Cerebrospinal fluid (CSF), serological and microbiological findings in the 2 patients with IgG4 hypertrophic pachymeningitis.
A | Patient 1 | Patient 2 | |||
---|---|---|---|---|---|
Reference Values | Before Therapy | After Therapy | Before Therapy | After Therapy | |
Appearance | Colorless, clear | Colorless, clear | Colorless, clear | Colorless, clear | Colorless, clear |
Total proteins (mg/dl) | 12–60 | 76 | 76 | 44 | 73 |
Glucose (mg/dl) | 40–80 | 42 | 55 | 59 | 56 |
Cells (μl) | 0–1 | 32 (lymphocytes) | 20 (lymphocytes) | 2 (lymphocytes) | 1 (lymphocytes) |
Cytological analysis | No atypical cells | No atypical cells | No atypical cells | No atypical cells | |
Microbiological analyses* | Negative | Negative | Negative | Negative | Negative |
Autoimmunity and ACE§ | Negative | Negative | Negative | ||
CSF albumin (mg/dl) | 10–30 | 85 | 45 | 25 | 37 |
Serum albumin (mg/dl) | 3500–5000 | 2370 | 2650 | 3350 | 4180 |
Albumin Quotient^ | < 0.7 | 3.6 | 1.7 | 0.7 | 0.9 |
CSF IgG (mg/dl) | 0.8–3.8 | 17.4 | 5.8 | 8.7 | 3.5 |
Serum IgG (mg/dl) | 840–1600 | 607 | 797 | 651 | 736 |
IgG Index | < 0.70 | 0.84 | 0.43 | 1.8 | 0.53 |
IgGLoc | 0 | 1 | 0 | 5.5 | 0 |
CSF IgG4 (mg/dl) ° | 0.01–0.32 | 5.35 | 0.07 | 4.94 | 0.54 |
Serum IgG (mg/dl) | 3–230 | 46 | 19 | 210 | 75 |
IgG4 Index° | 0.25–0.91 | 3.21 | 0.23 | 3.16 | 0.81 |
IgG4Loc | 0 | 4.1 | 0 | 3.91 | 0.09 |
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↵A. *PCR for Herpes simplex virus types 1 and 2, varicella-zoster virus, cytomegalovirus, Epstein-Barr virus, enterovirus, Mycobacterium tuberculosis; gram, acid-fast stain, and microbiological cultures; search for Aspergillus fumigatus and Cryptococcus neoformans antigens; serology for Lyme disease, brucellosis, syphilis.
↵§ Rheumatoid factor, antinuclear, anticyclic citrullinated peptide, and antineutrophil cytoplasmic antibodies; angiotensin converting enzyme (ACE).
↵^ Albumin quotient measures the blood-CSF barrier permeability.
↵° Normal range for CSF IgG4 concentration and IgG4 Index was calculated using 9 patients affected by pituitary nonsecreting adenomas without blood-CSF barrier damage and OCB on CSF analysis, nor evidence of meningitis on magnetic resonance imaging. IgG4-HP: immunoglobulin G4-related hypertrophic pachymeningitis.
B. Cerebrospinal fluid concentration of IgG subclasses (IgG1, IgG2, IgG3, and IgG4) show a significant decline after immunosuppressive treatment of IgG4 levels in both patients, as well as of IgG1 levels in Patient 1. IgG2 and IgG3 were not significantly affected by immunosuppressive therapy.