IgG4-related Disease: 2013 Update
- PDF / 158,644 Bytes
- 10 Pages / 595.276 x 790.866 pts Page_size
- 30 Downloads / 180 Views
Vascular Disease (H Gornik and E Kim, Section Editors)
IgG4-related Disease: 2013 Update Paul A. Monach, MD, PhD Address Vasculitis Center and Section of Rheumatology, Boston University School of Medicine, 72 East Concord Street, E-533, Boston, MA 02118, USA Email: [email protected]
Published online: 8 February 2013 * Springer Science+Business Media New York 2013
Keywords IgG4-Related disease
I
Aortitis
I
Periaortitis
I
Retroperitoneal fibrosis
I
Autoimmune pancreatitis
Opinion statement Having diagnosed a patient as having IgG4-related disease, I would have a low threshold for recommending immune-suppressive treatment, and would make that recommendation for any patient with vascular involvement. My initial approach would be prednisone at 40–60 mg/day with a plan to reduce the dose every two weeks, e.g., 40, 30, 20, 15, 10, 7.5, 5, and 2.5 mg for 2 weeks each. In the event of relapse, I would double the current prednisone dose, slow the taper, and add azathioprine, anticipating using that drug for one year if the patient were to remain in remission. In the event or subsequent relapse, I would stop azathioprine and use rituximab. In a patient with large artery involvement, I would consult a vascular surgeon soon after diagnosis, anticipating a need for surgical repair.
Introduction Over the past decade, researchers from multiple specialties have discovered that a group of rare fibro-inflammatory diseases of diverse organ systems share pathologic features, including a striking prominence of plasma cells secreting the immunoglobulin subtype IgG4 [1•]. A recent consensus conference produced a recommendation to use the term “IgG4-related disease” to encompass these entities, and to add “IgG4-related” to terms denoting inflammation in individual organ systems [1•]. Those organ systems are numerous and include the pancreas, orbit (including lacrimal gland, extraocular muscles, and/or other soft tissues producing an orbital pseudotumor), salivary glands, meninges, pituitary, thyroid, lymph nodes, lungs (parenchyma or pleura), kidney (tubulointerstitial
nephritis), biliary tree, gall bladder, liver, breast, prostate, skin, peripheral nerve, and—of greatest importance to cardiovascular physicians and surgeons— pericardium [2–4], mediastinum [2], retroperitoneum [5, 6], aorta [5–7, 8•], and large arteries [9]. It is likely that involvement of additional organ systems by IgG4related disease will be confirmed. Many patients have only one organ system involved (at least on clinical grounds), but the majority have two or more organs involved, although involvement of numerous organ systems is uncommon [10, 11]. Many patients with pancreatitis also have sclerosing cholangitis (70 %) and/or sialadenitis (40 %), but less than 20 % of patients presenting with sialadenitis also have pancreatitis [11, 12]. Involvement of addi-
IgG4-related Disease: 2013 Update tional organs can occur over time [13]. Since the disease was defined recently, the overall prevalence of this disease and involvement of specific organs is uncertain.
Data Loading...
