[CIS PIDD] [cis-pidd] Hyper IgA

[CIS-PIDD]

I had two sisters several years ago with a periodic fever picture who had very high levels of IgA.  They eventually were diagnosed with hyper IgD syndrome.


Peter B. Dent MD, FRCPC
Professor, Pediatrics, McMaster University
Division of Rheumatology & Immunology
McMaster Childrens' Hospital
1280 Main St W. L8S 4K1
Phone:  905-521-2100 ext 75382
FAX: 905-521-4968
E-mail: dentpb at mcmaster.ca



From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: November-23-16 10:08 AM
To: CIS-PIDD
Subject: [cis-pidd] Hyper IgA

I have followed a now 25 yo female with antibody deficiency and recurrent sinusitis with rare chest infections for 21 years. She is currently receiving IGSC 2.5g 6/7 days a week. She is followed by rheumatology for a non-deforming seronegative spondyloarthropathy and been getting Remicade for the past 18 months without much benefit. She is seldom infected and denies GI symptoms and diarrhea.

Her IgA has been steadily increasing:
Nov 17, 2016 - 925mg/dL
Nov 11, 2016 - 803
2015 - 690
2005 - 320
1995 - 125

Additional studies:

Protein, Total, Serum 7.8 g/dL                    6.0-8.5

  Albumin              3.5 g/dL                    2.9-4.4

  Alpha-1-Globulin   0.3 g/dL                    0.0-0.4

  Alpha-2-Globulin          0.7 g/dL                    0.4-1.0

  Beta Globulin        [H]  1.6 g/dL                    0.7-1.3

  Gamma Globulin            1.7 g/dL                    0.4-1.8

  M-Spike                   Not Observed g/dL           Not Observed

  Globulin, Total      [H]  4.3 g/dL                    2.2-3.9

  A/G Ratio                 0.8                         0.7-1.7

 Immunofixation Result, Serum

    An apparent normal immunofixation pattern.

 Immunoglobulin G, Qn, Serum    1493 mg/dL                  700-1600

 Immunoglobulin A, Qn, Serum  [H]  925 mg/dL                   87-352

    Results confirmed on dilution

 Immunoglobulin M, Qn, Serum         181 mg/dL                   26-217

 LDH                       159 IU/L                    119-226

 Sedimentation Rate-Westergren   [H]  51 mm/hr                    0-32

 C-Reactive Protein, Quant           [H]  8.2 mg/L                    0.0-4.9

I would appreciate any thoughts and recommendations for additional studies.

Thanks,

Richard Wasserman
--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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