Manifestations of Skull Base IgG4-Related Disease: A Multi-Institutional Study.

OBJECTIVE: IgG4-related disease (IgG4-RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor-like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4-RD. METHODS: Review of all cases at three tertiary-referral centers since disease description in 2003. RESULTS: Eleven patients were identified at a median age at presentation of 58 years (IQR, 38-65; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6/11) with extension to the petroclival junction in 50% (3/6). Infiltration of the internal auditory canal was present in 27% (3/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma / multiple meningioma syndrome. Local biopsy demonstrated >30 IgG4-positive plasma cells per high-powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy. CONCLUSIONS: IgG4-RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:2574-2580, 2020.

Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients.

Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.

"In-house" pharmacological management for computed tomography coronary angiography: heart rate reduction, timing and safety of different drugs used during patient preparation.

We retrospectively evaluated the effect, timing and safety of different pharmacological strategies during 64-slice CT coronary angiography (CT-CA). From the institutional database of CT-CAwe enrolled 560 consecutive patients with suspected coronary artery disease. The type of drug preparation (group 1 = no treatment; group 2 = oral metoprolol; group 3 = other; group 4 = intravenous (IV) atenolol; group 5 = IV atenolol + nitrates; NR = non-responders), timing, and adverse effects were recorded. Heart rate (HR) during different preparation phases was recorded. Four adverse effects were recorded, none of which was attributable to pharmacological treatment. In all groups, except group 1, the HR on arrival was significantly reduced by the pharmacological treatment (p<0.01). Group 4 showed the best (-16±8 bpm) HR reduction. There was no significant effect on HR due to nitrates (p = 0.49), while a slight increase due to contrast material was noted (p<0.05). Average time required for preparation was 44±25min. Groups 4 and 5 showed the most effective timing (8±9 min and 8±8 min, respectively; p<0.01). Pharmacological preparation in patients undergoing CT-CA is safe and effective. Best results in terms of HR reduction and fast preparation are obtained with IV administration of beta-blockers.

Computed tomography coronary angiography plaque burden in patients with suspected coronary artery disease.

PURPOSE: To determine the relationship between established cardiovascular risk factors, clinical presentation and the extent of coronary artery disease (CAD), as described with computed tomography coronary angiography. MATERIAL AND METHODS: In this cross-sectional study, we included 567 symptomatic individuals without a history of CAD who consecutively underwent 64-slice computed tomography coronary angiography for evaluation of suspected CAD. We analyzed the prevalence of CAD depending on sex, age, symptoms and risk factors. RESULTS: A total of 8542 segments were analyzed. No evidence of CAD was observed in 225 patients (40%), nonsignificant CAD in 221 patients (39%) and significant CAD (luminal narrowing >50%) in the remaining 121 patients (21%). CAD increased with advancing age, significantly above 50 years (P < 0.05). Female patients had a higher prevalence of normal coronary arteries and males of significant CAD (P < 0.01). With the increase of risk factors, there was a significant increase of the significant disease (P < 0.01). Typical pain with respect to atypical pain had the strongest association with significant CAD (16 vs. 38%; P < 0.05). In multivariate analysis, the number of risk factors, age, male sex and typical pain remained strong predictors of significant CAD (P < 0.0001). CONCLUSION: Computed tomography coronary angiography may play an important role in risk stratification of patients with suspected CAD.