Patient-Reported Outcomes in Adult Idiopathic Inflammatory Myopathies Abstract Purpose of Review Idiopathic inflammatory myopathies (IIM) have considerable impact on patient symptoms and quality of life. We have reviewed the evolution of patient-centered care and use of patient-reported outcome measures (PROMs) for adults with IIM. Recent Findings Use of PROMs in myositis care and research is limited, although the importance of incorporation into routine practice and trials has become increasingly recognized. Several key domains/measures have been identified including the patient global assessment of disease activity, physical function as measured by the health assessment questionnaire-disability index (HAQ-DI), Short Form Health Survey-36 (SF-36), or the Patient-Reported Outcome Measurement Information System ® (PROMIS®) in adult IIM. Data are limited for these instruments concerning their reliability, content and construct validity, and responsiveness. Summary Incorporation of the patient perspective into clinical care and research may be used to address the unmet/unaddressed needs of the patient living with myositis. Several ongoing projects aim to bring validated PROMs to the IIM community.

“Mobile Health” for the Management of Spondyloarthritis and Its Application in China Abstract Purpose of the Review Spondyloarthritis (SpA) is a group of inflammatory diseases characterized by inflammation in the spine, peripheral joints, and entheses that usually start at the prime of one’s life and lead to impaired physical function and reduced quality of life. Ankylosing spondylitis (AS) is prototype of SpA. This article reviews the opportunities and challenges of using mobile health (mHealth) in managing SpA, and report some of our experiences using a mHealth solution for management of SpA patients and performing related research in China. Recent Findings The recent rapid development of mobile communications and the common use of intelligent electronic devices have led to the increasing application of mHealth for chronic disease management by healthcare providers and patients alike. This is a promising new technology that can help mitigate limitations in time and space for patient management, promote easier communication between patients and their healthcare providers, reduce medical expenses, and optimize medical services. We have developed a smartphone-based mHealth SpA management system (SpAMS) that also helps the patients to monitor, manage, and share information on their disease with their physician at regular intervals. Summary There is a shift from a paternalistic model of healthcare to more personalized healthcare in which disease management is conducted by the patient together with their healthcare providers. The increasing utility of mHealth is expected to benefit disease management, promote patient–doctor communication, reduce medical expenses, and optimize medical services.

Chlamydia -Induced Reactive Arthritis: Disappearing Entity or Lack of Research? Abstract Purpose of Review Recent studies regarding the frequency of Chlamydia-induced reactive arthritis (ReA) are reviewed, with a focus on the question of whether the entity is in fact disappearing or whether it is simply being underdiagnosed/underreported. Epidemiological reports indicate diversity in the frequency of Chlamydia-associated ReA in various parts of the world, with evidence of declining incidence in some regions. Recent Findings The hypothesis that early effective treatment with antibiotics prevents the manifestation of Chlamydia-associated ReA requires further investigation. Summary For clinicians, it is important to remember that ReA secondary to Lymphogranuloma venereum (LGV) serovars L1–L3 of C. trachomatis is probably underestimated due to a limited awareness of this condition, the re-emergence in Western countries of LGV overall, and the present increasingly rare classical inguinal presentation.

Understanding the Cryoglobulinemias Abstract Purpose of the Review Cryoglobulins are immunoglobulins with the ability to precipitate at temperatures <37 °C. They are related to hematological disorders, infections [especially hepatitis C virus (HCV)], and autoimmune diseases. In this article, the state of the art on Cryoglobulinemic Vasculitis (CV), in a helpful and schematic way, with a special focus on HCV related Mixed Cryoglobulinemia treatment are reviewed. Recent Findings Direct – acting antivirals (DAA) against HCV have emerged as an important key in HCV treatment to related Cryoglobulinemic Vasculitis, and should be kept in mind as the initial treatment in non–severe manifestations. On the other hand, a recent consensus panel has published their recommendations for treatment in severe and life threatening manifestations of Mixed Cryoglobulinemias. Summary HCV-Cryoglobulinemic vasculitis is the most frequent form of CV. There are new treatment options in HCV-CV with DAA, with an important number of patients achieving complete response and sustained virologic response (SVR). In cases of severe forms of CV, treatment with Rituximab and PLEX are options. The lack of data on maintenance therapy could impulse future studies in this setting.

Qigong and Musculoskeletal Pain Abstract Purpose of Review Musculoskeletal pain is a widespread symptom that commonly produces considerable disability, particularly in later life. This brief review strove to summarize and critically review the recent research base concerning the use of Qigong as a possible strategy for alleviating longstanding or chronic musculoskeletal pain states. Methods Research reports and literature reviews specifically focusing on Qigong and its impact on various forms of musculoskeletal pain between 2015 and 2019 were sought and analyzed, along with related data. Results Collectively, these data reveal that while more research is indicated, Qigong practice may help to attenuate pain in varying degrees among adults with different forms of chronic pain with few side-effects. Conclusion More research is needed to ensure health professionals working with adults who have chronic unrelenting musculoskeletal pain may safely recommend these exercises as one possible remedy for reducing intractable musculoskeletal pain.

The Pathogenesis of Ankylosing Spondylitis: an Update Abstract Purpose of Review Ankylosing spondyloarthritis (AS) is a chronic inflammatory disease that involves the axial joints and entheses. Extra-spinal manifestations such as anterior uveitis, psoriasis, and colitis also occur frequently. This review on the pathogenesis of AS includes an update on the recent discoveries within the field. Recent Findings HLA-B*27 is still considered of major importance in the pathogenesis, and it has recently been shown to profoundly affect the gut microbiome and its metabolites and the handling of bacteria during infection. Biochemical and biophysical properties of HLA-B*27 influence its ability to misfold, to induce an endoplasmic reticulum stress response, and to promote autophagy/unfolded protein responses (UPR). HLA-B*27 free heavy chains may induce inflammation through T cells, NK cells, and myeloid cells. Induction of UPR genes results in release of tumor necrosis factor-α (TNF-α), interleukin-17 (IL-17), IL-23, and interferon-γ and increase in T helper (Th) 17 cells. Several other HLA-B and non-B molecules have been associated with AS, although their role in the pathogenesis is unknown. Summary Genotypes of endoplasmic reticulum aminopeptidases (ERAP) 1 and 2 have been associated with alterations in the antigenic pool expressed by HLA-B*27 molecules. In the gut, innate immune cells type 3 (ILC3) influence T cell expression of IL-17 and IL-22. Gamma-delta (γ/δ) T cells are induced by IL-23 to produce IL-17. IL-7 induces mucosa-associated invariant T (MAIT) cells to produce IL-17. Besides the microbiome, zonulin may be important through its effects on the permeability of tight junctions in the intestinal epithelial barrier.

Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome Abstract Purpose of Review Diffuse alveolar hemorrhage (DAH) is a rare but devastating manifestation of antiphospholipid syndrome (APS) patients with or without other systemic autoimmune diseases. Data regarding diagnosis and treatment are limited to case series. We review diagnostic and therapeutic strategies employed in APS patients with DAH and discuss our experience in managing these complex patients. Recent Findings Pulmonary capillaritis likely contributes to the pathogenesis, however is only observed in half of the biopsies. Corticosteroids induce remission in the majority of patients, however almost half recur and require a steroid-sparing immunosuppressive to maintain remission. Cyclophosphamide- or rituximab-based regimens achieve the highest remission rates (50%); other strategies include intravenous immunoglobulin, plasmapheresis, mycophenolate mofetil, and/or azathioprine. Summary Given the rarity of DAH in APS, treatment is guided by interdisciplinary experience. Why certain patients achieve full remission with corticosteroids while others require immunosuppressive agents is unknown; future research should focus on the pathophysiology and optimal management.

Transitional Care in Rheumatology: a Review of the Literature from the Past 5 Years Abstract Purpose of Review Effective transitional care for adolescents and young adults (AYA) with rheumatic musculoskeletal disease (RMD) is fundamental to rheumatology care provision. Here we review the recent evidence from the literature on transition in rheumatology and debate why universal implementation has yet to be recognised. Recent Findings Evidence of need for transitional care continues to be reported. The triphasic nature of transitional care remains poorly recognised, and the third phase following transfer to adult rheumatology is particularly under-researched in spite of the recognition of the age-related trajectories of transition skill development during young adulthood. Several rheumatology-specific transitional care interventions have now been evaluated but the search for valid measures including outcome continues. Finally, the need to study transition at a health system level is increasingly recognised. Summary Future research in this area should consider the developmental trajectories of AYA as well as the social-ecological model of transition readiness, which focuses on the interactions between AYA, caregivers and providers (and the systems they are part of) as these are the likely targets of any intervention to improve health transitions.

Orbital Vasculitides–Differential Diagnosis Abstract Purpose of Review The orbit is subject to a variety of vascular insults that manifest with both specific and nonspecific patterns of vision compromise. The aim of the following review is to highlight the ophthalmic clinical features of systemic vasculitides that most frequently involve the orbit and differentiate them from the most common non-vasculitic orbital disorders. Recent Findings New studies continue to explore the autoimmune nature of vasculitic disease and seek to determine optimal use of newer therapies such as biologic agents. Summary The pattern of ocular involvement in the context of clinical history allows the knowledgeable physician to distill a differential diagnosis into a specific or likely cause. Establishing a diagnosis in a timely fashion allows for a custom-tailored approach to therapy.

Novel Developments in Primary Immunodeficiencies (PID)—a Rheumatological Perspective Abstract Purpose of Review The purpose of this review is to provide an overview of the most relevant new disorders, disease entities, or disease phenotypes of primary immune deficiency disorders (PID) for the interested rheumatologist, using the new phenotypic classification by the IUIS (International Union of Immunological Societies) as practical guide. Recent Findings Newly recognized disorders of immune dysregulation with underlying mutations in genes pertaining to the function of regulatory T cells (e.g., CTLA-4, LRBA, or BACH2) are characterized by multiple autoimmune diseases—mostly autoimmune cytopenia—combined with an increased susceptibility to infections due to hypogammaglobulinemia. On the other hand, new mutations (e.g., in NF-kB1, PI3Kδ, PI3KR1, PKCδ) leading to the clinical picture of CVID (common variable immmune deficiency) have been shown to increasingly associate with autoimmune diseases. Summary The mutual association of autoimmune diseases with PID warrants increased awareness of immunodeficiencies when diagnosing autoimmune diseases with a possible need to initiate appropriate genetic tests.