Audiovestibular outcomes in adult patients with cogan syndrome: a systematic review.

PURPOSE: To determine factors associated with steroid responsiveness and efficacy of biologic disease-modifying anti-rheumatic (DMARD) use in patients with Cogan Syndrome (CS). METHODS: A systematic search of Cochrane Library, PubMed, CINAHL, and Scopus was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. Any study describing audiometric or vestibular data and pharmacologic treatment in patients with CS was included. Due to limited literature, only case reports/case series were included. RESULTS: Seventy case reports or case series studies comprising 79 individual cases of CS were included. A difference in vestibular symptoms with a higher prevalence in the steroid-resistant group than the steroid-responsive group was found (79.5% vs 57.9%, p = 0.04). Eighteen (60.0%) patients treated only with oral steroids had no audiological improvement, while twelve (n = 12; 85.7%) patients treated with biologic DMARD showed audiological improvement. The steroid-responsive group had an overall better response to DMARDs than the steroid-resistant group (62.1% vs 45.0%; 100.0% vs 77.8%). CONCLUSIONS: Our study synthesized the available literature to better characterize steroid resistance in patients with Cogan syndrome and treatment outcomes. Vestibular symptoms were noted to be more prevalent in patients who were eventually labeled as steroid resistant. There were higher rates of audiological improvement in patients given biologic DMARDs rather than conventional DMARDs or steroids only. Further studies are needed to characterize each individual vestibular symptom and investigate the utility and timing of biologic DMARDs in managing patients with Cogan syndrome.

Hearing loss in patients with systemic lupus erythematosus: A systematic review and meta-analysis.

OBJECTIVE: To determine the prevalence of hearing loss (HL) in patients with systemic lupus erythematosus (SLE), describe frequency-specific hearing threshold changes in this patient population as compared to age-matched control, and compare the clinical and serological profiles of patients with SLE with and without HL. METHODS: A systematic review querying four databases (PubMed, Web of Science, Scopus, and Cochrane) was performed. Meta-analysis of available data was performed to determine the overall prevalence and odds ratio (OR) of HL, and compare mean differences in frequency-specific hearing thresholds between patients with SLE and control. Additionally, meta-analysis of proportions allowed for comparison of disease features present in patients with SLE with and without sensorineural HL. RESULTS: This review included 17 studies reporting on 1326 patients (635 with SLE and 691 control). The pooled prevalence of HL in patients with SLE was 27%. In comparison to control, patients with SLE had a significantly higher odds of HL (OR 14.6, 95% CI: 8.5 to 25.0). Mean air-conduction hearing thresholds in patients with SLE were significantly elevated relative to control at 125 and 250 Hz. Mean bone-conduction hearing thresholds were significantly elevated in patients with SLE across all measured frequencies except at 3000 and 6000 Hz compared to control. Disease features did not significantly differ between patients with SLE with and without HL. CONCLUSION: Compared to age-matched control, patients with SLE have increased odds of HL, which primarily manifests at low frequencies. Therefore, this patient population requires greater audiologic attention.

Fish oil attenuates neurologic severity of antiphospholipid syndrome in a mice experimental model.

INTRODUCTION: Murine experimental models of antiphospholipid syndrome (eAPLS) showed neurologic dysfunction and therapeutic effect of the anticoagulant enoxaparin is well established. Omega-3 fatty acids and curcumin, tested in neuroinflammation and auto-immunity diseases, might be interesting therapeutic candidates. The aim of this study was to evaluate the effects of these candidates on neurologic severity in eAPLS. METHODS: One month after immunization of BALB/c mice with beta-2-glycoprotein I, daily treatments were initiated with enoxaparin (1 mg/kg), omega-3 fatty acids (0.5 g/kg), and curcumin (200 mg/kg) for 3 months. RESULTS: Mortality was significantly decreased by enoxaparin and omega-3 treatments. Fish oil and curcumin group exhibited the highest mean of swimming behavior in forced swim test in surviving mice. Mice under omega-3 fatty acids or curcumin presented low anxiety-like behavior in the elevated plus-maze test. Cerebral histopathology revealed heavy inflammatory infiltrates in cortical and subcortical regions with vacuolization, swelling, and degeneration of astrocytes in the control group, with aggravation under curcumin; no infiltrate was retrieved in enoxaparin and omega-3 groups. CONCLUSION: Our study is the first to demonstrate a potential therapeutic effect of omega-3 fatty acids in eAPLS. Enoxaparin and omega-3 fatty acids combination would be interesting for further investigation.

Disease-Modifying Antirheumatic Drugs in the Treatment of Autoimmune Inner Ear Disease: A Systematic Review and Meta-Analysis of Auditory and Vestibular Outcomes.

OBJECTIVE: To answer the following question: In patients with primary autoimmune inner ear disease (AIED), (population) what impact do disease-modifying antirheumatic agents (DMARDs) (intervention) when compared with no treatment or corticosteroids (comparison) have on auditory and vestibular outcomes (outcome)? STUDY DESIGN: Systematic review and meta-analysis. DATA SOURCES: According to PRISMA guidelines, PubMed, Scopus, CINAHL, and Cochrane Library databases were searched from inception to March 10, 2022. STUDY SELECTION: Studies of patients receiving DMARDs for the treatment of AIED were selected for review. Case reports, phase I/II trials, studies of patients with secondary AIED, and studies of AIED patients receiving solely corticosteroids were excluded. DATA EXTRACTION: Primary outcomes were pure-tone audiometry and speech discrimination scores at baseline and after DMARD treatment. Secondary outcomes were rates of subjective audiovestibular complaints and rates of adverse reactions. No objective vestibular outcomes underwent meta-analysis. DATA SYNTHESIS: Mean differences were calculated using RevMan 5.4. Heterogeneity was assessed with the Q test and I2 statistic. Pooled prevalence rates of audiovestibular symptoms were expressed as a percentage with 95% confidence intervals. RESULTS: Ten studies with a total of 187 patients were included. Treatments included methotrexate, etanercept, azathioprine, anakinra, cyclophosphamide, rituximab, and infliximab. Mean treatment duration was 10.8 ± 22.2 months and mean follow-up was 13.7 ± 8.1 months. The pure-tone audiometry and speech discrimination scores mean differences between baseline and post-DMARD were -2.1 [-4.1, -0.1] dB and 13.9 [8.5, 19.4] %, respectively. Seven studies reported 38 adverse events, four of which were classified as serious. CONCLUSION: DMARDs showed statistically significant improvement in auditory outcomes, as well as subjective symptoms, with relatively low rates of adverse events. They warrant further exploration to better compare with corticosteroids.

Secondary autoimmune immune ear disease (AIED): a systematic review and meta-analysis on vestibular manifestations of systemic autoimmune and inflammatory disorders.

Secondary autoimmune inner ear disease (AIED) is often bilateral and asymmetric in patients presenting with audiovestibular symptoms due to a systemic autoimmune disease. This systematic review and meta-analysis are aimed at identifying and highlighting patterns in prevalence of vestibular dysfunction, symptom presentation, and diagnostic methods in extant literature by combining clinical context from case reports with quantitative analyses from cohort studies. Screening of articles by title, abstract, and full text was completed by four reviewers (K.Z., A.L., S.C., and S.J.). In this study, we grouped secondary AIED and systemic autoimmune diseases by pathophysiologic mechanism: (1) connective tissue disease (CTD), (2) vasculitides (VAS), (3) systemic inflammatory disorders (SID), and (4) other immune-mediated disorders (OIMD). The search for AIED disease identified 120 articles (cohorts and case reports) that met the final inclusion criteria. All 120 were included in the qualitative review, and 54 articles were included for meta-analysis. Of these 54 articles, 22 included a control group (CwC). Ninety individual cases or patient presentations from 66 articles were included for analysis in addition to the 54 cohort articles. Secondary AIED does not have a diagnostic algorithm for managing vestibular symptoms. The management of audiovestibular symptoms requires close collaboration between otolaryngologists and rheumatologists to preserve end-organ function of the ear. To improve our ability to understand the impact on the vestibular system, vestibular clinicians need to develop a standardized reporting method. Clinical presentation should frequently be paired with vestibular testing to contextually investigate symptom severity and provide higher quality care.

Polymyalgia Rheumatica or Late Onset Lupus? A Case Report.

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with a peak age of presentation between the 15 and 40 years with a wide variety of disease manifestations. Although there is no formal definition, late onset SLE is generally defined in the literature as onset after the age of 50. It is estimated that 2% to 20% of patients with SLE overall fall into this category. It is important for the clinician to recognize this less-common entity because arthralgia, myalgia, fatigue, and sicca symptoms in the elderly can so easily be attributed as symptoms of normal aging or other common degenerative processes rather than a systemic disease similar to SLE or Sjogren's syndrome. The following report outlines a case of late onset SLE which initially was suspected to be polymyalgia rheumatica (PMR).

Hearing Loss in Patients With Ankylosing Spondylitis: A Systematic Review and Metaanalysis.

OBJECTIVE: To determine the prevalence of hearing loss (HL) in patients with ankylosing spondylitis (AS) and to describe frequency-specific hearing threshold changes in this patient population compared to patients without AS. METHODS: A systematic review querying 4 databases (PubMed, OVID Medline, Scopus, Cochrane) was performed to identify studies evaluating HL in patients with AS. Metaanalysis was performed to identify overall prevalence rate and OR of HL, as well as to compare mean differences in frequency-specific hearing thresholds between patients with and without AS. RESULTS: Our metaanalysis included 14 studies and 1083 patients (598 with AS vs 485 without AS). The pooled prevalence of HL in patients with AS was 42.4% (95% CI 29.2-56.2). Patients with AS had a significantly higher OR of HL than patients without AS (OR 4.65, 95% CI 2.73-7.91). Mean differences in pure-tone hearing thresholds ranged from 0-5 decibels (dB) for frequencies of 0.25-4 kHz, and from 5-15 dB for frequencies of 6-16 kHz. CONCLUSION: Patients with AS have higher odds of having HL than patients without AS. The AS population also presents with significantly impaired hearing thresholds across all conventional and extended pure-tone frequencies. This may manifest as slight to moderate HL. Results of this systematic review might justify increased attention to audiologic manifestations of patients with AS.

Therapeutic Challenges And Advances In The Management Of Systemic Sclerosis-Related Pulmonary Arterial Hypertension (SSc-PAH).

Systemic sclerosis (SSc) is a rare autoimmune disorder with multi-organ involvement. SSc-associated pulmonary arterial hypertension (SSc-PAH) is one of the leading causes of morbidity and mortality in the SSc population. With advances in our understanding of pulmonary arterial hypertension (PAH) diagnosis and treatment, outcomes for all PAH patients have significantly improved. While SSc-PAH patients have also benefited from these advances, significant challenges remain. Diagnosis of PAH is a challenging endeavor in SSc patients who often have many co-existing pulmonary and cardiac comorbidities. Given the significantly elevated prevalence and lifetime risk of PAH in the SSc population, screening for SSc-PAH is a critically useful strategy. Treatment with pulmonary arterial (PA) vasodilators has resulted in a dramatic improvement in the survival and quality of life of PAH patients. While therapy with PA vasodilators is beneficial in SSc-PAH patients, therapy effects appear to be attenuated when compared to responses in patients with idiopathic PAH (IPAH). This review attempts to chronicle and summarize the advances in our understanding of the optimal screening strategies to identify PAH in patients with SSc. The article also reviews the advances in the therapeutic and risk stratification strategies for SSc-PAH patients.

The Association of Serum 25-Hydroxyvitamin D Status in Patients with Osteoarthritis in the Primary Care Office.

BACKGROUND: Low serum 25-hydroxyvitamin D [25(OH)D] and osteoarthritis (OA) are commonly found in patients followed up in a primary care office. Clear evidence to support the link between 25-hydroxyvitamin D levels and OA is lacking. AIM: To describe the association of serum 25-hydroxyvitamin D status in patients with OA in the primary care office. MATERIALS AND METHODS: We reviewed the records of 1,455 patients seen in our primary care office between November 2013 and October 2014. All patients were older than 18 years and had a diagnosis of OA. Demographic characteristics as well as 25(OH)D levels and comorbidities were analyzed. RESULTS: Levels of 25(OH)D were available in 1,222 patients with OA. Fifty-one percent of the patients had a low 25(OH)D level. Patients with OA and low 25(OH)D were on an average 5 years younger than patients with OA and normal 25(OH)D (P < 0.001). African Americans (71.7%) and Hispanics (63.1%) had a higher prevalence of low 25(OH)D compared to Whites (42.9%) and other races (49.1%) (P < 0.001). There were significantly more smokers (15.4%) and patients with type 2 diabetes (27.6%) in the group of patients with osteoarthritis and low 25(OH)D (P < 0.001). A lower prevalence of hypothyroidism (18.5% versus 27.4%) and higher body mass index (BMI) were also noted in the group of interest. CONCLUSION: Patients with low levels of 25(OH)D and OA are younger than their counterparts with low 25(OH)D level. Future studies are needed to clarify the relationship between 25(OH)D level and OA.

A rheumatoid nodule in an unusual location: mediastinal lymph node.

Rheumatoid arthritis (RA) is a multisystem inflammatory disease characterised by destructive synovitis and varied extra-articular involvement. Rheumatoid lung nodules are the most common pulmonary manifestations of RA. Rheumatoid nodules in mediastinal lymph nodes are extremely uncommon. We describe a male patient with long-standing RA and subcutaneous rheumatoid nodules presenting with multiple lung nodules and mediastinal lymphadenopathies. Definite histopathology of a lymph node was consistent with necrobiotic granuloma due to RA. Clinicians should be aware of rheumatoid nodules as a potential cause of mediastinal lymphadenopathies, mainly in advanced rheumatoid arthritis.