Igg4-related sclerosing disease of the breast successfully treated by steroid therapy

The . Pharmacopoeial Convention has concluded that rituximab is indicated for treatment of Waldenström’s macroglobulinemia.  Dimopolous et al (2002) reported on 27 patients with symptomatic Waldenström’s macroglobulinemia who were treated with rituximab.  Twelve patients (44 %; 95 % confidence interval [CI]:  % to %) achieved a partial response after treatment with rituximab.  Median time to response was months (range of  to months).  The median time to progression for all patients was 16 months, and with a median follow-up of months, 9 of 12 responding patients remain free of progression.  The investigators reported that approximately 25 % of patients experienced some mild form of infusion-related toxicity, usually fever and chills. 

Relapses are common, and a previous history of relapse appears to be a strong predictor of future relapse. When relapse occurs while off therapy and there has been a prolonged disease remission following initial glucocorticoid induction, then the relapse can usually be managed successfully with a re-induction strategy using glucocorticoids. Introducing a steroid-sparing agent might also need to be considered for relapses; however, none has been tested in prospective, controlled studies, and evidence for their efficacy beyond that offered by concomitant glucocorticoid therapy is scarce. [5]

Some have advocated supportive therapy for abdominal pain on the premise that fibrosis and scarring ultimately progress to pancreatic burnout and spontaneous relief of pain. Although long-term improvement in pain has been observed in some patients with CP, a significant subset of patients experiences debilitating pain for decades. The AGA technical review has stated that "a strategy of waiting for spontaneous pain relief is not reliable and may be unreasonable advice for the patient with persistent, severe pain." Furthermore, there is growing evidence that there is no association between duration of CP and improvement in pain. Medical options for pain relief include abstinence from alcohol and smoking, analgesics, and pancreatic enzymes. Abstinence from alcohol is critical because continued use can hasten disease progression, aggravate chronic pain, and increase mortality. Non-narcotic analgesics (., nonsteroidal anti-inflammatory drugs, acetaminophen, and tramadol) are the next step in managing painful CP. The role of smoking in the progression of fibrosis and functional impairment has been established in recent studies. Therefore smoking cessation should also be strongly advised. If pain persists, low doses of mild narcotics may be added. Severe or recalcitrant pain can warrant the use of stronger opiates in selected cases.

Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

Igg4-related sclerosing disease of the breast successfully treated by steroid therapy

igg4-related sclerosing disease of the breast successfully treated by steroid therapy

Based on histological and immunohistochemical examination of various organs of patients with autoimmune pancreatitis (AIP), a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4-related sclerosing diseases. This disease includes AIP, sclerosing cholangitis, cholecystitis, sialadenitis, retroperitoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AIP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.

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