Immunopathology: IgG4-related disease: practice question

A Pathology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

Try it

Pathology · Immunopathology: IgG4-related disease

Sample item

A 58-year-old man has painless obstructive jaundice, a diffusely enlarged sausage-shaped pancreas and bilateral submandibular gland swelling. Pancreatic biopsy shows dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis, and serum IgG4 is raised. Malignancy has been excluded. What is the most appropriate treatment?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

B. Oral glucocorticoids

IgG4-related disease is a fibro-inflammatory condition affecting the pancreas (type 1 autoimmune pancreatitis), salivary glands, bile ducts and retroperitoneum. Typical histology is lymphoplasmacytic infiltrate with many IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It responds dramatically to glucocorticoids, with rituximab for relapse.

Option by option

  • A. Lifelong ursodeoxycholic acid alone

    Ursodeoxycholic acid is used for primary biliary cholangitis and does not suppress this inflammation.

  • B. Oral glucocorticoids (correct)

    Correct. Steroids are first-line once cancer is excluded.

  • C. Gemcitabine-based chemotherapy

    Chemotherapy is for pancreatic adenocarcinoma, not for an immune-mediated fibrosing disease.

  • D. Pancreaticoduodenectomy (Whipple procedure)

    Surgery is for resectable pancreatic carcinoma, which was excluded here and does not explain the systemic features.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

More Pathology questions

Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is B, Oral glucocorticoids. IgG4-related disease is a fibro-inflammatory condition affecting the pancreas (type 1 autoimmune pancreatitis), salivary glands, bile ducts and retroperitoneum.

Why is "Lifelong ursodeoxycholic acid alone" not the answer?

Ursodeoxycholic acid is used for primary biliary cholangitis and does not suppress this inflammation.

Why is "Gemcitabine-based chemotherapy" not the answer?

Chemotherapy is for pancreatic adenocarcinoma, not for an immune-mediated fibrosing disease.

Why is "Pancreaticoduodenectomy (Whipple procedure)" not the answer?

Surgery is for resectable pancreatic carcinoma, which was excluded here and does not explain the systemic features.

What concept does this question test?

It tests Immunopathology: IgG4-related disease in Pathology. Typical histology is lymphoplasmacytic infiltrate with many IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It responds dramatically to glucocorticoids, with rituximab for relapse.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Immunopathology: IgG4-related disease?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.