Citations

Background Cerebellar tonsillar ectopia may arise from altered cerebrospinal fluid (CSF) dynamics and can mimic Chiari I malformation. Differentiating between pressure-induced tonsillar descent and congenital malformations is essential. We present an interesting case that visualizes variable tonsillar displacement levels as an indicator of CSF dynamics. Case A 3-year-old boy presented with headache and sixth nerve palsy and was diagnosed with idiopathic intracranial hypertension. Initial magnetic resonance imaging (MRI) revealed a 5 mm descent of the cerebellar tonsils. During hospitalization, the patient underwent mastoidectomy for an incidental T2 hyperintense lesion. Subsequent MRI scans demonstrated the resolution of tonsillar displacement. At age 5, the patient experienced worsening headache. Brain MRI revealed a 13 mm descent of the cerebellar tonsils below the foramen magnum and pachymeningeal enhancement. Further evaluation revealed transtemporal CSF leakage through the previous mastoidectomy site into the soft neck tissues. Conclusion This case exemplifies cerebellar tonsillar displacement associated with both intracranial hypertension (push) and hypotension (pull), illustrating opposite causal directions of CSF dynamics in the same patient. Understanding the phenomenon as a result of dynamic CSF balance rather than unidirectional force or fixed pathophysiology can enhance monitoring of patients.

ABSTRACT Background Hidradenitis suppurativa (HS) is a chronic inflammatory disease associated with substantial impairment in quality of life and psychological well‐being. The impact of cumulative treatment exposure and its relationship with multidimensional disease burden in HS has not been sufficiently discussed. The aim of this study is to investigate the associations between cumulative treatment burden, quality of life impairment, psychological symptom burden, disease activity, and patient‐perceived severity in patients with HS. Methods This multicenter cross‐sectional study included previously treated adult HS patients. Participants completed validated Turkish versions of the Dermatology Life Quality Index (DLQI), Symptom Checklist‐90‐Revised (SCL‐90‐R), and the Hidradenitis Suppurativa Self‐Assessment Tool (HSSA). A Treatment Burden Index (TBI) was developed to quantify cumulative therapeutic exposure based on guideline‐weighted treatment modalities. Results Ninety‐three patients were included (mean age 34.5 ± 11.0 years; 57% male). Higher TBI was moderately correlated with greater QoL impairment (Spearman ρ = 0.401, p < 0.001) and flare frequency. TBI was weakly correlated with psychological symptom burden. DLQI scores were positively associated with flare frequency and SCL‐90‐R scores, whereas the SCL‐90‐R scores were not associated with flare frequency or patient‐perceived disease severity. Conclusions HS burden is multidimensional, with partially independent domains of disease activity, treatment burden, quality of life impairment, and psychological distress. Cumulative treatment exposure is closely linked to QoL impairment and disease activity but only weakly associated with psychological symptom burden. Identification of distinct burden phenotypes may facilitate more personalized and patient‐centered management strategies in HS. image

Objective We provide evidence‐based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). Methods We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. Results Thirty‐five recommendations were generated (including two strong recommendations) for first‐line SARD‐ILD treatment, treatment of SARD‐ILD progression despite first‐line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis–ILD as a first‐line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first‐line ILD treatment in all other SARDs. Conclusion This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

Objective We provide evidence‐based recommendations regarding the treatment of interstitial lung disease (ILD) in adults with systemic autoimmune rheumatic diseases (SARDs). Methods We developed clinically relevant population, intervention, comparator, and outcomes questions. A systematic literature review was then performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A panel of clinicians and patients reached consensus on the direction and strength of the recommendations. Results Thirty‐five recommendations were generated (including two strong recommendations) for first‐line SARD‐ILD treatment, treatment of SARD‐ILD progression despite first‐line ILD therapy, and treatment of rapidly progressive ILD. The strong recommendations were against using glucocorticoids in systemic sclerosis–ILD as a first‐line ILD therapy and after ILD progression. Otherwise, glucocorticoids are conditionally recommended for first‐line ILD treatment in all other SARDs. Conclusion This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the treatment of ILD in people with SARDs.

ObjectiveWe provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease.MethodsWe developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation.ResultsFifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high‐resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6‐minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs.ConclusionThis clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.

Objective We provide evidence‐based recommendations regarding screening for interstitial lung disease (ILD) and the monitoring for ILD progression in people with systemic autoimmune rheumatic diseases (SARDs), specifically rheumatoid arthritis, systemic sclerosis, idiopathic inflammatory myopathies, mixed connective tissue disease, and Sjögren disease. Methods We developed clinically relevant population, intervention, comparator, and outcomes questions related to screening and monitoring for ILD in patients with SARDs. A systematic literature review was performed, and the available evidence was rated using the Grading of Recommendations, Assessment, Development, and Evaluation methodology. A Voting Panel of interdisciplinary clinician experts and patients achieved consensus on the direction and strength of each recommendation. Results Fifteen recommendations were developed. For screening people with these SARDs at risk for ILD, we conditionally recommend pulmonary function tests (PFTs) and high‐resolution computed tomography of the chest (HRCT chest); conditionally recommend against screening with 6‐minute walk test distance (6MWD), chest radiography, ambulatory desaturation testing, or bronchoscopy; and strongly recommend against screening with surgical lung biopsy. We conditionally recommend monitoring ILD with PFTs, HRCT chest, and ambulatory desaturation testing and conditionally recommend against monitoring with 6MWD, chest radiography, or bronchoscopy. We provide guidance on ILD risk factors and suggestions on frequency of testing to evaluate for the development of ILD in people with SARDs. Conclusion This clinical practice guideline presents the first recommendations endorsed by the American College of Rheumatology and American College of Chest Physicians for the screening and monitoring of ILD in people with SARDs.