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

Intravenous immune globulin for HMGCR immune-mediated necrotizing myopathy (February 2025)

The optimal treatment for immune-mediated necrotizing myopathy (IMNM) associated with antibodies against 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) is unknown. In a retrospective study of 53 patients with HMGCR IMNM, patients treated with intravenous immune globulin (IVIG) for six months were more likely to have normalization of creatinine kinase than patients treated with other agents (odds ratio 9.4) [1]. While more patients treated with IVIG regained normal or near-normal muscle strength at six months compared to patients treated with other agents (75 versus 62 percent), the difference between the two groups was not statistically significant. While these results are promising, the role of IVIG for the treatment of HMGCR IMNM requires further study. (See "Treatment of immune-mediated necrotizing myopathy", section on 'Anti-HMGCR-positive'.)

Causes and presentation of vasculitic myopathy (January 2025)

Myopathy can be associated with rheumatic diseases other than polymyositis or dermatomyositis. A recent retrospective review identified 25 patients with vasculitic myopathy, which was most commonly associated with granulomatosis with polyangiitis, polyarteritis nodosa, and rheumatoid vasculitis [2]. Myopathy was part of the patient's initial vasculitis presentation in 80 percent of cases, usually manifesting as progressive weakness predominantly affecting the proximal muscles. This study suggests that vasculitic myopathy should be considered in patients evaluated for an inflammatory idiopathic myopathy, particularly when the patient presents with evidence of systemic inflammation. (See "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults", section on 'Differential diagnosis'.)

Dazukibart for dermatomyositis (January 2025)

Dazukibart is an investigational monoclonal antibody directed against interferon-beta, which has been implicated in the pathogenesis of dermatomyositis. In a randomized trial of 75 patients with active skin-predominant or muscle-predominant dermatomyositis, patients assigned to monthly treatments with intravenous dazukibart (either 150 or 600 mg monthly) had a greater reduction in skin disease after 12 weeks of therapy compared with those assigned to placebo [3]. In an analysis of the muscle-predominant dermatomyositis group, dazukibart at the higher dose was also associated with greater improvements in several muscle parameters, including serum creatine kinase, muscle strength, and patient global assessment, compared with patients treated with placebo. These results imply that interferon-beta blockade may be a new option for patients with dermatomyositis. (See "Initial treatment of dermatomyositis and polymyositis in adults", section on 'Rationale and selection of DMARD'.)

ORTHOPEDICS, SPINE, AND SOFT TISSUE RHEUMATIC DISORDERS

Diabetic neuroarthropathy risk factors (January 2025)

Risk factors for diabetic neuroarthropathy (ie, Charcot foot) include repeated trauma, foot ulceration, and infection or surgery of the affected foot. A retrospective study of 3400 patients with diabetic neuroarthropathy (and 27,000 patients with diabetes alone) identified additional risk factors for diabetic neuroarthropathy, including atherosclerosis, macroalbuminuria, microalbuminuria, and retinopathy, both in patients with type 1 and type 2 diabetes [4]. These data suggest that patients with atherosclerosis and/or microvascular complications of diabetes mellitus may benefit from screening for diabetic neuroarthropathy. (See "Diabetic neuroarthropathy", section on 'Epidemiology and risk factors'.)

PEDIATRIC RHEUMATOLOGY

Withdrawal of adalimumab in patients with uveitis related to juvenile idiopathic arthritis (April 2025)

Chronic anterior uveitis (CAU) is a common comorbidity for patients with juvenile idiopathic arthritis (JIA); however, the optimal approach to tapering therapy after sustained remission is unclear. A trial of 87 patients with JIA-related uveitis in sustained remission on adalimumab randomly assigned patients to continue adalimumab or switch to placebo [5]. While treatment failure during the two-year trial was more common among patients switched to placebo (68 versus 14 percent), these patients were able to re-establish sustained disease control with re-initiation of adalimumab after a median of 105 days. This trial supports the approach to tapering therapy after a period of sustained remission in patients with CAU and JIA. (See "Juvenile idiopathic arthritis: Immunizations and complications", section on 'Subsequent monitoring and duration of therapy'.)

TNF inhibitors for temporomandibular joint arthritis in juvenile idiopathic arthritis (April 2025)

Temporomandibular joint (TMJ) arthritis is a frequent manifestation of juvenile idiopathic arthritis (JIA) that can cause significant pain and dysfunction; how to manage such patients, however, has been less clear. A recent prospective study of 18 patients with JIA-related TMJ arthritis evaluated the efficacy of combining either methotrexate or leflunomide with a tumor necrosis factor (TNF) inhibitor [6]. Over 24 months of follow-up, there were improvements in TMJ function and symptoms of pain and morning stiffness, and 17 patients (47 percent) had stable or improved deformity scores as determined by repeat clinical evaluation and magnetic resonance imaging (MRI). While randomized trials are needed, this suggests that combining a TNF inhibitor with methotrexate or leflunomide can be an effective treatment strategy for TMJ arthritis in JIA. (See "Juvenile idiopathic arthritis: Immunizations and complications", section on 'Treatment and prognosis'.)

RHEUMATOID ARTHRITIS

Risk of rheumatoid arthritis in patients with arthralgias (April 2025)

Patients presenting with arthralgias and seropositivity (ie, the presence of anti-citrullinated protein antibodies (ACPA) and/or rheumatoid factor (RF) are presumed to be at risk of progression to rheumatoid arthritis (RA), but the magnitude of risk has been unclear. In a prospective cohort of over 600 seropositive patients presenting with arthralgias, approximately one-third of patients were diagnosed with RA after a mean follow-up of approximately four years [7]. Risk factors for progression to RA included morning stiffness, high titer ACPA, double-positivity for ACPA and RF, and having a first-degree relative with RA. Patients with arthralgias who have these characteristics should be monitored closely for the development of RA. (See "Undifferentiated inflammatory arthritis in adults", section on 'Prognosis'.)

Kidney function and rheumatoid arthritis disease activity (February 2025)

The impact of rheumatoid arthritis (RA) disease activity on estimated glomerular filtration rate (eGFR) has been unknown. In an observational study of over 31,000 patients with RA, those with mild disease activity had an additional decline in their annual eGFR of 0.1 mL/min/1.73 m² compared with patients with RA in remission [8]. Patients with moderate or severe disease activity had an additional annual eGFR decline of 0.2 mL/min/1.73 m². This study implies that tight control of disease activity may help preserve kidney function among patients with RA, but further studies are needed. (See "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Kidney disease'.)

Sustained remission in rheumatoid arthritis (February 2025)

Patients with rheumatoid arthritis (RA) who stop taking disease-modifying anti-rheumatic drugs (DMARDs) are at increased risk of relapse; however, whether all patients with RA are at equal risk is unclear. In a study of over 1000 patients with early RA from two prospective cohort studies, none of the patients who required treatment with a biologic DMARD were able to remain in a sustained remission following cessation of DMARD therapy [9]. By contrast, 15 to 37 percent of patients who were treated only with conventional immunosuppression (eg, methotrexate) were able to achieve a DMARD-free sustained remission. This study implies that it may be possible to stop DMARD therapy cautiously in selected patients with RA. (See "Treatment of rheumatoid arthritis in adults resistant to initial biologic DMARD therapy".)

SCLERODERMA AND OTHER SYSTEMIC RHEUMATIC DISEASES

Gastrointestinal manifestations of scleroderma (January 2025)

Although systemic sclerosis (SSc) is commonly associated with cutaneous and cardiopulmonary manifestations, gastrointestinal (GI) manifestations of SSc are equally important. Among 907 patients from the Australian Scleroderma Cohort Study, 789 patients (87 percent) reported GI symptoms, including moderate to severe levels of reflux, abdominal bloating, diarrhea, and constipation [10]. Compared with patients with mild GI symptoms, patients with more severe symptoms had worse quality of life, physical function, energy, and mental health. Severe GI manifestations were also linked to higher unemployment rates but not increased mortality. These findings underscore the need for proactive management of GI symptoms in patients with SSc. (See "Gastrointestinal manifestations of systemic sclerosis (scleroderma)", section on 'Epidemiology'.)

SPONDYLOARTHRITIS AND PSORIATIC ARTHRITIS

Ixekizumab for axial spondyloarthritis (May 2025)

Interleukin-17 (IL-17) inhibitors are effective at improving the symptoms associated with axial spondyloarthritis (axSpA), but whether they prevent structural joint damage has been unclear. In this post-hoc analysis of a trial of over 300 patients with axial spondyloarthritis, patients assigned to either ixekizumab (an IL-17 inhibitor) or adalimumab (a tumor necrosis factor inhibitor) had a reduction in structural lesions in the sacroiliac joints after 16 weeks of therapy compared with placebo [11]. From 16 to 52 weeks, all patients received ixekizumab; those assigned to every-two-weeks administration had greater improvements in structural damage than those treated every four weeks. Patients who transitioned from adalimumab to ixekizumab at week 16 had further improvements in structural damage by week 52. These findings suggest that treatment with ixekizumab leads to both clinical and structural improvement among patients with axSpA; whether other IL-17 inhibitors are also effective at preventing structural damage warrants further study. (See "Treatment of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Efficacy of IL-17 inhibitors'.)

Sex differences in ankylosing spondyloarthritis (April 2025)

Whether sex impacts outcomes among patients with ankylosing spondylitis (AS) has been unclear. A meta-analysis of 22 trials including 7331 patients with AS demonstrated that male patients were more likely to respond to either biologic or targeted synthetic disease-modifying antirheumatic drugs than female patients (odds ratio [OR] 1.88) [12]. This same observation was made when the analysis was restricted to tumor necrosis factor inhibitors (OR 2.42) or interleukin-17A inhibitors (OR 1.82). These findings may reflect that, compared with male patients with AS, female patients have longer delays in diagnosis, higher pain scores, and more peripheral arthritis, all of which correlate with worse treatment outcomes. (See "Treatment of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Prognostic indicators'.)

Bimekizumab for axial spondyloarthritis (January 2025)

Bimekizumab is a monoclonal antibody that selectively neutralizes both interleukin-17A and 17F. Previously, two randomized trials in 500 patients with axial spondyloarthropathy (axSpA) demonstrated that bimekizumab improved a composite endpoint (ie, the Assessment in SpondyloArthritis International Society 40%; ASAS40), but the meaning of this endpoint for an individual patient with axSpA has been less clear. A subsequent analysis of patient-reported outcomes data found that, at week 16, bimekizumab reduced spinal pain, morning stiffness, and fatigue compared with placebo [13]. These benefits were sustained through week 52, with over half of the patients achieving a clinically meaningful improvement in fatigue. Whether bimekizumab is more effective for axSpA than other biologics that inhibit interleukin-17A alone requires further study. (See "Treatment of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Efficacy of IL-17 inhibitors'.)

Axial spondyloarthritis in patients with psoriasis, uveitis, or inflammatory bowel disease (January 2025)

Axial spondylarthritis (axSpA) often goes unrecognized in patients with chronic back pain who also have psoriasis, acute anterior uveitis, or inflammatory bowel disease. In two prospective cohort studies of over 350 such patients with undiagnosed chronic back pain, axSpA was present in approximately 25 percent of patients with psoriasis or inflammatory bowel disease and 60 percent of patients with acute anterior uveitis [14]. However, musculoskeletal symptoms, response to nonsteroidal anti-inflammatory drugs, and family history (which are commonly used to identify axSpA) did not help discriminate axSpA from other causes of chronic back pain. This study implies that axSpA is common in patients with psoriasis, acute anterior uveitis, or inflammatory bowel disease, but may be challenging for a nonexpert to distinguish from other causes of chronic back pain. (See "Diagnosis and differential diagnosis of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'History'.)

Ultrasound imaging of enthesitis (January 2025)

Enthesitis (the inflammation of tendon or ligament attachment sites) is a hallmark of spondyloarthritis (SpA), but its identification can be challenging. In a cross-sectional study involving 413 patients with SpA, ultrasound of the entheses demonstrated no evidence of active inflammation in 68 percent of patients diagnosed clinically with enthesitis, while ultrasound identified subclinical enthesitis in 15 percent of patients [15]. Ultrasound may be useful for identifying subtle evidence of enthesitis and differentiating active enthesitis from inactive disease. (See "Diagnosis and differential diagnosis of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'Imaging of enthesitis'.)

SYSTEMIC LUPUS ERYTHEMATOSUS AND SJÖGREN'S DISEASE

Ianalumab for Sjogren's disease (April 2025)

Patients with Sjögren's disease (SjD) have limited treatment options. In this trial of 190 patients with SjD, patients randomized to subcutaneous ianalumab (an investigational anti-B-cell activating factor receptor monoclonal antibody) 300 mg monthly for one year had sustained improvement in disease activity and stimulated salivary flow rates [16]. Three patients had delayed neutropenia (not linked to infection) during the six months following the last ianalumab administration. These results support ianalumab 300 mg as a promising treatment for Sjögren's disease, but these results need to be validated by other clinical trials. (See "Treatment of Sjögren's disease: Constitutional and non-sicca organ-based manifestations", section on 'Other agents in clinical trials'.)

VASCULITIS

Large vessel vasculitis in patients with inflammatory bowel disease (April 2025)

The overlap between inflammatory bowel disease (IBD) and large vessel vasculitis (LVV) has been poorly characterized. In this multicenter case-control study of 196 patients, Crohn disease was more common among patients with Takayasu arteritis (TAK-IBD, 67 percent), while ulcerative colitis predominated in those with giant cell arteritis (GCA-IBD, 58 percent) [17]. LVV most commonly developed after the patient had already been diagnosed with IBD (TAK-IBD: 56 percent; GCA-IBD: 75 percent). LVV should be considered in patients with IBD who develop evidence of systemic inflammation or vascular compromise (eg, aortic regurgitation, asymmetric pulses, or stroke). (See "Overview of gastrointestinal manifestations of vasculitis", section on 'Co-occurrence with inflammatory bowel disease'.)

Upadacitinib for giant cell arteritis (April 2025)

Tocilizumab, an anti-interleukin 6 antibody, is the only biologic agent currently used to treat giant cell arteritis. In a randomized trial of 209 patients with either newly diagnosed or relapsing giant cell arteritis, the Janus kinase (JAK) inhibitor upadacitinib (15 mg, administered orally, once daily) plus a 26-week glucocorticoid taper was superior to a 52-week glucocorticoid taper at achieving sustained remission (46.4 versus 29.0 percent) [18]. Upadacitinib also reduced flare rates and glucocorticoid exposure without increasing serious adverse events. JAK inhibitors have been associated with an increased risk of malignancy, venous thromboembolic events, and cardiovascular events in older adults. While an increased risk of these events was not seen in this study among patients assigned to upadacitinib, long-term follow-up studies are required. We consider upadacitinib as an option for patients who require a steroid-sparing agent but cannot use tocilizumab due to comorbidities such as diverticulitis. (See "Treatment of giant cell arteritis", section on 'Upadacitinib'.)

Giant cell arteritis in patients initially diagnosed with polymyalgia rheumatica (April 2025)

Polymyalgia rheumatica (PMR) may be an isolated diagnosis or a feature of giant cell arteritis (GCA); however, the frequency with which patients with isolated PMR are eventually diagnosed as having GCA has been unclear. In a prospective cohort study of 62 patients with PMR, 3 percent of patients had radiologic evidence of subclinical GCA and another 3 percent developed late-onset GCA during the following year [19]. Although the risk is relatively low, GCA should be considered in all patients presenting with PMR. At presentation and at each follow-up visit, we assess for symptoms or physical findings referable to GCA (eg, new-onset headache, visual impairment, jaw pain with mastication) and pursue large-vessel imaging as indicated by symptoms. (See "Clinical manifestations and diagnosis of polymyalgia rheumatica", section on 'Association with GCA'.)

Prevalence of eosinophilic granulomatosis with polyangiitis in patients presenting with severe asthma (March 2025)

Patients presenting with difficult-to-control asthma require careful clinical assessment to determine optimal therapy and rule out other disease processes. This is well illustrated by a recent study of 596 patients presenting with severe asthma, of whom nearly 4 percent were found to have eosinophilic granulomatosis with polyangiitis (EGPA) [20]. Nearly all patients identified with EGPA had a peak blood eosinophil count greater than 1000 cells/microL, and the most frequent additional disease manifestations were upper airway disease, neuropathy, and kidney involvement. Our authors support carefully assessing all severe asthma patients for hypereosinophilia and extrapulmonary disease. (See "Evaluation of severe asthma in adolescents and adults", section on 'Assessing conditions that mimic asthma'.)

Ischemic events in giant cell arteritis (February 2025)

Which patients with giant cell arteritis (GCA) may be at highest risk for cranial ischemic events (CIC) is not clear. In a multicenter consortium database study of over 1,900 patients with GCA, 17 percent had CIC (eg, blindness, stroke) [21]. Risk factors for CIC included age at diagnosis and hypertension, whereas anticoagulation (but not antiplatelet agents such as aspirin) was associated with lower risk. This study suggests that traditional cardiovascular risk factors may be associated with CIC in patients with GCA, but whether anticoagulation is protective requires further study and prospective validation. (See "Clinical manifestations of giant cell arteritis", section on 'Risk factors'.)

Subclinical vasculitis in polymyalgia rheumatica (January 2025)

Imaging studies may detect subclinical aortitis in patients with polymyalgia rheumatica (PMR), but the clinical significance of this finding is not clear. In a retrospective study of 337 patients with PMR, patients with subclinical vasculitis did not have a higher relapse rate or require longer courses of glucocorticoids than patients with PMR alone [22]. However, patients with subclinical vasculitis received higher starting doses of glucocorticoids than patients with PMR alone (24 versus 15 mg), likely due to an increased frequency of inflammatory symptoms (ie, constitutional symptoms, weight loss, neck pain). We treat patients with PMR based on their clinical presentation (rather than imaging findings) and do not recommend routine imaging, although this is an area of active research. (See "Treatment of polymyalgia rheumatica", section on 'Assessment for GCA'.)

Antibody-negative granulomatosis with polyangiitis (January 2025)

Some patients with granulomatosis with polyangiitis (GPA) lack antineutrophil cytoplasmic autoantibodies (ANCA), but the significance of this finding has not been clear. In a cohort study that compared clinical features and outcomes among 110 patients with ANCA-negative GPA and 110 with ANCA-positive GPA, patients with ANCA-negative GPA had milder disease and less pulmonary and kidney involvement than those with ANCA-positive GPA [23]. They also had lower remission rates (75 versus 89 percent) and lower relapse rates (9 versus 22 percent) at 60 months, which may reflect the tendency to use conventional immunosuppression (eg, methotrexate, azathioprine) to treat mild GPA. These findings suggest that patients with ANCA-negative GPA experience a more benign course than those with ANCA-positive disease. (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies", section on 'Granulomatosis with polyangiitis'.)

Avacopan for necrotizing granuloma in granulomatosis with polyangiitis (January 2025)

A randomized trial in over 330 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) previously found comparable rates of remission with avacopan (an oral complement inhibitor) or a prednisone taper when used with standard remission induction therapy; whether avacopan is effective for the necrotizing granulomatous lesions of GPA (which typically affect the ear, nose, and throat [ENT] and lung) is less clear. In a post-hoc analysis of this trial that included 144 patients with ENT manifestations and 142 patients with lung manifestations, remission rates at 26 and 52 weeks were similar among those treated with avacopan or a prednisone taper [24]. At 52 weeks, patients with lung manifestations receiving avacopan had a lower relapse rate than those receiving prednisone. These findings imply that avacopan can be used in lieu of glucocorticoids to manage the necrotizing granulomatous manifestations of GPA. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and maintenance therapy", section on 'Avacopan'.)

Sex and IgA vasculitis outcomes (January 2025)

IgA vasculitis (IgAV) is more common in males, but the impact of sex on disease presentation and outcomes has been unclear. A recent retrospective analysis of 164 males and 95 females with IgAV found that while baseline rates of cutaneous, joint, gastrointestinal, and kidney involvement were similar, males had more severe kidney disease (ie, lower glomerular filtration rate, higher proteinuria) than females [25]. Males were also more likely to develop refractory disease (30 versus 13 percent). These findings suggest that sex may be an important prognostic factor in IgAV, particularly in predicting kidney disease severity and response to treatment. (See "IgA vasculitis (Henoch-Schönlein purpura): Management", section on 'Prognosis'.)

Avacopan-associated drug-induced liver injury (January 2025)

Avacopan, an oral complement inhibitor used for the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), has been associated with reversible increases in liver function tests in clinical trials; however, the risk of drug-induced liver injury (DILI) may be higher in some populations. In an observational study of 22 Japanese patients with GPA or MPA who were treated with avacopan, nine (41 percent) developed DILI; eight improved after discontinuation of avacopan and other medications, and one developed vanishing bile duct syndrome resulting in death [26]. These findings suggest that patients of Japanese descent may be at increased risk of avacopan-associated DILI and may benefit from closer monitoring. (See "Granulomatosis with polyangiitis and microscopic polyangiitis: Induction and maintenance therapy", section on 'Avacopan'.)

Cutaneous and noncutaneous manifestations of IgA vasculitis (January 2025)

The relationship between the cutaneous and noncutaneous manifestations of immunoglobulin A (IgA) vasculitis is not well understood. In a retrospective study of 489 patients with IgA vasculitis, 7 percent of patients had a persistent rash (ie, lasting for longer than one month) and 29 percent had a rash that affected the trunk, arms, or face [27]. Patients with a persistent rash were more likely to develop glomerulonephritis, while patients with lesions above the level of the buttocks were more likely to develop gastrointestinal or genital tract involvement. These results imply that the cutaneous manifestations of IgA vasculitis may be helpful to predict outcomes in patients with this diagnosis. (See "IgA vasculitis (Henoch-Schönlein purpura): Management", section on 'Prognosis'.)

Baricitinib for polymyalgia rheumatica (January 2025)

There has been growing interest in finding alternatives to glucocorticoids for the treatment of polymyalgia rheumatica (PMR). In a randomized trial of 34 patients with PMR, patients assigned to 12 weeks of treatment with baricitinib 4 mg daily (without oral glucocorticoids) achieved remission more frequently than patients assigned to placebo (78 versus 13 percent) [28]. Subsequent treatment with an additional 12 weeks of baricitinib 2 mg daily was adequate to maintain remission. Baricitinib may be an option for patients with PMR who cannot tolerate oral glucocorticoids. (See "Treatment of polymyalgia rheumatica", section on 'Indications and rationale'.)

OTHER RHEUMATOLOGY

Adalimumab tapering for non-infectious pediatric uveitis (May 2025)

Most patients with noninfectious pediatric uveitis (NIU) will achieve remission with adalimumab, a tumor necrosis factor inhibitor; however, the optimal approach to tapering adalimumab following a prolonged remission (ie, >26 weeks) has been unclear. A retrospective study of 114 patients (46 percent of whom had juvenile idiopathic arthritis-associated uveitis) demonstrated that slower tapering schedules (ie, increasing the interval between adalimumab administrations by no more than one week every four months) were associated with a reduction in relapse risk (hazard ratio 0.40) and a longer median time to recurrence (37 versus 10 weeks) [29]. For children with NIU in a prolonged remission, slowly increasing the time between adalimumab administrations may reduce the risk of uveitis relapse. (See "Juvenile idiopathic arthritis: Immunizations and complications", section on 'Subsequent monitoring and duration of therapy'.)

Inebilizumab for IgG4-related disease (April 2025)

Although rituximab (an anti-CD20 monoclonal antibody) is commonly used for the treatment of IgG4 related disease (IgG4 RD) refractory to glucocorticoids, this practice has not been well validated in clinical trials. Recently, the US Food and Drug Administration (FDA) granted approval for the use of inebilizumab, an anti-CD19 monoclonal antibody, for the treatment of IgG4 RD based on a trial of 135 patients with IgG4 RD randomized to receive inebilizumab (300 mg intravenous infusions on days 1 and 15 and week 26) or placebo [30]. At the end of 52 weeks of observation, patients treated with inebilizumab had fewer flares than patients treated with placebo (10 versus 62 percent). Patients assigned to inebilizumab were also more likely to have a flare-free, glucocorticoid-free, complete remission, than patients treated with placebo (odds ratio 5.0). Theoretically, antibodies directed against CD19 result in deeper depletion of the B-cell compartment than anti-CD20 antibodies; however, absent comparative data, the choice between these agents is based on payor preferences and the clinician’s experience with these agents. (See "Treatment and prognosis of IgG4-related disease", section on 'Rituximab or inebilizumab'.)

Causes of hypertrophic pachymeningitis (March 2025)

Hypertrophic pachymeningitis is the thickening of the dura mater, which is usually secondary to immune-related, infectious, or neoplastic etiologies. In a retrospective single-center study of 74 patients with hypertrophic pachymeningitis, an immune-related origin was identified in 43 percent of cases, most commonly granulomatosis with polyangiitis, systemic lupus erythematosus, or IgG4-related disease [31]. Nearly half of cases were due to malignancy (28 percent; mostly hematologic cancers) or tuberculosis (19 percent); only three cases (4 percent) were deemed idiopathic after extensive evaluation. Hypertrophic pachymeningitis has varied etiologies and is often a manifestation of a multisystem disorder. (See "Clinical manifestations and diagnosis of IgG4-related disease", section on 'Other organ involvement and multisystem disease'.)

Vitamin K2 supplementation for older adults with nocturnal muscle cramps (January 2025)

Nocturnal muscle cramps are common, particularly among older patients, but the optimal treatment is unknown. A recent multicenter trial randomly assigned 199 patients aged 65 and older with nocturnal leg cramps to an eight-week course of vitamin K2 (180 micrograms/day) or placebo [32]. Compared with the placebo group, patients treated with vitamin K2 experienced a greater reduction in the mean frequency of cramps from baseline to the intervention period; the vitamin K2 group also experienced comparably greater improvements in the severity and mean duration of cramping. This study suggests that vitamin K2 may be a reasonable alternative to other supplements used to treat nocturnal muscle cramps, particularly in older adults. (See "Nocturnal muscle cramps", section on 'Initial drug therapies'.)

Recurrence of acute anterior uveitis (November 2024)

Patients with acute anterior uveitis (AAU) may experience a single episode or evolve to have recurrent disease; however, it is unclear what factors are associated with disease recurrence. In a study that followed over 2000 patients with AAU for a median of 8.9 years, 46 and 27 percent of patients experienced disease recurrence in the ipsilateral or contralateral eyes, respectively [33]. Factors associated with a higher risk of recurrent uveitis included underlying inflammatory arthritis/positive human leukocyte antigen B27 (HLA-B27), Māori ethnicity, and, for ipsilateral recurrence, viral uveitis, Asian ethnicity, and older age. Patients with risk factors for recurrent AAU should be counseled appropriately; future research is required to investigate if they might benefit from more aggressive monitoring and treatment. (See "Uveitis: Treatment", section on 'Prognosis'.)

Pregnancy in vascular Ehlers-Danlos syndrome (October 2024)

While pregnant persons with vascular Ehlers-Danlos syndrome (vEDS) have an increased risk of maternal morbidity and mortality, more information is needed on this rare disorder to appropriately counsel and manage patients. In a systematic review including six studies with a total of 412 pregnancies, the maternal mortality rate was 5.7 percent (22 of 386 patients); deaths were attributed to vascular rupture or dissection (12 patients), uterine rupture (7 patients), and rupture of other organs (1 patient) [34]. There were no deaths in the group of 28 patients who underwent a scheduled cesarean delivery. Providers should carefully counsel patients with vEDS about the risks associated with pregnancy and consider scheduling cesarean delivery at 37 weeks of gestation. (See "Ehlers-Danlos syndromes: Overview of the management", section on 'Pregnancy, delivery, and postpartum care'.)