IgG4 Related Disease

Think of Mr. G for IgG4 Related Disease. Think Head to Toe:

• associated with allergies, rhinitis and asthma

• involves the eyes: retro-orbital tumor or periorbital edema

• Mikulicz syndrome describes enlargement of head & neck glands (salivary and lacrimal glands)

• affects the thyroid (Riedel's thyroiditis)

• autoimmune pancreatitis and sclersoing cholangitis

• aortitis/ periaortitis

• renal involvement (tubulointerstitial nephritis)

• Retro peritoneal fibrosis

Fibrosis in IgG4 related disease is described as storiform (like a swirly storm) arrangement of fibroblasts and inflammatory cells. Workup includes CT CAP, urinalysis, C3/C4, serum Ig, and biopsy. Treat with a prednisone taper and for second line use rituximab.