Title:IgG4 Related Syndrome: Another Multiorgan Disease in the Interest Field of Internal Medicine
Volume: 22
Issue: 30
Author(s): Antonino Tuttolomondo, Irene Simonetta, Domenico Di Raimondo, Tiziana Di Chiara, Roberta Conigliaro, Francesca Corpora, Valerio Vassallo and Antonio Pinto
Affiliation:
Keywords:
Immunoglobulin G4, multiorgan syndrome, type 1 autoimmune pancreatitis, progressive fibrosis, response to glucocortcoids.
Abstract: Background: IgG4-related disease is a rare, clinical and pathologic disease entity
of unknown etiology. Its main features are increased serum concentrations of IgG4 > 1,35
g/l, lymphocyte and IgG4+plasma-cell infiltration within tissues, fibrosis or sclerosis. The
classical presentation of IgG4-RSD is pancreatitis which is combined with the involvement
of biliary ducts in 74 percent of patients. Extrapancreatic manifestations include: abdominal
or mediastinal lymphadenopathy; the involvement of salivary glands and lacrimal glands,
kidneys, lung, retroperitoneum. Since IgG4-related disease is a multiorgan lymphoproliferative
syndrome, it requires a careful differential diagnosis from other distinct disorders (sarcoidosis,
immune rheumatic diseases, hematologic diseases, malignancies). Another distinctive
feature is a fairly fast response to steroids, that represents the first-choice therapy. Immunosuppressant
drugs (azathioprine, mycophenolate mofetil, methotrexate) might be chosen
as glucocorticoid-sparing medications or to maintain steroid-induced remission (Fig. 1).
Methods: We report the case of a 70-year-old man and we performed a brief review of loiterature.
Results: Our patient has a clinical history including bronchial asthma, aortic aneurysm, histologically confirmed
retroperitoneal fibrosis causing hydroureteronephrosis, prostatitis, interstitial pulmonary fibrosis, sclerosing
chronic pancreatitis (histologically documented), previous chronic cholecystitis (histologically confirmed), previous
pericarditis, xeroftalmia, polyclonal hypergammaglobulinemia, eosinophilia. His serum IgG4 levels were
significantly increased (5560 mg/dl). In regard to the above mentioned elements a systemic disease characterized
by elevated serum levels of IgG4 and IgG4-positive lymphoplasmacytic infiltrative lesions in several tissues, was
suspected. Immune-rheumatic diseases and infectious diseases were excluded. Steroid treatment was started
achieving a significant swift response.
Conclusion: Until now IgG4 related disease has been considered rare in the West and exclusive of Japanese and
Korean countries, our case report leads us to reflect on the necessity to take into account this disease in patients
with multisystemic involvement.
