuria declined significantly (from diagnosis to month 24) only in the cyclophosphamide group (p = 0.0007). No significant differences in adverse event frequencies were observed., Conclusions: MTX may be effective and safe for remission-maintenance in AAV., Trial Registration: clinicaltrials.gov NCT00751517.

Objective: To investigate the epidemiology of granulomatosis with polyangiitis (GPA) over a 15-year period in a defined area of northern Italy., Methods: All patients with incident GPA diagnosed from January 1, 1995 to December 31, 2009 living in the Reggio Emilia area were identified by looking at computerized hospital discharge diagnoses, by contacting Reggio Emilia Hospital physicians and community-based specialists, and by checking the databases of the pathology and the laboratory departments and the Reggio Emilia district database for rare diseases. Patients were classified according to the European Medicines Agency (EMA) algorithm. Patients were followed up from the time of diagnosis until either their death or December 31, 2011. For each case, we identified 20 control subjects from the same geographic area matched for age and gender., Results: A total of 18 patients (7 men and 11 women) with GPA were identified. The overall age- and sex-adjusted incidence rate (IR) was 2.4 per million (95% CI: 1.2-3.5). The mean annual IR increased from 1.7/million/year during 1995-1999 to 3.4 during 2005-2009. The highest IR occurred in females aged 70-79 years (13.5 per million; 95% CI: 5.0-30.0) and in males aged ≥ 80 years (14.9 per million; 95% CI: 2.5-49.4). The prevalence of GPA on December 31, 2009 was 34.3 per million (95% CI: 20.3-54.2). The point prevalence per million increased from 17.8 (95% CI: 7.7-35.1) in 1999 to 34.3 (95% CI: 20.3-54.2) in 2009. Survival among individuals with GPA was significantly reduced compared to that observed in the matched control population (p < 0.001)., Conclusion: In the Italian population, GPA is very uncommon and GPA patients have reduced survival., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Granuloma annulare (GA) is a relatively common, self-limiting condition that can be associated with disorders such as diabetes mellitus, malignancy, and thyroid disease, and with the use of some drugs. Topiramate is approved for the prevention of migraine. Its adverse effects include somnolence, fatigue, paresthesia, anorexia and weight loss, and other abnormalities., Objectives: We report a 50-year-old woman in whom topiramate at 50 mg/d was initiated in January 2010., Case Report: One month after starting topiramate, the patient presented with painless nodules on the left ankle, which later spread to the left leg. Histopathology of a punch biopsy revealed lymphohistiocytic infiltrate, collagen degeneration, and mucin deposition, all of which are characteristic of GA. Two weeks after the discontinuation of topiramate, the lesion resolved. Two years later, the patient resumed topiramate. Two weeks later, a new GA appeared in the same area and disappeared within a few weeks of discontinuation of the drug., Conclusions: Associations between the use of topiramate and a GA-like reaction have been reported in recent years. Based on the present case, it would appear that an actual association between GA and topiramate is possible given that: (i) the GA appeared only after the initiation of topiramate; (ii) the GA resolved after the discontinuation of topiramate; (iii) the GA reappeared with the resumption of topiramate in the same area and with the same characteristics as previously; and (iv) the lesion healed after topiramate was suspended., (© 2013 The International Society of Dermatology.)

Objectives: PTPN22 is involved in T-cell activation and its R620W single-nucleotide polymorphism (SNP) has been shown to predispose to different autoimmune diseases. The aims of this study were to investigate the role of the PTPN22 R620W SNP in conferring susceptibility to the ANCA-associated vasculitides (AAVs), and to explore potential associations between the PTPN22 genotype and the disease manifestations., Methods: PTPN22 R620W SNP was genotyped in a cohort of 344 AAV patients [143 with granulomatosis with polyangiitis (Wegener's) (GPA), 102 with microscopic polyangiitis (MPA) and 99 with Churg-Strauss syndrome (CSS)] and in 945 healthy controls., Results: The frequency of the minor allele (620W) was significantly higher in GPA patients than in controls [P = 0.005, χ(2 )= 7.858, odds ratio (OR) = 1.91], while no statistically significant association was found with MPA or CSS. Among GPA patients, the 620W allele was particularly enriched in ANCA-positive patients as compared with controls (P = 0.00012, χ(2 )= 14.73, OR = 2.31); a particularly marked association was also found with ENT involvement (P = 0.0071, χ(2 )= 7.258, OR = 1.98), lung involvement (P = 0.0060, χ(2 )= 7.541, OR = 2.07) and skin manifestations of all kinds (P = 0.000047, χ(2 )= 16.567, OR = 3.73)., Conclusion: The PTPN22 620W allele confers susceptibility to the development of GPA (but not of MPA or CSS), and particularly of its ANCA-positive subset.

Background and Purpose: Wernicke encephalopathy is a severe neurologic disorder that results from a dietary vitamin B1 deficiency. It is characterized by changes in consciousness, ocular abnormalities, and ataxia. This study was undertaken to analyze and compare findings on MR imaging and neurologic symptoms at clinical presentations of patients with Wernicke encephalopathy with and without a history of alcohol abuse., Materials and Methods: A multicenter study group retrospectively reviewed MR brain imaging findings, clinical histories, and presentations of 26 patients (14 female, 12 male) diagnosed between 1999 and 2006 with Wernicke encephalopathy. The age range was 6-81 years (mean age, 46 .6+/-19 years)., Results: Fifty percent of the patients had a history of alcohol abuse, and 50% had no history of alcohol abuse. Eighty percent showed changes in consciousness, 77% had ocular symptoms, and 54% had ataxia. Only 38% of the patients showed the classic triad of the disease at clinical presentation. At MR examination, 85% of the patients showed symmetric lesions in the medial thalami and the periventricular region of the third ventricle, 65% in the periaqueductal area, 58% in the mamillary bodies, 38% in the tectal plate, and 8% in the dorsal medulla. Contrast enhancement of the mamillary bodies was statistically positively correlated with the alcohol abuse group., Conclusions: Our study confirms the usefulness of MR in reaching a prompt diagnosis of Wernicke encephalopathy to avoid irreversible damage to brain tissue. Contrast enhancement in the mamillary bodies is a typical finding of the disease in the alcoholic population.

Objective: To identify the prognostic factors of relapse and/or death during the course of primary small-vessel vasculitides (PSVV), and to differentiate their prognostic relevance by the type of vasculitis., Methods: Seventy-five patients were retrospectively followed up after diagnosis: 36 with Wegener's granulomatosis (WG), 23 with Churg-Strauss syndrome (CSS), and 16 with microscopic polyangiitis. Cox regression analysis was used to identify the significant predictors of relapse and death., Results: Gastrointestinal (GI) involvement was associated with an increased risk of relapse, mainly in the patients with CSS, whereas renal disease and perinuclear antineutrophil cytoplasmic antibody positivity were correlated with a lower risk of relapse. Presence of nasal Staphylococcus aureus tended to increase the risk of relapse in CSS [hazard ratio (HR) 4.45, p = 0.087], but to decrease it in WG (HR 0.12, p = 0.066). Older age, renal and hepatic involvement, erythrocyte sedimentation rate >or= 100 mm/h, and serum creatinine level >or= 1.5 mg/dl were all related to higher risk of death in univariate analysis; however, only cerebral (HR 8.52, p = 0.021) and hepatic involvement (HR 4.40, p = 0.028) and serum creatinine level >or= 1.5 mg/dl (HR 5.72, p = 0.044) were independently correlated with an unfavorable prognosis for survival. The risk of death associated with each of these indicators did not depend on the form of PSVV., Conclusion: GI involvement increases the risk of relapse in CSS, whereas the prognostic significance of nasal S. aureus in terms of relapse seems to be opposite in patients with CSS and those with WG. Patients with cerebral, hepatic, and renal involvement have the poorest prognosis for survival. Our data do not show that the prognostic relevance of these factors depends on the form of PSVV.

Background: Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients., Methods: We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS., Results: There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10)., Conclusion: Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.

We report a case of widespread immune activation with moderate cytopenia during acute infection with human parvovirus B19 in an adult female patient, in whom five criteria for the diagnosis of systemic lupus erythematosus were present at disease onset. Our case is unusual due to the presence of a cutaneous rash mimicking leukocytoclastic vasculitis at presentation, moderate leukopenia with thrombocytopenia and the presence of a broad array of autoantibodies. Diagnosis was established on the grounds of serological tests confirming recent infection with human parvovirus B19; spontaneous regression of clinical and laboratory abnormalities was observed within 16 weeks, ruling out classic systemic lupus erythematosus. We conclude by proposing that human parvovirus B19 infection should be included in the differential diagnosis of lupus-like syndromes in adult patients.

Kikuchi's disease or histiocytic necrotizing lymphadenitis is a recognized cause of benign lymphadenopathy, although the current clinical literature also supports a link with systemic lupus erythematosus (SLE). Eight patients represent all the cases of Kikuchi's disease admitted to our hospital from 1990 to 1997. They were all young women of median age of 31 years (range 22-43 years). All patients showed cervical lymphadenopathy, and in two cases splenomegaly was present. In one patient, the diagnosis of lupus preceded the onset of Kikuchi's disease by 5 months. In 4 other patients, antinuclear antibodies (ANA) were positive at titers ranging between 1:80 and 1:2560 and were associated with leukopenia ranging from 1190 to 2800 white blood cells/mm3 when the diagnosis of Kikuchi's disease was made. Among these, 3 patients developed SLE after periods ranging from 6 to 25 months. The other ANA positive patient and the 2 remaining ANA negative patients did not develop any autoimmune disease after 2-A years of follow-up.The differential diagnosis between Kikuchi's disease and lupus lymphadenitis cannot always be clarified histologically. We recommend that patients with necrotizing lymphadenitis, leukopenia, and ANA be closely followed. This should ensure an early diagnosis of development of an autoimmune disorder that will require treatment, rather than always considering these patients to be affected by a benign self-limited disorder like Kikuchi's disease.

Riedel's thyroiditis is a rare condition in which the thyroid gland is replaced by fibrous tissue. Fibrosis in various distant sites is a possible concomitant event. We report a case of Riedel's thyroiditis complicated by mediastinal fibrosis, a tumefactive fibro-inflammatory lesion of the neck and primary hypothyroidism. A review of the literature in which only 8 previous cases of hypoparathyroidism secondary to Riedel's thyroiditis have been recounted concludes the report.

It is well known that sex hormones can regulate the course of systemic lupus erythematosus (SLE). We describe a young woman who had a fatal exacerbation of SLE after controlled ovarian hyperstimulation for induction of ovulation. We recommend careful attention to the immunological status of the infertile women before starting any ovulation inducing treatment for assisted reproduction.

Kikuchi's necrotizing lymphadenitis is a histologically alarming but self-limiting lesion typically affecting the cervical lymph nodes of young women. The authors report 2 cases of systemic lupus erythematosus with the finding of Kikuchi's disease on lymph node biopsy. Considering the common infectious and/or immunologic etiologies described in both disorders, coexistence of both conditions suggests the possibility that they share a common etiology.

Objective: To investigate the efficacy of high-dose intravenous immunoglobulin (IVIg) in the treatment of refractory rheumatoid arthritis (RA)., Methods: Ten patients with active, severe RA that was unresponsive to first- and second-line agents were administered IVIg monthly, for 6 months., Results: Following IVIg treatment, there was significant improvement in both subjective and objective parameters of disease activity in all 9 patients who completed the protocol. This improvement was noted to occur as early as after the second infusion of IVIg. After discontinuation of the treatment, all patients had a relapse of the disease within a few weeks., Conclusion: Since the reduction in clinical activity paralleled a decrease in the CD4+CDw29+:CD4+CD45RA+ cell ratio, some of the therapeutic benefits associated with IVIg may be due to a direct influence on the CD4+CD45RA+ subset. Although the possibility of carrying out further controlled studies on a larger scale is limited by the high cost of the treatment, IVIg appears to be an effective therapy for refractory RA.

A patient with rheumatoid damage of the cervical spine with cervical cord compression, complaining of a typical syringomyelic syndrome is described. Magnetic resonance imaging revealed cord compression due to an upward displacement of the odontoid peg with a mass of pannus behind the dens, and a syrinx cavity extending from C1 to T1. We postulate that this patient's syringomyelia probably represents a complication of cervical rheumatoid arthritis that has not been described.

High intravenous doses (400 mg/kg) of gammaglobulin (IVIG) were administered monthly for six months to 7 patients with severe rheumatoid arthritis (RA). In all cases, previous treatment with NSAIDs and corticosteroids and in 3 of them with gold and/or methotrexate had been ineffective. A 50 per cent improvement of Ritchie index was obtained in 6/7 patients, morning stiffness was reduced from greater than 2 hours to less than 30 minutes in 6/7 patients. Swollen joints and Lee index improved in all patients. ESR did not show any change but RCP improved in 6/7 patients. The study of lymphocyte subpopulation showed no substantial changes in CD20+, CD3+, CD4 and CD8 cells as well as in CD4/CD8 ratio and a significant increase in 2H4+T cells without changes in 4B4+ subpopulation. IVIG improved the clinical and laboratory features of patients with severe RA. The major problem raised by IVIG therapy is its high cost suggesting that this therapy should only be applied in well selected patients with RA.

A case of acute thrombocytopenic purpura, presented with sudden onset of petechiae and ecchymoses, as a possible complication of antinfluenza vaccination with inactivated viruses is reported. Within 15 days of vaccination the platelet count fell down, serum antiplatelet antibodies were detected in high titre and bone marrow aspiration revealed an increased number of megakaryocytes. Corticosteroid therapy was followed by a complete recovery within ten days; three months later, platelets number remained in the normal range.