A D'Ascanio

Thyroid Cancer in Systemic Lupus Erythematosus: A Case-Control Study

CONTEXT Although advances in treatment have permitted patients with systemic lupus erythematosus (SLE) to live longer, the rates of several types of cancers in these patients appear to be increasing. OBJECTIVE We used a prospective study to investigate the prevalence and features of thyroid cancer in SLE patients. DESIGN AND PATIENTS The prevalence of thyroid cancer in 153 unselected SLE patients was compared with that in two population-based, gender- and age-matched control groups: 1) 459 subjects from an iodine-deficient area (iodine-deficient control) and 2) 459 subjects from an iodine-sufficient area (iodine-sufficient control). Thyroid function was assessed by measuring circulating thyroid hormones and autoantibodies, thyroid ultrasonography, and where necessary, fine-needle aspiration cytology. MAIN OUTCOME AND RESULTS The levels of circulating TSH, and anti-thyroglobulin and anti-thyroperoxidase antibodies were significantly higher in SLE patients (P < 0.001 for all). In addition, patients with SLE also exhibited a higher prevalence of hypothyroidism (P < 0.001). Five cases of papillary thyroid cancer were detected among SLE patients, whereas no cases were observed among iodine-deficient controls (P = 0.001), and only one case was observed among iodine-sufficient controls (P = 0.001). Among SLE patients with confirmed thyroid cancer, 80% showed evidence of thyroid autoimmunity, whereas only 31% of SLE patients without thyroid cancer exhibited evidence of thyroid autoimmunity (P = 0.02). CONCLUSIONS These data suggest that the prevalence of papillary thyroid cancer in SLE patients is higher than in age-matched controls, particularly in patients with thyroid autoimmunity. Consequently, careful thyroid surveillance is recommended during the follow-up of these patients.

HLA antigens in Italian patients with primary Sjögren's syndrome.

Twenty eight Italian patients with primary Sjögrens syndrome were typed for class I and class II alloantigens of the major histocompatibility complex. Patients with Sjögrens syndrome had higher prevalence of DR3 (46.4% v 14% in the control population, p corrected less than 0.02), while similar prevalence was found for DR2 and DRw52 alloantigens. DR3 correlated with DRw52 (p less than 0.0001), anti-Ro(SSA) (p less than 0.0002) and anti-La(SSB) (p less than 0.02) antibodies, and extraglandular manifestations (p less than 0.02). In addition, extraglandular involvement was associated with anti-Ro antibodies (p less than 0.05) and raised gammaglobulins (p less than 0.02). In Italian patients with primary Sjögrens syndrome DR3 is the genetic marker related to this clinical entity and seems to identify a disease subset characterised by autoantibody production and extraglandular manifestations.

Mortality rate and outcome factors in mixed cryoglobulinaemia: the impact of hepatitis C virus

Objectives: Mixed cryoglobulinaemia (MC) is a chronic small-vessel vasculitis. Shortly after the discovery of hepatitis C virus (HCV) in 1989, an association between HCV infection and MC was being increasingly reported, suggesting the potential pathogenetic implication of HCV in most of the cases that had been previously diagnosed as essential MC. A number of studies have pointed out prognostic factors linked to mortality in this disorder. None of them, however, have clarified the impact of HCV discovery on the natural history of the disease. The aim of the present study was to evaluate mortality in MC after the discovery of HCV infection. Methods: We retrospectively collected clinical and serological data in 70 unselected HCV-positive patients being followed up at our unit from 1990. Clinical and prognostic factors linked to poor outcome were evaluated. Results: Chronic hepatitis, renal involvement, and intestinal vasculitis were the most frequent causes of death. Conclusion: Compared to other series, the outcome in our MC seemed to be better. Factors linked to a poor outcome were renal involvement, widespread vasculitis, male sex, and cryocrit.

Thigh magnetic resonance imaging for the evaluation of disease activity in patients with idiopathic inflammatory myopathies followed in a single center.

Introduction: In patients with idiopathic inflammatory myopathies (IIM), magnetic resonance imaging (MRI) has been proposed as a useful tool for diagnosis and follow‐up. It may identify muscle inflammation (edema) and fatty infiltration for evaluation of disease activity and damage. Little information is available on the role of MRI in assessment of large cohorts of adult patients with IIM. Methods: Fifty‐one patients underwent MRI of the thigh muscles, laboratory tests, and clinical evaluation, including Physician Global Assessment (PGA) of myositis activity and the Manual Muscle Test 8 (MMT8). Results: Muscle edema correlated significantly with creatine kinase values (P = 0.017) and PGA (P < 0.001). A significant correlation between edema and MMT8 values (P = 0.025) was observed when patients with muscle fatty infiltration were excluded. With respect to clinical diagnosis, the sensitivity of MRI was 92.3%, and specificity was 83.3%. Conclusions: MRI appears to provide additional information that complements clinical and biochemical examinations. Muscle Nerve 54: 666–672, 2016

When the heart is burning: Amino-terminal pro-brain natriuretic peptide as an early marker of cardiac involvement in active autoimmune rheumatic disease

BACKGROUND We evaluated the influence of inflammation on cardiac endocrine function in autoimmune rheumatic disease (RD) patients with preserved left ventricular systolic function. METHODS 160 consecutive RD patients (29 males, age 55 ± 14 years, left ventricular ejection fraction, LVEF, 63 ± 5%: inflammatory polyarthritis: 13%, systemic sclerosis: 25%, connective tissue diseases: 39%, systemic vasculitides: 23%) and 120 healthy controls (24 males, 55 ± 10 years) underwent clinical, echocardiographic evaluation and blood sampling for erythrocyte sedimentation rate, C-reactive protein (CRP), fibrinogen and plasma NT-proBNP. RESULTS A significant correlation was found between plasma NT-proBNP and inflammatory markers (all p<0.001), with CRP and diastolic dysfunction being the only independent predictors of NT-proBNP level. RD patients with active disease (57%) showed higher values of inflammatory markers and NT-proBNP (all p<0.01). Patients with subclinical cardiac involvement (Stage B by ACC/AHA HF-classification) had higher NT-proBNP (p<0.001) than controls and patients only at risk for HF (Stage A). NT-proBNP showed a significant diagnostic accuracy in discriminating stage B (n=93) versus stage A patients (n=67, AUC=0.755 ± 0.038, p<0.001) and controls (AUC=0.834 ± 0.030, p<0.001). CONCLUSION Higher CRP and the presence of left ventricular diastolic dysfunction were independently associated with higher NT-proBNP. NT-proBNP might be used in RD as a marker of both disease activity and subclinical cardiac involvement.

Oral sildenafil in skin ulcers secondary to systemic sclerosis

Digital ulcers (DUs) are a major clinical problem in scleroderma patients, associated with reduced quality of life, pain, and disability, and resulting in loss of productivity and mutilation that c...

Occurrence of organ-specific and systemic autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases: report of data obtained through direct patient interviews.

Studies have demonstrated a familial aggregation of systemic and organ-specific autoimmune diseases. The aim of the present survey was to obtain, by patient interviews, a preliminary estimate of the prevalence of systemic and organ-specific autoimmune diseases among the first- and second-degree relatives of Caucasian patients with connective tissue diseases (CTD) or inflammatory arthritis followed at our unit. Between June 2007 and January 2008, 626 patients and 85 controls (patients with osteoarthritis, osteoporosis, or fibromyalgia) were interviewed. Three hundred ten patients (50%) versus 21 controls (25%) were found to have at least one relative affected with an autoimmune condition (p < 0.0001). The most common conditions were organ-specific autoimmune diseases: 160 (34%) autoimmune thyroid (AT) disease, 112 (24%) psoriasis, 21 vitiligo, and 19 insulin-dependent diabetes mellitus. Systemic autoimmune diseases were reported in 126 relatives: rheumatoid arthritis (66 cases, 14%), 16 sacroileitis, and CTD (43 cases). A significant difference was observed in the prevalence of AT disease between the relatives of the patients and controls (3% versus 0.5%). In conclusion, these data confirm the high prevalence of autoimmune conditions, particularly of AT disease, among the relatives of patients.

Long-term cyclic intravenous iloprost in systemic sclerosis: clinical experience from a single center

The aim of the present study was to retrospectively evaluate response to therapy in 73 patients affected by systemic sclerosis (SSc) who underwent long-term cyclic treatment with intravenous iloprost for peripheral vascular involvement (average duration of treatment 54.12±41.04 months). Seventy-three SSc patients were enrolled. Data were collected by reviewing clinical records and by phone or direct interview. Patients underwent a thorough physical examination at the end of follow up. The incidence of severe vascular manifestations was also assessed. Statistical analysis was performed by Wilcoxons signed rank test and descriptive statistics using Statview software. In this study cohort, 55 of 73 (75.2%) patients had a history of ischemic digital ulcers (DUs); 28 patients (38.4%) had active DUs at the beginning of treatment. Skin ulcers healed completely in 25 of 28 patients (89.3%) at the end of the first treatment. However, 40 of 55 patients (72.6%) relapsed after an average of 24 months. There was a significant correlation between relapse rate and/or number of ulcers and clinical factors (diffuse subset, changes in results of Allens test, NT-pro BNP levels). The annual incidence of pulmonary arterial hypertension (PAH) was 2.34 (95%CI: 0.94-4.83) per 100 person years, the rate of gangrene was 2.7%, and no cases of scleroderma renal crisis were recorded. The incidence of PAH and of digital gangrene was higher than that observed in unselected SSc case series. These data suggest that our patients treated with iloprost have a higher vascular involvement than large case series of unselected SSc patients. A number of clinical factors are correlated to the severity of vascular involvement and could have an impact on the response to therapy. The clinical significance of these findings requires clarification and further investigation is needed.

Functional and isokinetic assessment of muscle strength in patients with idiopathic inflammatory myopathies

Objective: To assess muscle strength in patients with idiopathic inflammatory myopathies (IIM) using neuromuscular scales and isokinetic testing. Methods: Muscle function was evaluated in 27 IIM patients being followed at the Rheumatology Unit of the University of Pisa using: (i) a modified version of the grading system used to assess Duchenne dystrophy, (ii) the four-stage grading system of Henriksson and Sandstedt, (iii) an isokinetic muscle strength test (Kin Com, Chatanooga) and (iv) the Health Assessment Questionnaire (HAQ). Results: The neuromuscular scales showed normal or only mildly impaired muscle strength in 60% (Duchenne scale) and 80% (Henriksson and Sandstedt scale) of the patients, respectively, whereas isokinetic testing detected moderate to severe reductions in muscle strength in almost 70% of the patients. No correlations were observed between muscle strength and disease activity, therapy, age at evaluation and disease duration. There was a correlation between the results of the HAQ and neuromuscular testing, but not the isokinetic test. Conclusions: Although less easy and more expensive to administer, isokinetic testing appears to be a more sensitive instrument than the standard neuromuscular tests for assessing muscle function in IIM patients. In particular, it can detect small reductions in muscle strength.

The Clinical Relevance of Antinuclear Antibodies in Connective Tissue Diseases

Detection of antinuclear antibodies (ANA) is of growing relevance in the management of connective tissue diseases (CTD). ANA are useful diagnostic tools, since most CTD have a peculiar ANA profile, characterized either by restricted specificities, abnormally high titers, or both. Furthermore, the study of the intimate structure and function of nuclear antigens, and of their corresponding antibodies may provide important insights to understand both origin and pathogenesis of CTD. Finally, the more or less close association of ANA levels and/or specificity with certain signs or symptoms of CTD is of increasing help to the clinician in the correct monitoring and management of CTD.