11 Apr 2021

12/17/20: IgG4 Pachymeningitis

PGY3 Amanda Herrmann presented a case of a 74 year old female with history of alopecia, PUD, chronic recurrent mastoiditis s/p L mastoidectomy/debridement approximately 4 months prior, and chronic non-traumatic SDH who presents to the ED with progressive headache, vertigo, nausea and vomiting.  The patient is pleasant, however somewhat of a vague historian.  She reports bilateral/holocranial headaches, worsening over the last 2-3 months.  She reports associated “room spinning” dizziness, nausea and non-bloody, non-bilious vomiting.  No home medications except Tylenol as needed.  She denies any tobacco, drugs or alcohol use.

Vitals: T 36.8, HR 78, RR 18, BP 150/109, O2 sat 98% on RA

Physical Exam significant for alert and awake female, no significant HEENT findings, R external auditory canal is clear, TM with T-tube in place, no effusions; L external auditory canal is clear, TM with large central perforation, dry; R conductive hearing loss, L normal vs sensorineural hearing loss, normal cardiac/lung exam and neuro exam normal

CBC: WBC 6.6> Hb 11.1/Hct 33.4 <Plt 404 (MCV 83.5, RDW 13.8%)

CMP:

Na 139/K 4.5/Cl 102/HCO3 25/BUN 26/Cr 0.62<Glucose 100

ALP 97>T.protein 7.6/Albumin 4.2/AST 17/ALT 15/Tbili 0.2/Dbili <0.2

Ca 9.5, Mg 2.4, Phos 4.5

Coags: PT 14.2, INR 1.11, PTT 34.9

Infectious workup initiated, with the following findings:

  • UA, COVID-19 negative
  • Lumbar puncture performed:
    • RBC 2
    • Nucleated cell 7 (Lymp 90%, Monocytes 10%)
    • Glucose 55 (normal: 50-80)
    • Protein 252 (normal 15-45)
    • Infectious studies including bacterial, viral and fungal and culture all negative
    • Cytology: no malignant cells identified

Autoimmune workup initiated with the following findings:

  • ESR and CRP elevated at 88 and 15.7 respectively
  • Vitamin D 48 (normal 30-100)
  • ANA: 1:40
  • pANCA: 1:40, MPO 18.2 (detected)
  • C3 and C4 are wnl
  • SSA >8, SSB <1
  • RF <10
  • SM Ab <1
  • SM/RNP Ab <1
  • Serum Ig: normal IgA, IgE; IgG elevated at 1770 (normal 700-1600)
  • Serum IgG4: 130.8 (normal: 4-86)
  • Lumbar puncture:
    • ACE <5
    • IgG 80.1 (normal 0.8 – 7.7)
    • IgG index 2.3 (normal <0.66)
    • IgG synthesis rate +318.2mg/24h (normal -9.9 – +3.3)
    • Oligoclonal bands present

CT head done which showed thickening of the falx and L tentorium has increased since the prior CT and interval increase in the mass effect and increased L to R midline shift.

MRI brain done showed interval progression of diffuse pachymeningeal thickening and enhancement along the L cerebral and L cerebellar convexities.  Interval mass effect including new partial effacement of the L lateral ventricle and 5mm leftward midline shift.

Treatment: After infection was ruled out, patient was started on IV solumedrol x3 days for treatment of IgG4 pachymeningitis and she reported complete resolution of the headache and neck pain.  She was discharged on prednisone taper with plan for Rheumatology and neurology follow up.

Teaching points:

  1. IgG4- related disease = relatively newly recognized group of fibro-inflammatory conditions characterized by a tendency to form tumefactive lesions at multiple sites, histopathological hallmarks (lymphoplasmacytic infiltrate rich in IgG4 positive plasma cells, storiform fibrosis, obliterative phlebitis), elevated serum IgG concentration and favorable objective response to immunosuppression (corticosteroids)
  2. Treatment: glucocorticoids

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