IgG4-related systemic disease and lymphoplasmacytic aortitis.
We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4-related systemic disease, linking IgG4-related systemic disease with a newly-recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in situ hybridization, the plasma cells showed polytypic staining for kappa and lambda light chains, consistent with a polyclonal plasma cell infiltrate. Serologic evaluation revealed that the patient's IgG4 levels were elevated nearly 10-fold. Four years before aortic surgery, the patient had undergone a mediastinal lymph node biopsy. Reexamination of the lymph node revealed features consistent with IgG4-related systemic disease, which had not been recognized at the time of the original biopsy. Glucocorticoid therapy for the IgG4-related systemic disease yielded a prompt response. Recognition that IgG4-related systemic disease can involve the ascending as well as the descending abdominal aorta indicates the need for a change in the way idiopathic aortitis is regarded. This case offers new potential considerations for short- and long-term management of noninfectious aortitis, because of the frequent good response of IgG4-related systemic disease to glucocorticoid treatment without additional therapy. Treatment of the aortitis may prevent progression of the IgG4-related systemic disease to involvement of other organs. IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology.
rhumatology Unit, Massachusetts General Hospital, Boston, MA 02114, USA. jhstone@partners.org
John H
JH
Stone
Arezou
A
Khosroshahi
Alan
A
Hilgenberg
Amy
A
Spooner
Eric M
EM
Isselbacher
James R
JR
Stone
D000368:Aged; D000784:Aortic Dissection / Q000601:surgery; D001011:Aorta / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery; D001025:Aortitis / Q000175:diagnosis* / Q000601:surgery; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007154:Immune System Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D007074:Immunoglobulin G / Q000378:metabolism*; D008297:Male; D011241:Prednisone / Q000627:therapeutic use
D005938:Glucocorticoids; D007074:Immunoglobulin G; D011241:Prednisone