Rodriguez-Pla, Alicia

Rationale: Immunoglobulin (Ig) G4–related disease (IgG4-RD) reportedly has a strong relationship with adult-onset asthma and periocular xanthogranuloma (AAPOX) and may be linked to sclerosing mucoepidermoid carcinoma (MEC). We present a rare case of IgG4-RD and AAPOX occurring in a patient with resected eosinophilic or oncocytic MEC. Patient concerns: A 52-year-old woman was referred to our rheumatology clinic in 2020 to be evaluated for suspected IgG4-RD. Diagnoses: The patient had diagnoses of periorbital xanthelasmas, worsening glucocorticoid-dependent chronic rhinosinusitis and adult-onset asthma, and cervical lymphadenopathy persisting 2 years after resection of a low-grade MEC of a minor salivary gland. Interventions: Because the patient’s symptomatic relief was glucocorticoid dependent, IgG4-RD was suspected, and she was referred to our medical center. Her amylase and lipase levels were elevated. Serum IgG4 levels were initially within normal limits, but IgG4-RD was diagnosed because of the presence of lymphadenopathy and evidence of pancreatitis, which was shown on positron emission tomography/computed tomography. Furthermore, the IgG4 levels later increased without explanation. After the patient began combination therapy with a glucocorticoid (prednisone) and methotrexate, her symptoms improved but recurred when the daily oral glucocorticoid dosage decreased below 10 mg. An excisional biopsy of her right submandibular gland in 2021 yielded results consistent with IgG4-RD. In addition, AAPOX was diagnosed, given the presence of periocular edema and plaques, adult-onset asthma, and rhinosinusitis. Outcome: The patient was carcinoma free at last follow-up and was receiving medication to treat the other conditions. Lessons: The diagnosis of these 3 concomitant, uncommon entities required approximately 7 years of medical investigations. Clinicians should know that IgG4-RD, AAPOX, and MEC may occur together.

We report the case of a 78-year-old woman who presented with cardiovascular risk factors and a history of an atypical transient ischaemic attack. She was referred by her primary care physician to the vascular surgery department at our institution for evaluation of progressive weakness, fatigue, arm claudication and difficulty assessing the blood pressure in her right arm. She was being considered for surgical revascularisation, but a careful history and review of her imaging studies raised suspicion for vasculitis, despite her normal inflammatory markers. She was eventually diagnosed with biopsy-proven giant cell arteritis with diffuse large-vessel involvement. Her symptoms improved with high-dose glucocorticoids.

The current data on rates and geographic distribution of vasculitis mortality are limited. We aimed to estimate the mortality rates of primary systemic vasculitis and its geographic distribution using recent population data in the United States. The mortality rates of vasculitis from 1999 to 2019 were obtained from the Center for Disease Control (CDC) Wonder Multiple Cause of Death (MCD). The age-adjusted rates per million for vasculitis as MCD and as an underlying cause of death (UCD) were calculated by state using demographics. A joinpoint regression analysis was applied to evaluate trends over time. The age-adjusted mortality rate of vasculitis as MCD was 4.077 (95% CI: 4.029–4.125) and as a UCD was 1.888 per million (95% CI: 1.855–1.921). Since 1999, mortality rates have progressively decreased. The age-adjusted mortality rate was higher in females than in males. The highest mortality rate for vasculitis as MCD was in White patients (4.371; 95% CI: 4.317–4.424). The northern states and areas with lower populations had higher mortality rates. We found a trend of progressive decreases in the mortality rates of vasculitis, as well as gender, racial, and geographic disparities. Further analyses are warranted to better understand the factors associated with these disparities in order to implement targeted public health interventions to decrease them.