IgG4-related aortitis/periaortitis and periarteritis: a distinct spectrum of IgG4-related disease

Abstract

Background: Large vessels could be involved in IgG4-related disease(IgG4-RD).This study aimed to clarify the clinical features and evaluate the treatment efficacy for IgG4-RD with aortitis/periaortitis and periarteritis (PAO/PA). Methods: This study enrolled 587 IgG4-RD patients in a prospective cohort with a follow-up time for more than 6 months. The distribution of IgG4-related PAO/PA was classified into four types: type 1, thoracic aorta; type 2a, abdominal aorta; type 2b, abdominal aorta and iliac artery; type 2c, iliac artery; type 3, thoracic and abdominal aorta; type 4, other arteries. Patient’s demographic data, clinical characteristics, laboratory parameters, and treatment efficacy were analyzed. Results: Of 587 IgG4-RD patients, 89(15.2%) had PAO/PA. The average age was 58.3±11.1 years, with male predominance (85.4%). Vessels affected were as follows: abdominal aorta (83.1%), iliac artery (70.8%), thoracic aorta (13.5%) and other vessels (13.5%). The most prevalent distribution type of IgG4-related PAO/PA was type 2b, with 74 (83.1%) patients, followed by type 2a, type 2c, type 3, and type 1. 55 (61.8%) PAO/PA patients had hydronephrosis, with renal insufficiency occurred in 43 (48.3%), and 31 (34.8%) PAO/PA patients had D-J stent drainage due to severe ureteral obstruction. After treatment with glucocorticoid and immunosuppressants, 82% patients achieved a remission with shrinking of perivascular mass. Conclusions: IgG4-RD with PAO/PA was distinct from non-PAO/PA in demographic features, organs involvement distribution, inflammatory markers, serum IgG4 and IgE. The most common affected vessel was abdominal aorta, and most patients responded well with treatment.

Marfan syndrome resulting from a rare pathogenic FBN1 variant, ascertained through a proband with IgG4‐related arteriopathy

“…The most frequently involved blood vessel is the aorta (abdominal more often than thoracic) with aortitis and retroperitoneal fibrosis (Peng et al, 2020). Medium‐sized to large arteries can also be involved; 4–10% of all cases of IgG4‐RD and 15–25% of cases of IgG4‐RD with aortic involvement.…”

“…Arterial wall thickening is characteristic but aneurysms and stenoses occur. The most common extra‐aortic sites are the iliac and coronary arteries (Fragoulis, Evangelatos, & Tektonidou, 2020; Ozawa et al, 2017; Peng et al, 2020).…”

“…Both disorders can affect the aorta; in MFS the aortic root/ascending thoracic aorta is involved, while in IgG4‐RD it is most oftenthe infrarenal aorta. Age at onset may also help to distinguish the two disorders: in MFS, aortic root dilatation may start as early as prenatal life, is often present in childhood and teenage and in at least 80% of those affected by 40 years, although it may be first detectable up to 70 years (Aburawi & O'Sullivan, 2007; Vanem et al, 2020); by contrast aortic involvement is later in IgG4‐RD, with mean ages in several studies ranging from 55 to 67 years (Lanzillotta, Campochiaro, et al, 2020; Ozawa et al, 2017; Peng et al, 2020; Perugino et al, 2016).…”

“…Large and medium‐sized extra‐aortic arteries may be involved in both disorders: in MFS, the iliac, subclavian, femoral, intercostal, carotid and visceral arteries can be affected (Awais et al, 2013; Engelfriet et al, 2006; Gaertner et al, 2014; Mariucci et al, 2013; Pyeritz, 2019; Schoenhoff et al, 2019; Yetman et al, 2011) and in IgG4‐RD, the iliac, coronary, carotid, subclavian, vertebral, femoral, popliteal, pulmonary, and visceral arteries can be affected (Fragoulis et al, 2020; Ozawa et al, 2017; Peng et al, 2020).…”