Liver involvement in adult-onset Still's disease: our experience in a third level liver unit and review of the literature.

BACKGROUND: Adult-onset Still's Disease (AOSD) is a systemic inflammatory condition, mainly characterized by high spiking fevers, leukocytosis, skin rash, arthralgia and myalgia. Liver involvement is a frequent feature, usually presenting with hepatomegaly and mild liver enzymes abnormalities, which usually normalize after treatment with anti-inflammatory or immunomodulatory drugs given for AOSD. Although uncommon, the onset of severe acute hepatitis and even of life-threatening liver failure is possible and requires a prompt diagnosis and an aggressive therapy and, in some cases, an emergency liver transplantation. The differential diagnosis of the cause of the liver injury can be very challenging in these patients. METHODS: We reviewed the charts of all consecutive patients admitted for acute hepatitis, between January 2019 and December 2019, to the unit of Gastroenterology and Hepatology, Molinette Hospital, Turin, Northern Italy, searching for episodes AOSD-related. RESULTS: In this period, 21 cases of acute hepatitis were recorded with one among them diagnosed as due to AOSD. The incidence was 5% (1/21). This patient was a woman with a recent diagnosis of AOSD who developed a severe acute seronegative biopsy-proven autoimmune hepatitis. She was successfully treated with high-dose methylprednisolone, with a full and stable recovery from the liver injury. CONCLUSIONS: We discuss the incidence, etiology, pathophysiology, diagnosis, and standard of treatment in the clinical management of AOSD with a special attention and a systematic review on the available therapies for severe liver involvement associated with AOSD.

Rheumatologic manifestations of hepatitis C in the era of direct-acting antiviral agents.

Beyond the classic hepatic complications, hepatitis C (HCV) infection is considered as a systemic disease, since extrahepatic manifestations become clinically evident in 40% to 70% of the patients and it can frequently include rheumatic ones. Furthermore, HCV can promote the production of several autoantibodies, thus complicating the differential diagnosis between primitive and HCV-related rheumatic disorders. The recent development of direct-acting antivirals (DAA) against HCV has revolutionized the field, reducing the damage stemming from systemic inflammatory phenomena and persistent immune activation associated with continuous HCV replication. Our review focuses on the main rheumatologic manifestations associated with chronic HCV infection as well as the impact of DAA interferon-free treatments on such extrahepatic clinical involvement.

Ultrasound-guided intra-articular injection: efficacy of hyaluronic acid compared to glucocorticoid in the treatment of knee osteoarthritis.

BACKGROUND: Osteoarthritis (OA) is a degenerative joint disease which causes pain and functional impairment in adults over 50 years old with consequent important disability. Unfortunately, there is no definitive cure for OA, thus the approach is characterized by multiple treatments that can manage its symptoms. Even though data from randomized controlled trials and meta-analyses indicate that intra-articular hyaluronic acid (IAHA) offers the best benefit/risk balance among the various pharmacologic treatments to improve OA-related knee pain, there is a lack of agreement among national and international guidelines about such uses of IAHA for the medical management of symptomatic knee OA. To minimize confounding factors and biases, the aim of our study was to evaluate the efficacy of the different weight and concentration of IAHA treatment in patients suffering from knee OA comparing to glucocorticoids (GC) joint injections. Furthermore, to make the procedure more accurate and assessment more objective, we use ultrasonography (US) with power Doppler (PWD) to help us differentiate between active and inactive inflammation within joints and periarticular soft tissues. METHODS: We performed a retrospective evaluation of a cohort of patients with knee OA, diagnosed according to the ACR criteria, treated by US-guided joint injection of HA and GC. The patients were catalogued according to the type of treatment they underwent: group A, patients treated with HA (1.5%) >1500 kDa (three US-guided knee injections one week apart); group B, patients treated with HA (2%) 800-1200 kDa (three US-guided knee injections one week apart); group C, patients treated with glucocorticoids (three US-guided knee injections of triamcinolone acetate 40 mg one week apart). All patients were monitored for 6 months, evaluating: subjective pain using a 10-cm Visual Analogue Scale; pain, stiffness, and functionality using the Western Ontario and McMaster Universities Arthritis Index (WOMAC); the concomitant intake of anti-inflammatory and/or analgesic drugs through a questionnaire; and US results by grey scale and PWD. RESULTS: A total of 171 patients affected by knee OA were evaluated (women 72.3%) with a mean age of 69.3±4.1 years. All the subjects analyzed showed a pain reduction at 6 months after treatment (group A: -39.5; group B: -36.9; group C: -30.8). The difference between the three groups was statistically significant (Kruskall-Wallis P=0.001) and in particular between group A and group C (P=0.000) and between group B and group C (P=0.005), but not between A and B (P=0.258). WOMAC was statistically significantly improved from baseline in all groups examined (group A: -11.9; group B: -14.9; group C: -11.2). The PWD score showed a statistically significant improvement in group B (-0.64) even after 6 months (P=0.004). All patients in the different groups showed a statistically significant reduction of concomitant therapy compared to baseline with respect to paracetamol and non-steroidal anti-inflammatory drugs (NSAIDs)/COX2 therapy, while only group B showed a statistically significant reduction for opioids. CONCLUSIONS: This study demonstrated the efficacy of OA treatment with medium molecular weight HA in favor of the higher concentration of HA that may affect the reduction of pro-inflammatory mediators. Furthermore, US monitoring allowed to evaluate aspects related to synovial involvement, which cannot be appreciated with standard imaging.

The clinical presentation in adulthood of juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is a chronic systemic inflammatory disease, which affects children and adolescents, characterized by significant differences when compared to inflammatory rheumatisms in adulthood. Today, in a panorama enriched in the last decades with great improvements in the diagnostic and therapeutic field, a far from negligible portion and an increasing number of patients with JIA require the continuation of treatments in adulthood. This specific population of patients, given the high incidence of extra-articular manifestations, residual irreversible disabilities, comorbidities related to an inflammatory process and extended immunosuppressive treatments during the age of development, requires precise attentions in the follow-up and a multidisciplinary approach characterized by different clinical, psychological and social aspects.

Serological and clinical profile of systemic sclerosis: analysis in a cohort of patients from a single center in Northern Italy.

BACKGROUND: The purpose of this study was to identify the frequency of autoantibodies among patients affected by systemic sclerosis (SSc) in our Rheumatology Center and analyze the correlation between serological and clinical presentations. METHODS: An automated fluoro-enzyme-immunoassay is used for the qualitative detection of sixteen antibodies: anti-dsDNA, antiRo52, antiRo60, antiSS-B, antiTopoisomerasi-I, antiCENP-B, anti-fibrillarin, antiMi-2, anti-Sm, antiU1sn-RNP, antiRNP70, antiPm/Scl100, antiPCNA, antiJo-1, antiRibosomal-P, antiRNA-Polymerase-III. These parameters were further correlated with clinical presentation of the disease. RESULTS: One-hundred and six patients who fulfilled the ACR classification criteria of SSc have been screened. Similarly to the findings of other studies, a strong association between anti-Centromere antibodies and clinical indicators of better prognosis has been showed; conversely, the anti-Scl70 antibodies are associated with diffuse SSc and higher severity. Some antibodies (antiR052, antiU1RNP) are correlated with a diagnosis of autoimmune overlap. A protective effect of AntiCentromere regarding pulmonary fibrosis and skin ulcers has been shown (P<0.05). The presence of AntiScl70 correlated with cardiac involvement (arrhythmias, pericarditis and myocarditis) and the Anti-U1RNP correlated with the presence of skin ulcers. CONCLUSIONS: The diagnostic importance of SSc antibodies against a variety of nuclear and cytoplasmic antigens has become increasingly recognized, as confirmed by the inclusion into 2013American College of Rheumatology (ACR)/the European League Against Rheumatism clinical classification criteria for SSc. A number of studies reported variable geographic rates of antibody prevalence in SSc, which may be related to either genetic or environmental factors. However, the association of specific antibodies with clinical manifestations continues to be claimed. New testing methods which include a wider spectrum of detectable antibodies may support the daily rheumatological and dermatological clinical practice in defining clinical subsets of disease and provide prognostic information.