Idiopathic aortitis: an underrecognized vasculitis

Aortitis is a general term denoting inflammation of the aortic wall. Various infectious and non-infectious diseases can be complicated by aortitis; in addition, isolated idiopathic aortitis has also been described. In a 12-year nationwide Danish population-based study, the prevalence of aortitis among 1,210 resected thoracic aorta samples was 6.1%, with nearly three-quarters of cases being idiopathic. Identified risk factors for aortitis included advanced age, a history of connective tissue disease, diabetes mellitus, and heart valve pathology. As in virtually all pathological studies, this study has a bias toward reporting the most severe cases of aortitis requiring surgical repair

On- and off-label use of rituximab in rheumatic diseases

Steadily growing knowledge about pathogenetic mechanisms in autoimmune rheumatic diseases (RDs) has paved the way to different therapeutic approaches. In particular, the availability of biologics on the market has dramatically modified the natural history of rheumatic chronic inflammatory diseases with a meaningful impact on patients’ quality of life. Among the wide spectrum of available biological treatments, rituximab (RTX), initially used in the treatment of non- Hodgkin’s lymphoma, was later approved for rheumatoid arthritis and anti-neutrophil cytoplasmic antibodies-associated vasculitis. Currently, in rheumatology, RTX is also used with off-label indications in patients with systemic sclerosis, Sjögren’s syndrome and systemic lupus erythematosus. RTX is a monoclonal antibody targeted to CD20 molecules expressed on the surface of pre-B and mature B lymphocytes. It acts by causing apoptosis of these cells with antibody- and complement-dependent cytotoxicity. As inflammatory responses to cell-associated immune complexes are key elements in the pathogenesis of several autoimmune RDs, such an approach might be effective in these patients. In fact, RTX promotes a rapid and long-term depletion of circulating and lymphoid tissue-associated B cells, thus leading to a lower recruitment of these effector cells at sites of immune complex deposition, therefore reducing inflammation and tissue damage. RTX is extremely interesting for rheumatologists, as it represents an important additional therapeutic approach. Therefore, the advent in clinical practice of approved RTX biosimilars, such as CT-P10, may help in improving treatment access as well as reducing cost

Epidemiology of psoriatic arthritis

Epidemiological studies on psoriatic arthritis have long been hampered by the absence of widely accepted classification criteria. The development of the CASPAR (ClASsification criteria for Psoriatic ARthritis) criteria has recently provided the framework for conducting epidemiological studies in psoriatic arthritis using uniform recruitment criteria. However, so far, only a minority of studies have adopted such criteria. In addition to the lack of shared classification criteria, differences in study settings, designs, and ascertainment methods have contributed to yield substantial disparities in the estimates of the incidence (from 3,02 to 23,1 cases per 100,000 people) and prevalence (from 49,1 to 420 cases per 100,000 people) of psoriatic arthritis around the globe. Overall, the available data suggests that the prevalence of psoriasis in the general population is approximately 2-3%, with about a third of patients with psoriasis having arthritis. Therefore, psoriatic arthritis may affect 0,3- 1,0% of the population, a frequency not dissimilar from that of rheumatoid arthritis. Future epidemiological studies should be carried out in larger numbers of patients diagnosed using consistent criteria

Clinical assessment in psoriatic arthritis

Due to the heterogeneous clinical picture, with a possible combination in any individual patient of axial disease, peripheral arthritis, enthesitis and dactylitis, psoriatic arthritis (PsA) is difficult to assess. Validated assessment tools for PsA are lacking. Recently, international study groups have a special interest in developing and validating standardized tools to assess PsA. We will review the existing assessment modalities of PsA focusing on axial disease, peripheral arthritis, enthesitis and dactylitis. Measures of function and disability recommended for PsA will be also reviewed

The management of large vessel vasculitides

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) represent the most common large vessel vasculitides (LVV). An early recognition of these conditions is crucial in order to start a prompt treatment to prevent severe ischemic complications, such as irreversible visual loss in GCA and cardiovascular or cerebrovascular accidents in TAK. Isolated glucocorticoids (GCs) still remain the cornerstone of GCA therapy. However, long-term treatment with GCs is burdened by an important toxicity. Furthermore, relapses are frequent during the follow-up period and relapsing patients have to cope with a longer duration of the GC therapy and a higher cumulative GC dose. On the other hand, TAK treatment usually relies on immunosuppressors in addition to GCs from the beginning. Also, since TAK patients are in general young women with a progressive disease, it is essential to treat this vasculitis with steroidsparing drugs in order to avoid excessive GC exposure. For this reason, efforts have been made to discover new therapeutic options able to reduce the cumulative GC dose that is strictly related to GC-toxicity. In recent years, new advances in the management of LVV have become available and have changed the therapeutic approach to these diseases. The aim of this review is to report new evidence of treatment efficacy and safety in LVV

EULAR guidelines on ANCA-associated vasculitis in the real life

Anti-neutrophil cytoplasmic antibodies-associated vasculitides (AAVs) are a heterogenous group of inflammatory diseases which primarily involve small vessels and include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). They present heterogeneous clinical manifestations, while their diagnosis and management still remain a challenge for clinicians. Nowadays, the treatment is based on two different regimens: the remission-induction treatment and the remission-maintenance treatment. The therapeutic armamentarium has grown over the years, with the aim to lessen adverse effects, improve quality of life of patients and maintain the disease under control. Biological treatments are the future: they act on different pathogenic pathways and may offer in the future a personalized management approach tailored to actual clinical manifestations. The latest guidelines were published in 2015 by the European League Against Rheumatism (EULAR) and still represent the vade mecum for the management of AAVs. In this review, we will focus on the principal strategies to treatAAVs. We discuss the remission-induction therapy and the remission-maintenance therapy; we have also distinguished the management of GPA and MPA from that of EGPA, because of their different clinical picture

Polymyalgia rheumatica: an autoinflammatory disorder?

Polymyalgia rheumatica: an autoinflammatory disorder

Combination Therapy with Nintedanib and Sarilumab for the Management of Rheumatoid Arthritis Related Interstitial Lung Disease

Rheumatoid arthritis (RA) is a chronic, systemic, inflammatory disease characterized by joint and extra-articular involvement. Among them, interstitial lung disease (ILD) is one of the most common and severe extra-articular manifestations, with a negative impact on both therapeutic approach and overall prognosis. ILD can occur at any point of the natural history of RA, sometimes before the appearance of joint involvement. Since no controlled studies are available, the therapeutic approach to RA-ILD is still debated and based on empirical approaches dependent on retrospective studies and case series. Here, we report the case of a 75-year-old patient affected by RA complicated by ILD successfully treated with a combination therapy of an antifibrotic agent, nintedanib, and an inhibitor of IL-6 receptor, sarilumab. We obtained a sustained remission of the joint involvement and, simultaneously, a stabilization of the respiratory symptoms and function, with a good safety profile. To date, this is the first report describing a combination therapy with nintedanib and a disease-modifying antirheumatic drug (DMARD) for the management of RA complicated by ILD. Future prospective studies are needed to better define efficacy and safety of this approach in the treatment of these subjects