Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study

Abstract

IntroductionImmunoglobulin G4 (IgG4)–related aortitis/periaortitis and periarteritis are vascular manifestations of IgG4-related disease. In this disease, the affected aneurysmal lesion has been suspected to be at risk of rupture. In this study, we aimed to clarify the clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis.MethodsWe retrospectively evaluated clinical features, including laboratory data, imaging findings and the course after corticosteroid therapy, in 40 patients diagnosed with IgG4-related aortitis/periaortitis and periarteritis on the basis of periaortic/periarterial radiological findings, satisfaction of the comprehensive diagnostic criteria or each organ-specific diagnostic criteria, and exclusion of other diseases.ResultsThe patients were mainly elderly, with an average age of 66.4 years and with a marked male predominance and extensive other organ involvement. Subjective symptoms were scanty, and only a small proportion had elevated serum C-reactive protein levels. The affected aorta/artery were the abdominal aortas or the iliac arteries in most cases. Thirty-six patients were treated with prednisolone, and the periaortic/periarterial lesions improved in most of them during the follow-up period. Two (50.0%) of four patients with luminal dilatation of the affected lesions before corticosteroid therapy had exacerbations of luminal dilatation after therapy, whereas none of the twenty-six patients without it had a new appearance of luminal dilatation after therapy.ConclusionsThe results of this retrospective multicenter study highlight three important points: (1) the possibility of latent existence and progression of periaortic/periarterial lesions, (2) the efficacy of corticosteroid therapy in preventing new aneurysm formation in patients without luminal dilatation of periaortic/periarterial lesions and (3) the possibility that a small proportion of patients may actually develop luminal dilatation of periaortic/periarterial lesions in IgG4-related aortitis/periaortitis and periarteritis. A larger-scale prospective study is required to confirm the efficacy and safety of corticosteroid therapy in patients with versus those without luminal dilatation and to devise a more useful and safe treatment strategy, including administration of other immunosuppressants.

Endovascular Repair of a False Aneurysm Developing from IgG4-Related Periaortitis during Corticosteroid Therapy

“…3 Meanwhile, IgG4-related periaortitis has been considered to pose little risk of aortic rupture because of the lack of aortic dilatation. 9 However, a recent report described a case of a false aneurysm developing from IgG4-related periaortitis caused by corticosteroid therapy, just as that observed in our present case. 10 In both cases, endovascular repair was a successful initial treatment for the false aneurysm.…”

Inflammatory diseases of the aorta

“…An observational study in IgG4-positive periaortitis showed a signifi cant reduction of infl ammatory parameters and vessel wall enlargement under variable prednisolone treatments, initiated with 15 -50 mg/d and tapered to 5 -10 mg/d during 6 -12 months. Aortic wall diameter decreased by 50 % within the fi rst two months of treatment; after that a further decrease was observed in half of the patients [73]. There were a few patients with IAA in whom aneurysmatic dilation increased despite reduction of the adventitial infl ammatory border.…”

Immunoglobulin G4-Related Cardiovascular Diseases

“…Mizushima and colleagues highlighted three aspects relating to corticosteroid therapy and clinical course in IgG4-related vascular diseases: 1) the possibility of latent existence and progression, 2) the efficacy of corticosteroid therapy in preventing new aneurysm formation in patients without luminal dilatation, and 3) the possibility that a small proportion of patients may actually develop luminal dilatation. 50) The therapeutic corticosteroid dosage required to treat IgG4-related diseases differs case-by-case, and Tajima and colleagues have suggested that treatment of IgG4-related cardiovascular diseases might require higher doses of corticosteroids than other IgG4-related diseases. 51) IgG4-related diseases can involve any organs.…”