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

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.

The differential diagnosis in patients with generalized lymphadenopathy includes sarcoidosis, multicentric Castleman disease, infection (eg, tuberculosis), and lymphoma or other malignancy. IgG4-related lymphadenopathy is distinguished from these conditions by the modest lymph node enlargement, histologic distinctions on biopsy, lack of constitutional features, and the usually striking clinical response to glucocorticoids [ 9 ]. Patients with bilateral hilar adenopathy may mimic sarcoidosis. (See "Evaluation of peripheral lymphadenopathy in adults" and "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Epidemiology, pathologic features, and diagnosis of classical Hodgkin lymphoma" and 'Lung and pleural disease' below and "Multicentric Castleman's disease" .)

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

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

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