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.

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.

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 four databases (PubMed, Ovid MEDLINE, SCOPUS, Cochrane) was performed to identify studies evaluating HL in patients with AS. Meta-analysis was performed to identify overall prevalence rate and odds ratio (OR) of HL, as well as to compare mean differences in frequency-specific hearing thresholds between patients with and without AS. Results This meta-analysis included 14 studies and 1,083 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 in patients with AS over patients without AS (4.65 OR, 95% CI 2.73 – 7.91). Mean differences in pure tone hearing thresholds ranged from 0-5 decibel (dB) for frequencies 0.25-4kHz and from 5-15 dB for frequencies 6-16 kHz. Conclusion Patients with AS have higher odds of having HL over patients without AS. This population also presents with significantly impaired hearing thresholds across all conventional and extended pure tone frequencies. This may manifest as slight to moderate hearing loss. Results of this systematic review might justify increased attention audiologic manifestations of patients with AS.

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 I 2 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.

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.