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AB0985 Methotrexate as induction of remission therapy for localized manifestations of IGG4-related disease
  1. L Rovati1,
  2. E Della-Torre1,
  3. M Lanzillotta1,
  4. E Bozzalla Cassione1,
  5. E Bozzolo1,
  6. C Canevari2,
  7. M Falconi3,4,
  8. L Dagna1,4
  1. 1Unit of Immunology, Rheumatology, Allergy and Rare Diseases
  2. 2Unit of Nuclear Medicine
  3. 3Pancreatic Surgery Unit, San Raffaele Scientific Institute
  4. 4Università Vita-Salute San Raffaele, Milan, Italy


Background Medium to high dose glucocorticoids represents the treatment of choice for inducing remission in IgG4-related disease (IgG4-RD) patients1. However, clinicians might prefer alternative equally effective drugs in clinical settings where long-term corticosteroids treatment is contraindicated, such as diabetes or osteoporosis. We recently reported the efficacy of methotrexate in maintaining glucocorticoids induced IgG4-RD remission2.

Objectives To evaluate the efficacy of methotrexate as induction of remission therapy in selected cases of mild and localized IgG4-RD complicated by clinical scenarios that might advise against corticosteroids treatment.

Methods Five patients with active untreated IgG4-RD were started on oral or subcutaneous methotrexate (up to 15–20 mg/week). Efficacy of methotrexate in inducing remission was assessed at 6 months by 18F-FDG PET/CT scan and by measuring the IgG4-RD Responder Index (RI)3 and circulating plasmablasts4. Partial response (PR) corresponded to an improvement of the IgG4-RD RI >2 points. Complete response (CR) corresponded to an IgG4-RD RI score <3.

Results All patients were males with a mean age of 67 years (range 53–78). Two had pancreatic involvement; one had lymph node enlargement; one had pancreatic and lymph node involvement; one had pancreatic, aortic, submandibular gland and lymph node involvement. Patients with pancreatic involvement presented with increased serum amylases or abdominal discomfort; none had obstructive jaundice; all had overt diabetes. The mean IgG4-RD RI, serum IgG4 concentration and plasmablasts counts at baseline were 8 (6–15), 483 mg/dL (136–983) and 3336/mL (330–9330 /mL), respectively. All patients had increased 18F-FDG uptake on PET/CT scan within the affected organs. After 6 months of methotrexate, Patients 1, 2, and 3 were on CR with improved or normalized PET/CT findings, serum IgG4 and plasmablasts levels. Patient 5 achieved PR, showing improved 18F-FDG-PET/CT findings, normal plasmablasts level, but stable serum IgG4 concentration; after 10 months of methotrexate, persistence of disease activity prompted the introduction of glucocorticoids. Methotrexate was stopped in Patient 4 after 5 months because of nausea and vomiting; at 6 months he showed persistently increased plasmablasts count and 18F-FDG uptake on PET/CT, thus requiring a rescue therapy with glucocorticoids. (Table 1)

Conclusions In localized forms of IgG4-RD with mild manifestations, methotrexate represents a promising alternative strategy for inducing disease remission, especially in the presence of contraindications to glucocorticoids.


  1. Khosroshahi A, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015.

  2. Della-Torre E, et al. Methotrexate for maintenance of remission in IgG4-related disease. Rheumatology (Oxford). 2015.

  3. Carruthers MN, et al. Development of an IgG4-RD Responder Index. Int J Rheumatol. 2012.

  4. Wallace ZS, et al. Plasmablasts as a biomarker for IgG4-related disease, independent of serum IgG4 concentrations. Ann Rheum Dis. 2015.


Disclosure of Interest None declared

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