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.

FIBROMYALGIA

Trial of low-dose naltrexone for treatment of fibromyalgia (February 2024)

Low-dose naltrexone has been proposed as a treatment for pain in patients with fibromyalgia, but supporting data are limited and mixed. In a recent randomized trial in 99 patients with fibromyalgia, low-dose naltrexone modestly improved pain scores compared with placebo at 12 weeks (between-group difference of 0.34 on a scale of 1 to 10), but the result was not statistically significant [1]. Among secondary outcomes, there was a small improvement in subjective memory ratings favoring naltrexone, which could be explored in future trials. Although evidence does not support routine use, low-dose naltrexone is generally safe and well-tolerated and may be an option for patients who are refractory to first- and second-line therapies. (See "Treatment of fibromyalgia in adults", section on 'Other medications'.)

NEUROMUSCULAR DISEASE

Features of drug-induced dermatomyositis (April 2024)

Drug-induced dermatomyositis can present with cutaneous features similar to idiopathic dermatomyositis. In a systematic review that identified 165 patients with possible drug-induced dermatomyositis, the most commonly reported inciting therapies were hydroxyurea, immune checkpoint inhibitors, statins, penicillamine, and tumor necrosis factor inhibitors [2]. The median time between initial drug exposure and disease development was 60 days. Although the strength of the drug association varied broadly among the 134 included articles, this study highlights drug-induced dermatomyositis as a consideration when evaluating patients with the cutaneous features of this disease. (See "Clinical manifestations of dermatomyositis and polymyositis in adults", section on 'Skin findings in dermatomyositis'.)

Benign acute childhood myositis (January 2024)

Benign acute childhood myositis (BACM) is a self-limited syndrome associated with calf pain and creatinine kinase elevation, often following infection with influenza. In a retrospective study of 65 patients with BACM, the median age was 6.6 years and 66 percent of patients were male [3]. The most common symptoms were bilateral calf pain, refusal to walk, and diffuse weakness. The median creatinine kinase was 1827 U/L, which normalized after an average of seven days. Early recognition of this syndrome allows the clinician to avoid an unnecessary evaluation for other muscle diseases. (See "Overview of viral myositis", section on 'Benign acute childhood myositis'.)

ORTHOPEDICS, SPINE, AND SOFT TISSUE RHEUMATIC DISORDERS

Trial of surgery, needle fasciotomy, or collagenase injection for Dupuytren's contracture (May 2024)

Dupuytren's contracture is a progressive fibroproliferative disease of the hand and fingers that can lead to permanent disability and for which the optimal treatment is not known. In a recent trial, over 300 patients with Dupuytren's contracture were randomly assigned to treatment with one of three interventions: surgery, needle fasciotomy (percutaneous needle aponeurotomy), or collagenase injection [4]. After three months, the success rate was similar among the groups; however, after two years, the success rate was higher for patients who had undergone surgery compared with those who had a needle fasciotomy or collagenase injection (78 versus 50 and 65 percent, respectively). The choice among available therapies for flexion contractures related to Dupuytren's should be based on the patient's preference after a discussion of the risks and benefits of each approach. (See "Dupuytren's contracture", section on 'Selection of therapy'.)

Barbotage procedure for calcific tendinopathy of shoulder (January 2024)

To date, few high-quality studies have assessed the effectiveness of barbotage, an ultrasound-guided procedure to remove deposits in patients with calcific tendinopathy of the shoulder. In a recent, multicenter trial, 220 adults with calcific tendinopathy of at least three months duration were randomly assigned to one of three treatment arms: barbotage plus injection with glucocorticoid and analgesic; sham barbotage plus injection with glucocorticoid and analgesic; or, sham barbotage plus injection of analgesic alone [5]. At four months, patients in all three groups experienced moderate improvement in shoulder symptoms and function, but no significant differences were noted among treatment groups. At 24 months, neither barbotage with glucocorticoid injection nor glucocorticoid injection alone was superior to sham treatment (ie, analgesic injection alone). While barbotage is likely less effective than previously thought, we believe it remains a useful therapy for some patients. (See "Calcific tendinopathy of the shoulder", section on 'Barbotage'.)

OSTEOARTHRITIS

Investigational use of denosumab for erosive hand osteoarthritis (April 2024)

There are limited treatment options for erosive osteoarthritis (OA) of the hand. Denosumab, a RANK ligand inhibitor used for the treatment of osteoporosis, appears promising. In a randomized trial of 100 patients with erosive hand OA, denosumab reduced radiographic progression and new erosive joint development at 48 weeks compared with placebo [6]. Pain and disability scores improved from baseline with denosumab during the open-label extension at 96 weeks, but not at earlier time points. This is the first study to demonstrate that targeted therapy can reduce structural damage in erosive hand OA, but further data on patient-important outcomes are necessary to clarify its potential role. (See "Management of hand osteoarthritis", section on 'Therapies with limited efficacy or of uncertain benefit'.)

PEDIATRIC RHEUMATOLOGY

Multisystem inflammatory syndrome in children incidence and severity (March 2024)

Although multisystem inflammatory syndrome in children (MIS-C) is increasingly rare as the overall COVID-19 burden declines, it continues to be associated with substantial morbidity. In 2023, 117 patients with MIS-C were reported to the Centers for Disease Control and Prevention, resulting in an estimated incidence of 0.11 cases per million person-months [7]. Of these patients, 50 percent required care in an intensive care unit, 34 percent experienced shock, and 27 percent experienced cardiac dysfunction; mortality was 2.6 percent (3 patients). More than 80 percent of patients were SARS-CoV2 vaccine eligible but had not been vaccinated; among 20 patients who had been vaccinated, 60 percent likely had waning immunity at the time of diagnosis. This study suggests that MIS-C severity remains high, but that SARS-CoV2 vaccination may provide some degree of protection. (See "COVID-19: Multisystem inflammatory syndrome in children (MIS-C) clinical features, evaluation, and diagnosis", section on 'Epidemiology'.)

RHEUMATOID ARTHRITIS

Reduction of immunosuppression in patients with rheumatoid arthritis (April 2024)

Whether patients with stable rheumatoid arthritis (RA) can be tapered off conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) is unclear. In a randomized trial exploring this question in 160 patients with RA in remission for at least one year on a stable csDMARD regimen, patients assigned to continue full-dose csDMARD therapy were most likely to remain in remission at three years (80 percent) [8]. Those assigned to half-dose therapy or discontinuation (after half-dose therapy for one year) had lower rates of ongoing remission (57 and 38 percent, respectively). While this study highlights a high risk of relapse among patients with RA following a reduction in immunosuppression, it also demonstrates that some patients do not require full-dose therapy to remain in remission. Additional studies are required to identify which patients with RA are most likely to tolerate reduction or discontinuation of csDMARD therapy. (See "Initial treatment of rheumatoid arthritis in adults", section on 'Adequate treatment response'.)

Extra-articular rheumatoid arthritis and mortality (March 2024)

Rheumatoid arthritis (RA) is a systemic inflammatory disease that can have extra-articular manifestations, which may be severe (eg, rheumatoid vasculitis) or nonsevere (eg, rheumatoid nodules). In a study that examined outcomes of 296 patients diagnosed with RA from 1985 to 1999 and 611 patients diagnosed from 2000 to 2014, extra-articular manifestations were associated with an increased risk of mortality among all patients (hazard ratio 3.0 for severe and 1.8 for nonsevere manifestations) [9]. However, the 10-year cumulative incidence of extra-articular RA declined between the two cohorts (45 versus 32 percent). Despite the declining incidence of extra-articular RA, the presence of any extra-articular features continues to be associated with a poor prognosis. (See "Disease outcome and functional capacity in rheumatoid arthritis", section on 'Risk factors for premature mortality'.)

Plain radiographs in the initial evaluation of rheumatoid arthritis (March 2024)

Patients suspected of having rheumatoid arthritis (RA) are routinely evaluated with plain radiographs of the hands and feet. However, whether this is an effective strategy has not been well established. In a study of 724 patients suspected of having RA, erosions were found in only 32 patients (4.4 percent), and radiographs led to a change in disease classification for only 2 patients (0.3 percent) [10]. Patients who lacked RA-associated autoantibodies and/or acute phase reactant elevation were less likely to demonstrate RA-associated erosions. Although the yield for these outcomes was low, plain radiographs may be useful for establishing alternate diagnoses that can mimic RA (eg, pseudogout) and can identify other findings associated with RA that guide management (eg, periarticular osteopenia). (See "Diagnosis and differential diagnosis of rheumatoid arthritis", section on 'Radiologic studies'.)

Abatacept for the prevention of rheumatoid arthritis (February 2024)

There is interest in whether biologic agents can be used to prevent the development of rheumatoid arthritis (RA) in high-risk patients. In a recent trial, 213 patients at risk of developing RA based on inflammatory joint pain and the presence of RA-associated autoantibodies were randomly assigned to 12 months of abatacept (a T cell costimulation modulator) or placebo and followed for an additional 12 months [11]. The proportion of patients who remained arthritis-free was higher in the abatacept group at 12 months (93 versus 69 percent) and 24 months (70 versus 59 percent); improvements in pain, function, and quality of life at 12 months were not sustained at 24 months. This study provides evidence that pharmacotherapy can delay the onset of RA in select populations, but prevention may require ongoing therapy. (See "Epidemiology of, risk factors for, and possible causes of rheumatoid arthritis", section on 'Clinical trials examining preventive therapies'.)

Triglycerides and cardiovascular risk in rheumatoid arthritis (January 2024)

Triglycerides may be particularly important to the pathogenesis of cardiovascular disease among patients with rheumatoid arthritis (RA). In a nationwide, population-based cohort study of >15,000 statin-naïve patients with RA in Korea, the risk of major adverse cardiovascular events was higher among patients with the highest baseline triglyceride levels (≥149 mg/dL) compared with those with the lowest triglyceride levels (≤72 mg/dL; adjusted hazard ratio 1.7) [12]. The same relationship was not seen with low-density lipoprotein cholesterol, high-density lipoprotein cholesterol, or total cholesterol. This study implies that careful management of triglycerides may be particularly important to prevent cardiac events among patients with RA. (See "Coronary artery disease in rheumatoid arthritis: Pathogenesis, risk factors, clinical manifestations, and diagnostic implications", section on 'Risk factors specific to rheumatoid arthritis'.)

SCLERODERMA AND OTHER SYSTEMIC RHEUMATIC DISEASES

Phosphodiesterase type 5 inhibition for Raynaud phenomenon (January 2024)

Phosphodiesterase type 5 (PDE5) inhibitors such as sildenafil and tadalafil are widely used to treat digital ischemia from Raynaud phenomenon. In an updated meta-analysis of nine randomized trials comprising 411 patients with Raynaud phenomenon (most of whom had scleroderma), treatment with PDE5 inhibition resulted in three fewer attacks weekly and a reduction in the average duration of the attacks by five minutes [13]. However, PDE5 inhibition led to minimal to no reduction in the pain associated with Raynaud phenomenon. This study implies that while PDE5 inhibition has a modest impact on the duration and frequency of Raynaud attacks, it might not be adequate to address all symptoms experienced by patients with severe disease. (See "Treatment of Raynaud phenomenon: Initial management", section on 'Phosphodiesterase type 5 inhibitor'.)

SPONDYLOARTHRITIS AND PSORIATIC ARTHRITIS

Tumor necrosis factor inhibitors for females with psoriatic arthritis (March 2024)

Tumor necrosis factor (TNF) inhibitors are commonly used as first-line therapy for the treatment of psoriatic arthritis (PsA). In a multicenter observational study of nearly 7700 patients with PsA who received initial treatment with a TNF inhibitor, females were less likely than males to have achieved low disease activity at 6 months (64 versus 78 percent) [14]. Females were also less likely to remain on a TNF inhibitor (50 versus 64 percent at 24 months). These data imply that TNF inhibitors may be less effective for females than males with PsA, and females should be followed closely after treatment initiation. (See "Treatment of peripheral psoriatic arthritis", section on 'Reasons for failure'.)

Diagnosis of axial spondyloarthritis using nonsteroidal anti-inflammatory agents (March 2024)

There is a long-standing belief that a dramatic response to nonsteroidal anti-inflammatory agents (NSAIDs) differentiates axial spondyloarthritis (axSpA) from other causes of lower back pain. However, in a recent prospective study of 68 consecutive patients with axSpA and 165 patients with chronic back pain from other causes who were treated with NSAIDs for four weeks, patients with axSpA were only modestly more likely to report at least 50 percent improvement in back pain (23 versus 16 percent), and the difference was not statistically significant [15]. Although this result casts some doubt on the diagnostic utility of an NSAID trial, the study was small and nonrandomized, and it does not exclude the possibility that some NSAIDs may be better than others at distinguishing axSpA from other diagnoses. (See "Diagnosis and differential diagnosis of axial spondyloarthritis (ankylosing spondylitis and nonradiographic axial spondyloarthritis) in adults", section on 'History'.)

SYSTEMIC LUPUS ERYTHEMATOSUS AND SJÖGREN'S DISEASE

Adverse effects of low-dose glucocorticoids in patients with systemic lupus erythematosus (April 2024)

Glucocorticoids (GCs) are frequently required for disease control in patients with systemic lupus erythematosus (SLE) but can cause multiple adverse effects; whether low doses of GCs offer a more acceptable balance of risks and benefits is uncertain. In a national cohort study in Sweden that followed over 5300 adults with SLE for up to 15 years, compared with patients not taking oral GCs, those taking GCs had higher rates of multiple adverse outcomes, including overall mortality, various bacterial and viral infections, gastroduodenal ulcers, hypertension, osteoporosis, osteonecrosis, and cataracts [16]. Patients taking low-dose GCs (<5 mg/day) had relatively lower risks for adverse effects compared with those taking higher doses, but still had higher risks compared with patients who were not taking GCs. These results underscore the importance of using the lowest GC dose possible to control SLE activity and monitoring for adverse effects, even in patients taking low doses. (See "Systemic lupus erythematosus in adults: Overview of the management and prognosis", section on 'Prognosis'.)

Histopathologic characteristics of Sjögren's disease (April 2024)

A diagnosis of Sjögren's disease (SjD) is typically established by identifying lymphocytic periductal infiltrates (ie, a "focus") in a salivary gland biopsy. In a study including 103 patients with suspected SjD, labial gland biopsies were examined for histopathologic features associated with this diagnosis [17]. The presence of at least one focus per 4 mm² salivary tissue (ie, a focus score ≥1) had the highest sensitivity for a diagnosis of primary SjD (82 percent). However, other histopathologic features (ie, germinal centers, lymphoepithelial lesions, plasma cell shift) were more specific for this diagnosis. This study implies that examining salivary gland biopsies for features other than the focus score may help confirm a diagnosis of SjD. (See "Diagnosis and classification of Sjögren's disease", section on 'Salivary gland biopsy'.)

Telitacicept in patients with Sjögren's disease (March 2024)

Novel therapies are under investigation for Sjögren's disease (SjD). Telitacicept is a fusion protein that combines a recombinant transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI) and the fragment crystallizable (Fc) domain of human immunoglobulin G. In a small randomized trial in 42 patients with primary SjD, telitacicept 160 mg subcutaneously weekly reduced the EULAR Sjögren's syndrome disease activity index (ESSDAI) compared with placebo, attributed to improvements in lymphadenopathy, glandular swelling, arthritis, and skin disease [18]. Telitacicept has regulatory approval in China and is under review in the United States for other indications; larger studies are needed in patients with SjD. (See "Treatment of Sjögren's disease: Constitutional and non-sicca organ-based manifestations", section on 'Other agents in clinical trials'.)

CD19 CAR-T cell therapy for systemic autoimmune disease (March 2024)

CD19 chimeric antigen receptor (CAR) T cell therapy, which has been used to treat certain hematologic malignancies, may be a potential pathway to target pathogenic B cells in systemic autoimmune disease. In a recent case series, 15 patients with severe autoimmune disease (systemic lupus erythematosus, systemic sclerosis, or idiopathic inflammatory myositis) received CD19 CAR-T cell therapy; over a median of 15 months, all patients had rapid B-cell depletion and improved disease activity and could stop immunosuppressive therapy [19]. Important safety events included cytokine release syndrome (11 patients), possible mild immune effector cell-associated neurotoxicity syndrome, and severe pneumonia. These preliminary data suggest that CD19 CAR-T cell therapy may be beneficial for multiple types of systemic autoimmune diseases and warrants further evaluation. (See "Systemic lupus erythematosus in adults: Overview of the management and prognosis", section on 'Agents under investigation'.)

Sjögren's disease and head and neck cancers (January 2024)

Patients with Sjögren's disease (SjD) are known to have an increased risk of lymphoproliferative disease, but they may also have a higher risk of head and neck cancers. In a population-based study in Taiwan that included nearly 39,000 patients with SjD and over 150,000 propensity-matched controls, the prevalence of head and neck cancers was higher among patients with SjD (1.8 versus 1.2 percent) [20]. This was predominantly due to an increased prevalence of oral cavity, oropharynx, nasopharynx, and thyroid cancers. Patients with SjD should be monitored carefully for both lymphoproliferative disease and head and neck cancers. (See "Overview of the management and prognosis of Sjögren's disease", section on 'Prognosis'.)

VASCULITIS

Tofacitinib in patients with polymyalgia rheumatica (May 2024)

Findings from a small proof-of-concept study suggest that tofacitinib, a Janus tyrosine kinase inhibitor, may be effective for the treatment of polymyalgia rheumatica (PMR). In this study, 67 patients with newly diagnosed PMR were randomly assigned to receive tofacitinib (5 mg twice daily) or prednisone 15 mg daily (followed by a subsequent taper) [21]. At the end of 12 and 24 weeks, patients had an equivalent response to both therapies, as measured by acute phase reactants (ie, erythrocyte sedimentation rate, C-reactive protein) and the PMR Activity Score (PMR-AS). More data with longer-term follow-up are needed to determine whether tofacitinib is an effective substitute for glucocorticoids for patients with PMR. (See "Treatment of polymyalgia rheumatica", section on 'Limited role for glucocorticoid-sparing therapies'.)

Antithyroid drugs and ANCA-associated vasculitis (May 2024)

Some cases of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) have been linked to the use of antithyroid drugs (ATD), although data are limited. In a recent retrospective study of 45 patients with ATD-induced AAV, nearly half were positive for myeloperoxidase (MPO)-ANCA, and 33 percent were positive for both MPO-ANCA and proteinase 3 (PR3)-ANCA [22]. Patients were most likely to present with cutaneous manifestations and arthralgias; in 16 percent of cases, discontinuation of the ATD led to resolution of vasculitis, while the remaining patients were treated with an immunosuppressive agent. This study highlights that some patients with ATD-induced AAV may not require immunosuppression, and that ATD-induced AAV should be considered in patients who present with both MPO-ANCA and PR3-ANCA, a combination rarely seen in other forms of AAV. (See "Clinical spectrum of antineutrophil cytoplasmic autoantibodies", section on 'Drug-induced ANCA-associated vasculitis'.)

Possible infectious causes of IgA vasculitis (April 2024)

Although IgA vasculitis (IgAV; formerly Henoch-Schönlein purpura) is the most common cause of systemic vasculitis among children, its cause is unknown. A hospital-based surveillance system in France identified over 9000 children diagnosed with IgAV from 2015 to 2023 [23]. A time-series analysis indicated that the incidence of IgAV decreased by over 50 percent following the implementation of social distancing policies for the COVID-19 pandemic. Subsequently, after relaxation of social distancing and mask-wearing, there was a temporal association between the monthly incidence of IgAV and outbreaks of Streptococcus pneumoniae and Streptococcus pyogenes, but not other common pathogens. This study demonstrates a potential causal relationship between specific infections and IgA vasculitis, but these results need to be replicated in other patient populations. (See "IgA vasculitis (Henoch-Schönlein purpura): Clinical manifestations and diagnosis", section on 'Pathogenesis'.)

Ultrasound for the diagnosis of giant cell arteritis (April 2024)

Vascular ultrasound is being investigated as a substitute for biopsy for the diagnosis of giant cell arteritis (GCA). In a prospective cohort study including 229 patients with suspected GCA, a prediction model using both a clinical prediction algorithm and a quantitative ultrasound was able to classify 74 percent of patients as having either a low or high probability for GCA [24]. The prediction model misclassified 2 percent of patients as low probability who eventually were diagnosed as having GCA; an additional 3 percent of patients classified as high probability for GCA were eventually reclassified as having other diagnoses. Although this study suggests that temporal artery biopsy may not be necessary to evaluate all patients with suspected GCA, it was conducted by rheumatologists who were specifically trained to use ultrasound for GCA. Until such expertise is more broadly available, we continue to evaluate patients with suspected GCA with temporal artery biopsies. (See "Diagnosis of giant cell arteritis", section on 'Patients with a positive biopsy or imaging'.)

Tocilizumab and short prednisone tapers for giant cell arteritis (January 2024)

Tocilizumab may allow patients with giant cell arteritis to tolerate very short prednisone tapers. In a recent study, 30 patients with newly diagnosed or relapsing giant cell arteritis were treated with 52 weeks of subcutaneous tocilizumab (162 mg weekly) and an eight-week prednisone taper (starting with 60 mg daily) [25]. All patients entered remission after four weeks of therapy; 77 percent remained in a glucocorticoid-free remission by one year. Among patients who relapsed, the mean time to relapse was 16 weeks. Although select patients receiving tocilizumab for giant cell arteritis may be appropriate for a very short prednisone taper, larger studies are needed before broad implementation. (See "Treatment of giant cell arteritis", section on 'Unproven or ineffective agents'.)

OTHER RHEUMATOLOGY

No association between monoclonal gammopathy of undetermined significance and autoimmune diseases or immune-related conditions (May 2024)

Studies have evaluated whether a relationship exists between monoclonal gammopathy of undetermined significance (MGUS) and autoimmune diseases or immune-related conditions. Although several retrospective studies had suggested an increased risk of MGUS among patients with these conditions, a large Icelandic prospective population-based screening study did not find an association [26]. The increased prevalence in prior retrospective studies likely reflects a sampling bias as testing was likely preferentially performed in patients with autoimmune diseases or immune-related conditions due to a perceived association. (See "Diagnosis of monoclonal gammopathy of undetermined significance", section on 'Associated conditions'.)

Risk of autoimmune inflammatory rheumatic disease following COVID-19 (May 2024)

The risk of developing autoimmune inflammatory rheumatic diseases (AIRDs) following COVID-19 has recently been studied (eg, rheumatoid, psoriatic, and spondyloarthritides and connective tissue disorders) [27]. A Korean and Japanese cohort analysis of 22 million patients reported an increased risk of AIRDs in patients who had COVID-19 compared with uninfected patients (hazard ratio [HR], 1.25 [Korea], 1.79 [Japan]) and with patients who had influenza (HR, 1.30 [Korea], 1.14 [Japan]). The risk appeared to diminish over time and was likely reduced by vaccination. Clinicians should be aware of the risk of AIRD following COVID-19 and investigate appropriately when suspected. (See "COVID-19: Clinical presentation and diagnosis of adults with persistent symptoms following acute illness ("long COVID")", section on 'Physical symptoms'.)

Genetic risk factors in Lofgren syndrome (May 2024)

There are relatively little long-term follow-up data regarding patients presenting with Lofgren syndrome, which is a form of sarcoidosis characterized by acute arthritis, hilar adenopathy, and erythema nodosum. In a retrospective study of 380 patients diagnosed with Lofgren syndrome, 11 percent of patients experienced disease relapse after a median of 5.5 years [28]. Patients who were human leukocyte antigen (HLA) DRB1*03 positive were more likely to relapse with Lofgren syndrome than with other manifestations of sarcoidosis. Of the HLA-DRB1*03-negative patients who developed a chronic arthritis, 67 percent were also HLA-DRB1*15 positive. Although these results need to be replicated, HLA genotyping may help identify patients with Lofgren syndrome who are more likely to develop a chronic arthritis and may benefit from long-term follow-up. (See "Sarcoid arthropathy", section on 'Acute arthritis and Lofgren syndrome'.)

Remission maintenance therapy for IgG4-related disease (March 2024)

Whether patients with immunoglobulin G4-related disease (IgG4-RD) should receive remission maintenance therapies is unclear. In an open-label trial of 146 patients with IgG4-RD who were in remission for at least 12 months and were randomly assigned to continue or discontinue immunosuppressive therapies, relapse within 18 months occurred in 52 percent of those assigned to stop all immunosuppression and 12 to 14 percent of those who continued oral immunosuppressive therapies (eg, mycophenolate mofetil, leflunomide) with or without glucocorticoids [29]. Unfortunately, the study was not able to identify clinical predictors of benefit from prolonged immunosuppression. However, the study implies that, like other rheumatic diseases, IgG4-RD has a substantial relapse rate, which may be mitigated by maintenance therapies. (See "Treatment and prognosis of IgG4-related disease", section on 'Maintenance therapy'.)

Clinical features of VEXAS syndrome (March 2024)

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a severe, late-adult onset autoinflammatory disease involving the nuclear factor kappa B (NFkB) pathway. The spectrum of clinical manifestations and features of this disease are demonstrated in three recent studies: In a French cohort, patients had an increased risk of serious infections, primarily bacterial and viral but also including invasive fungal and atypical infections despite antimicrobial prophylaxis and may be attributable to intrinsic immunodeficiency in addition to being associated with Janus kinase (JAK) inhibitor therapy [30]. In an Italian cohort, several patients with myelodysplastic syndrome progressed to acute myeloid leukemia [31]. In a Spanish cohort, UBA1 mosaicism was detected in both hematopoietic and nonhematopoietic tissues, indicating that the mutational event occurs during early embryonic development, not adulthood as was previously suspected [32]. The mortality rate was high in all cohorts, ranging from 12 to 40 percent. (See "Autoinflammatory diseases mediated by NFkB and/or aberrant TNF activity", section on 'Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome'.)

Revised classification criteria for antiphospholipid antibody syndrome (November 2023)

Antiphospholipid antibody syndrome (APS) presents with a wide array of clinical manifestations that can cause significant morbidity, making it both an important area for research and also challenging to define in studies. The American College of Rheumatology and the European Alliance of Associations for Rheumatology have recently updated the classification criteria for APS to allow more rigorous classification in research; these criteria should not substitute for clinical judgment when diagnosing APS [33]. The new criteria include a broader range of clinical manifestations (eg, microvascular complications, cardiac valve disease, thrombocytopenia, changes to pregnancy morbidity), risk stratification of macrovascular events, and a more nuanced weighting system for antiphospholipid antibodies (eg, considering immunoglobulin G versus immunoglobulin M isotypes). (See "Diagnosis of antiphospholipid syndrome", section on 'Classification criteria'.)