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Chronic Pachymeningitis with IgG4 Sclerosing Disease

A case study of a 42-year-old woman with headache and left transverse sinus occlusion. The diagnosis revealed chronic pachymeningitis with meningothelial proliferation and increased IgG4-positive plasma cells, potentially indicating IgG4 sclerosing disease. The differential diagnosis includes lymphoplasmacyte-rich meningioma, idiopathic hypertrophic pachymeningitis, lymphoproliferative disorder, and IgG4-related hypertrophic pachymeningitis.

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Chronic Pachymeningitis with IgG4 Sclerosing Disease

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  1. DIAGNOSTIC SLIDE SESSION CASE 8 Michelle Madden Felicella, MD Juan Bilbao, MD Arie Perry, MD Clinical history, follow-up and MR images kindly provided by Dr. Todd Mainprize

  2. CLINICAL HISTORY • 42-yo woman with headache and left transverse sinus occlusion • Treated with warfarin and followed by neurology • MRI: prominent dural thickening and nodularity of cerebral hemispheres including the falx and tentorium • Repeat MRI a few months later: worsened thickening • Referred to neurosurgery for biopsy of the left temporal lobe

  3. DIAGNOSIS???

  4. EMA EMA

  5. CD20 CD3

  6. Lambda Kappa

  7. IgG4 IgG

  8. SPECIAL STAINS • CD3, CD5: numerous reactive T-cells • CD20, CD21, CD23, CD10, BCL6: small lymphocytes in germinal centers • BCL2: positive in scattered interstitial small lymphocytes • Cyclin D1: negative • EMA: positive in meningothelial cells (limited to surface) and numerous plasma cells • ISH for kappa and lambda: polyclonal • Ki-67: moderate LI • IgG4: 80 plasma cells/HPF • IgG4/IgG: 15%

  9. DIAGNOSIS • Chronic pachymeningitis with meningothelial proliferation and increased IgG4-positive plasma cells • Most likely represent IgG4 sclerosing disease and meningothelial hyperplasia

  10. DIAGNOSTIC POINTS • Differential diagnosis: • Lymphoplasmacyte rich meningioma • Idiopathic hypertrophic pachymeningitis • Lymphoproliferative disorder • IgG4-related hypertrophic pachymeningitis • Key Histologic features: • Dense lymphoplasmacytic infiltrate with many plasma cells • Storiform Fibrosis • Phlebitis • Scattered eosinophils • Average count of 5 HPF in the area of greatest concentration: • Our case: 80 IgG4 + cells/HPF and 518 IgG + cells/HPF • IgG4:IgG ratio of 15%. • Normal ratio in serum is <5% • Average IgG4 PCs in other inflammatory conditions <10 HPF

  11. DIAGNOSTIC POINTS • Although cut-offs well established in other organ systems, they are not well established for meningeal disease • Can form a mass or a more diffuse plaque-like growth pattern. More commonly is mass forming in other organs. • Highly steroid responsive • Serum IgG4 levels may be helpful in some cases and should be done • Hyper-eosinophilia also common

  12. FOLLOW-UP • Rx after bx: high dose steroids for about 2 months • CT scans of neck, chest, abdomen negative for systemic evidence of a lymphoproliferative disorder. • MRI at 2 months showed significant improvement. She was tapered from steroids subsequently due to side effects. Follow-up MRI scans show continued improvement. • She is much improved clinically although she still has transverse sinus occlusion and is on warfarin.

  13. PRE-RX

  14. POST-RX

  15. REFERENCES • Smyrk TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol 2011;23(1):74-9. • Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol 2010;17(5):303-32. • Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol 2010;120(6):765-76. • Stone JH, Zen Y, Deshpande V. IgG4 related disease. N Engl J Med 2012;366:539-51.

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