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IgG4 Related Disease - An Italian Experience

June 12 2015 1:11 PM ET via RheumReports RheumReports

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells, storiform fibrosis and often, but not always, elevated serum IgG4 levels (found in 60-70%). Clinical manifestations can involve pretty much any organ in the body and most patients will respond to corticosteroids. Although the condition was not truly identified until 2003, most of us have been treating patients for years, without a clear diagnosis. The most common clinical manifestations include autoimmune pancreatitis, sclerosing cholitis, sclerosing sialadenitis, thyroiditis, retroperitoneal fibrosis, aortitis, tubulointerstitial nephritis and orbital disease.

The majority of patients will respond to treatment with moderate doses of prednisone, but they often have symptom relapse when prednisone is tapered. The best data available on steroid-sparing therapy comes from case series, most commonly with the use of B-cell depleting therapy (rituximab). Case reports and small case series have shown success with azathioprine and MMF.

Dr. EmanuelDella-Torre et al presented data from their centre in Milan, which aimed to assess the efficacy of methotrexate in relapsing IgG4-RD with systemic involvement. They included 10 subjects with biopsy-proven IgG4-RD followed between January 2008 and May 2014. Organ involvement included pancreatitis, sclerosing cholangitis, aortitis, sialadenitis, pachymeningitis and retroperitoneal fibrosis. On average, relapse occurred around 7 months after starting prednisone, on a mean dose of 9.6 mg daily. At the time of relapse, MTX 20mg (up to 20 mg/week) was added and prednisone was increased to 20mg daily, followed by a slow taper. Of the 10 patients, 3 were in clinical remission off prednisone at 6 months, and a further 2 were able to stop prednisone by 12 months. The remaining 5 patients were in clinical remission at 12 months, but required 5 mg daily of prednisone with their MTX. Based on this experience from Milan, MTX seems to be a viable immunosuppressive option for maintaining steroid-induced remission in patients with IgG4-RD.

As we all gain more clinical experience with this condition,we will hopefully become more comfortable knowing when and how to withdraw therapy. Even though this was a small case series, the success they had with MTX is promising and can inform how we practice.


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About the Author

Dr. Shahin Jamal
Dr. Shahin Jamal

Dr. Jamal is a Clinical Associate Professor at the University of British Columbia and an active staff physician at Vancouver Coastal Health. Her interests include diagnosis and prognosis of early inflammatory arthritis, and timely assessment and access to care for patients with rheumatoid arthritis.

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