ABSTRACT
IgG4-related disease-associated hypertrophic pachymeningitis (IgG4RD-HP) is a fibroinflammatory autoimmune disorder in which diagnosis is difficult without biopsy. Guidance on management of disease refractory to glucocorticoids and intravenous rituximab is limited. We present the case of a 68-year-old woman with IgG4RD-HP who developed sensorineural hearing loss with associated bulky basilar pachymeningeal enhancement. Her cerebrospinal fluid was inflammatory and had an elevated IgG4 concentration, strongly suggestive of IgG4RD-HP. Biopsy of involved meninges was not possible due to surgical risk. Over years she developed bilateral optic neuropathies and hydrocephalus, requiring intravenous rituximab and ventriculoperitoneal shunt. Her disease was refractory to glucocorticoids. Despite maintenance intravenous rituximab, she developed slowly progressive symptoms of intracranial hypertension and hydrocephalus with persistently inflammatory spinal fluid. Switching to intrathecal rituximab therapy led to dramatic improvement in gait and headache and reduced pachymeningeal bulk and metabolic activity. In patients with IgG4RD-HP refractory to glucocorticoids and intravenous rituximab, intrathecal rituximab may be an efficacious therapy.
ABSTRACT
OBJECTIVES: The COVID-19 pandemic disrupted the delivery of preventative care and management of acute diseases. This study assesses the effect of the COVID-19 pandemic on coronary calcium score and coronary CT angiography imaging volume. MATERIALS AND METHODS: A single institution retrospective review of consecutive patients presenting for coronary calcium score or coronary CT angiography examinations between January 1, 2020 to January 4, 2022 was performed. The weekly volume of calcium score and coronary CT angiogram exams were compared. RESULTS: In total, 1,817 coronary calcium score CT and 5,895 coronary CT angiogram examinations were performed. The average weekly volume of coronary CTA and coronary calcium score CT exams decreased by up to 83% and 100%, respectively, during the COVID-19 peak period compared to baseline (P < 0.0001). The post-COVID recovery through 2020 saw weekly coronary CTA volumes rebound to 86% of baseline (P = 0.024), while coronary calcium score CT volumes remained muted at only a 53% recovery (P < 0.001). In 2021, coronary CTA imaging eclipsed pre-COVID rates (P = 0.012), however coronary calcium score CT volume only reached 67% of baseline (P < 0.001). CONCLUSIONS: A significant decrease in both coronary CTA and coronary calcium score CT volume occurred during the peak-COVID-19 period. In 2020 and 2021, coronary CTA imaging eventually superseded baseline rates, while coronary calcium score CT volumes only reached two thirds of baseline. These findings highlight the importance of resumption of screening exams and should prompt clinicians to be aware of potential undertreatment of patients with coronary artery disease.
