What's new in rheumatology

The following represent additions to UpToDate from the past six months that were considered by the editors and authors to be of particular interest. The most recent What's New entries are at the top of each subsection.

NEUROMUSCULAR DISEASE

Trial of intravenous immune globulin for dermatomyositis (November 2022)

Intravenous immune globulin (IVIG) has been used in the treatment of dermatomyositis (DM) based on limited observational data and small randomized trials. In a new randomized trial of 95 patients with DM, the percentage of patients who achieved a response of at least minimal improvement based on a composite score of disease activity was higher among those who received IVIG compared with placebo (79 versus 44 percent) at 16 weeks [1]. IVIG was associated with more adverse events, including thromboembolic events. Based on these results, the US Food and Drug Administration approved IVIG for the treatment of adults with dermatomyositis. More data are needed to help determine the potential role of IVIG as first-line therapy for patients with DM. (See "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults", section on 'Intravenous immune globulin'.)

PEDIATRIC RHEUMATOLOGY

New NF-kB-mediated autoinflammatory disease (ROSAH syndrome) (November 2022)

Retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and headache (ROSAH) syndrome is an autosomal dominant disorder caused by gain-of-function heterozygous missense variants in the alpha kinase 1 gene (ALPK1). A recent study confirmed that ROSAH is an autoinflammatory disorder mediated by enhanced nuclear factor kappa B (NF-kB) signaling [2]. In this small case series, immunomodulation with anticytokine therapy was associated with improved autoinflammatory disease manifestations including fatigue, headache, and arthralgia, and also intraocular inflammation in those who did not have advanced retinal disease. Additional studies are needed to determine the best choice of therapy and whether earlier treatment can prevent development of disease manifestations. (See "Autoinflammatory diseases mediated by NFkB and/or aberrant TNF activity", section on 'ALPK1 gain-of-function defects (ROSAH syndrome)'.)

RHEUMATOID ARTHRITIS

Phase 3 trial of olokizumab for rheumatoid arthritis (September 2022)

Olokizumab is an investigational monoclonal antibody that targets the interleukin-6 cytokine directly. A recent phase 3 trial compared olokizumab (dosed every two or four weeks), adalimumab (a tumor necrosis factor inhibitor), and placebo in 464 patients with rheumatoid arthritis (RA) receiving treatment with methotrexate [3]. At 12 weeks, the proportion of patients who achieved an American College of Rheumatology 20 response (≥20 percent fewer tender and swollen joints and ≥20 percent improvement in three of five other domains) was greater for olokizumab versus placebo and similar for olokizumab versus adalimumab. The incidence of serious adverse events was similar across the three groups. Larger and longer-term trials are needed to further evaluate the efficacy and safety of olokizumab in the treatment of RA. (See "Interleukin 6 inhibitors: Biology, principles of use, and adverse effects", section on 'Structure and mechanism of action of specific agents'.)

SPONDYLOARTHRITIS AND PSORIATIC ARTHRITIS

Pregnancy outcomes among females with axial spondyloarthritis (March 2023)

Data on pregnancy outcomes in patients with axial spondyloarthritis (axSpA) have been mixed. In a nationwide cohort study in Sweden that included 1580 births in females with axSpA between 2007 and 2020, axSpA was not associated with an increased risk of preterm birth, pre-eclampsia, elective caesarean delivery, or serious infant infection [4]. In a separate European study of pregnancy outcomes in 304 females with axSpA starting in 2015, rates of preeclampsia, preterm birth, and low birth weight were either comparable or less than in the general European population [5]. Whether these improved outcomes can be attributed to more widespread use of tumor necrosis factor inhibitors is not clear, but both studies imply that patients with axSpA may not be at increased risk of pregnancy complications. (See "Clinical manifestations of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Pregnancy outcomes'.)

Prevalence of ankylosing spondylitis among United States military personnel (February 2023)

Traditionally, ankylosing spondylitis (AS) is thought to be more common among males, although this may depend on how the disease is identified and defined. A cohort study of over 728,000 United States military personnel demonstrated that males and females had an approximately equal incidence of AS (27 per 100,000 person-years each) [6]. The higher overall incidence of AS in this study may be partially explained by the use of clinical, rather than radiographic, criteria to diagnose AS. This study highlights the importance of evaluating patients with back pain for AS, regardless of sex. (See "Clinical manifestations of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Epidemiology'.)

SYSTEMIC LUPUS ERYTHEMATOSUS AND SJOGREN'S SYNDROME

Choice of antihypertensive agent and cardiovascular risk in patients with lupus (February 2023)

Cardiovascular disease is the leading cause of death among patients with systemic lupus erythematosus (SLE). In a recent observational study of over 220,000 patients with SLE, use of antihypertensive drugs that act on the renin-angiotensin system (RAS) was associated with a 20 percent lower relative risk of incident cardiovascular disease compared with other antihypertensive treatments [7]. Use of RAS-modifying therapies was also associated with increased probability of remaining free of cardiovascular disease over a five-year period (86 versus 78 percent). These findings favor the selection of angiotensin receptor blockers and angiotensin-converting enzyme inhibitors for the management of hypertension in patients with SLE. (See "Coronary heart disease in systemic lupus erythematosus", section on 'Control of hypertension'.)

VASCULITIS

Cardiovascular risk in patients with granulomatosis with polyangiitis and microscopic polyangiitis (February 2023)

Patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) have an increased risk of cardiovascular events, although data on long-term outcomes are limited. In a large cohort study that examined cardiovascular events among over 2300 patients with GPA or MPA and nearly 7000 control patients over a median of 9.5 years, a diagnosis of GPA or MPA was associated with an increased risk of ischemic heart disease, heart failure, myocardial infarction, atrial fibrillation, ventricular arrhythmia/defibrillator implantation, ischemic stroke, percutaneous coronary intervention, and in-hospital cardiac arrest [8]. These findings highlight the importance of monitoring and preventing cardiovascular disease in this patient population. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and maintenance therapy", section on 'Prognosis and other outcomes'.)

Role of tocilizumab in patients with polymyalgia rheumatica (October 2022)

While relatively low doses of glucocorticoids are the primary treatment for polymyalgia rheumatica (PMR), interest remains in identifying an effective steroid-sparing agent. In a randomized trial of 100 patients with steroid-dependent PMR, patients who received adjunctive intravenous tocilizumab were more likely to achieve a combined endpoint of lower disease activity score and reduced steroid requirement at 24 weeks (67 versus 31 percent) [9]. Infections were the most frequent adverse events, occurring in 47 and 39 percent of the tocilizumab and placebo groups, respectively. Additional data are needed to confirm these findings and determine the benefits and safety of adjunctive tocilizumab use for PMR. The routine use of steroid-sparing therapies, including adjunctive tocilizumab, is not recommended for patients with PMR. (See "Treatment of polymyalgia rheumatica", section on 'Limited role for glucocorticoid-sparing therapies'.)

OTHER RHEUMATOLOGY

CDC updates opioid prescribing guidelines (November 2022)

The United States Centers for Disease Control and Prevention (CDC) has published a new guideline for prescribing opioids for acute, subacute, and chronic pain, updating their 2016 guideline (table 1). The guideline is intended for clinicians who prescribe opioids to outpatients ≥18 years of age and does not apply to pain related to sickle cell disease, cancer, palliative care, or end of life care [10]. (See "Use of opioids in the management of chronic non-cancer pain", section on 'Opioid therapy in the context of the opioid epidemic'.)

Perioperative management of medications for systemic rheumatic diseases around elective total hip or knee arthroplasty (September 2022)

The American College of Rheumatology and the American Association of Hip and Knee Surgeons have recently updated their recommendations for the perioperative management of medications for patients with systemic rheumatic diseases undergoing elective total hip or knee arthroplasty [11,12]. The guidelines include newer immunosuppressive agents as well as updated recommendations regarding when to continue, withhold, and restart these medications. Our approach to perioperative medication management is generally consistent with these guidelines. (See "Preoperative evaluation and perioperative management of patients with rheumatic diseases", section on 'Medication management'.)