Question:
A patient with IgG4-related disease with pancreatitis initially responded to steroid therapy but experienced a relapse (gland size increased) after tapering. What is the next step in treatment?
A patient with IgG4-related disease with pancreatitis initially responded to steroid therapy but experienced a relapse (gland size increased) after tapering. What is the next step in treatment?
Show Hint
IgG4-RD classically presents as autoimmune pancreatitis (Type 1), Mikulicz disease (salivary/lacrimal gland enlargement), or retroperitoneal fibrosis. The histology triad includes: 1) dense lymphoplasmacytic infiltrate, 2) storiform fibrosis, 3) obliterative phlebitis. Rituximab is the definitive steroid-sparing agent.
Updated On: Sep 3, 2026
- High dose steroids
- Rituximab
- Methotrexate
- dapsone
Show Solution
Verified By Collegedunia
The Correct Option is B
Solution and Explanation
Concept:
IgG4-Related Disease (IgG4-RD) is a systemic fibro-inflammatory condition that forms tumefactive lesions in various organs (commonly pancreas, salivary glands, retroperitoneum), characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis.
While highly responsive to glucocorticoids initially, relapses are common upon tapering.
Explanation:
• The first-line induction therapy for IgG4-RD is systemic glucocorticoids. The response is often rapid and dramatic. However, up to 40-50% of patients relapse when steroids are tapered or discontinued.
• B-cell depletion therapy is highly effective in IgG4-RD because B cells and plasmablasts are central to its pathogenesis. By depleting the B-cell precursors, the continuous supply of short-lived plasmablasts driving the disease is halted.
• Rituximab (an anti-CD20 monoclonal antibody) is the preferred and most effective agent for steroid-refractory, relapsing, or high-risk IgG4-RD. It is the established next step to induce remission and act as a steroid-sparing agent.
• Option (A), High dose steroids, could temporarily treat the relapse, but restarting high-dose steroids without adding a steroid-sparing agent in a patient who already failed a taper is poor practice and leads to unacceptable glucocorticoid toxicity.
• Option (C), Methotrexate, and other conventional synthetic DMARDs (like Azathioprine or Mycophenolate) have been used, but their efficacy in IgG4-RD is variable and inferior to B-cell depletion. Rituximab is strongly preferred.
• Option (D), Dapsone, has no established role in the treatment of IgG4-RD.
Final answer:
Rituximab is the treatment of choice for relapsing IgG4-related disease after a steroid taper.
IgG4-Related Disease (IgG4-RD) is a systemic fibro-inflammatory condition that forms tumefactive lesions in various organs (commonly pancreas, salivary glands, retroperitoneum), characterized by dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells and storiform fibrosis.
While highly responsive to glucocorticoids initially, relapses are common upon tapering.
Explanation:
• The first-line induction therapy for IgG4-RD is systemic glucocorticoids. The response is often rapid and dramatic. However, up to 40-50% of patients relapse when steroids are tapered or discontinued.
• B-cell depletion therapy is highly effective in IgG4-RD because B cells and plasmablasts are central to its pathogenesis. By depleting the B-cell precursors, the continuous supply of short-lived plasmablasts driving the disease is halted.
• Rituximab (an anti-CD20 monoclonal antibody) is the preferred and most effective agent for steroid-refractory, relapsing, or high-risk IgG4-RD. It is the established next step to induce remission and act as a steroid-sparing agent.
• Option (A), High dose steroids, could temporarily treat the relapse, but restarting high-dose steroids without adding a steroid-sparing agent in a patient who already failed a taper is poor practice and leads to unacceptable glucocorticoid toxicity.
• Option (C), Methotrexate, and other conventional synthetic DMARDs (like Azathioprine or Mycophenolate) have been used, but their efficacy in IgG4-RD is variable and inferior to B-cell depletion. Rituximab is strongly preferred.
• Option (D), Dapsone, has no established role in the treatment of IgG4-RD.
Final answer:
Rituximab is the treatment of choice for relapsing IgG4-related disease after a steroid taper.
Was this answer helpful?
0
0
Top NEET SS Medical Questions
- A liver transplant patient presents with diarrhoea, colitis, a total leukocyte count of 1800, and a positive blood CMV PCR. What is the next step in management?
- A 32-year-old male recovering from dengue fever initially showed clinical improvement but now presents with recurrence of high-grade fever, fatigue, and pancytopenia on day 10 of illness. He is hemodynamically stable with no bleeding manifestations. Which of the following investigations should be performed first to evaluate the cause of relapse and cytopenia?
- A 48-year-old man with chronic hepatitis C infection is being evaluated for risk factors associated with progression of liver disease. He has a 20-year history of infection, drinks alcohol occasionally, and has a high serum HCV RNA viral load. Which of the following factors is not associated with an increased risk of progression of liver disease in chronic HCV infection?
- A 28-year-old veterinary worker who previously received a full 3-dose pre-exposure rabies vaccination series is bitten by a stray dog while handling an animal in the clinic. The wound is cleaned immediately. What is the most appropriate post-exposure management?
- A 42-year-old patient undergoing chemotherapy for acute myeloid leukemia develops neutropenia (ANC $<$ 500) and is treated with broad-spectrum antibiotics. After initial recovery of neutrophil counts (ANC = 1500), he again develops high-grade fever and right upper quadrant abdominal pain. Imaging shows multiple small hypodense lesions in the liver and spleen. What is the most likely diagnosis?
View More Questions
Top NEET SS Rheumatology Questions
- A 7-year-old unvaccinated child presents with a history of sore throat and fever 3 weeks ago. The child now develops nasal regurgitation of fluids, facial deviation, and tongue weakness with numbness. On examination, there is palatal paralysis and a nasal twang to the voice. What is the most likely diagnosis?
- A 45-year-old woman with long-standing rheumatoid arthritis (RA) develops numbness, paresthesia, non-healing leg ulcers, and absent ankle reflexes. She is on regular RA treatment. What is the most probable diagnosis?
- Rapidly progressive interstitial lung disease is associated with which of the following myositis specific antibodies?
- 42-year-old female, with diffuse cutaneous systemic sclerosis, presents with SOB/cough. CXR shows ILD. FVC 50% predicted, DLCO 30% predicted. ECG and imaging shows right ventricular enlargement and dilated PA s/o pulmonary hypertension. Most appropriate next step in management is?
- 60-year old male with RA on MTX for 5 years presents with SOB and cough. Chest examination reveals end-inspiratory crackles. HRCT shows reticular opacities and traction bronchiectasis (UIP pattern). Which of the following is correct?
View More Questions
Top NEET SS Questions
- A liver transplant patient presents with diarrhoea, colitis, a total leukocyte count of 1800, and a positive blood CMV PCR. What is the next step in management?
- A 32-year-old male recovering from dengue fever initially showed clinical improvement but now presents with recurrence of high-grade fever, fatigue, and pancytopenia on day 10 of illness. He is hemodynamically stable with no bleeding manifestations. Which of the following investigations should be performed first to evaluate the cause of relapse and cytopenia?
- A 48-year-old man with chronic hepatitis C infection is being evaluated for risk factors associated with progression of liver disease. He has a 20-year history of infection, drinks alcohol occasionally, and has a high serum HCV RNA viral load. Which of the following factors is not associated with an increased risk of progression of liver disease in chronic HCV infection?
- A 28-year-old veterinary worker who previously received a full 3-dose pre-exposure rabies vaccination series is bitten by a stray dog while handling an animal in the clinic. The wound is cleaned immediately. What is the most appropriate post-exposure management?
- A 42-year-old patient undergoing chemotherapy for acute myeloid leukemia develops neutropenia (ANC $<$ 500) and is treated with broad-spectrum antibiotics. After initial recovery of neutrophil counts (ANC = 1500), he again develops high-grade fever and right upper quadrant abdominal pain. Imaging shows multiple small hypodense lesions in the liver and spleen. What is the most likely diagnosis?
View More Questions