Table 5.
Rheumatological and Immunological Evaluation in 47 Consecutive Patients Evaluated in Our Stanford Pediatric Acute-Onset Neuropsychiatric Syndromes (PANS) Clinic Who Met Symptom Criteria for PANS, but Only the Acute-Onset Group Met Full Criteria for PANS
| Medical evaluations | Fraction (%) of patients with medical finding (n=47) | Acute-onset (PANS group) (n=19) | Subacute/insidious-onset (not PANS group) (n=28) | Significance between PANS and not PANS groups* |
|---|---|---|---|---|
| Reported neck and/or, back and/or sacroiliac joint pain. | 19/47 (40%) | 5/19 (26%) | 14/28 (50%) | 0.56 |
| Patients meeting criteria for inflammatory back pain | 10/47 (21%) | 4/19 (21%) | 6/28 (21%) | 0.45 |
| Patients having at least one episode consistent with reactive arthritis or persistent arthritis (enthesitis-related arthritis, spondyloarthropathy- related arthritis, or psoriatic arthritis). | 13/47 (28%) | 6/19 (32%) | 7/28 (25%) | 0.61 |
| Transient erythematous flat rashes | 6/47 (13%) | 0/19 (0%) | 6/28 (21%) | 0.03 |
| Elevated inflammatory markers ESR/CRP | 3/43 (7%) | 0/17 (0%) | 3/26 (12%) | 0.15 |
| Rheumatology/autoimmune markers | ||||
| ANA (>1:160 titer) | 7/43 (16%) | 5/18 (28%) | 2/25 (8%) | 0.19 |
| Antihistone antibodies (>1.0 Eliza) | 6/41 (15%) | 4/16 (25%) | 2/25 (8%) | 0.27 |
| Antithyroid antibodiesa | 3/41 (7%) | 2/16 (13%) | 1/25 (4%) | 0.11 |
| Tissue-transglutaminase antibodies | 4/37 (11%) | 1/14 (7%) | 3/23 (13%) | 0.38 |
| More than one disease- associated autoantibody | 7/44 (16%) | 4/18 (22%) | 3/26 (12%) | 0.40 |
| Paraneoplastic/autoimmune encephalitis panel (Mayo Medical Laboratory) positive | 1/16 (6%) | 0/5 (0%) | 1/3 (33%) | 0.16 |
| Hematological abnormalities on at least one laboratory examination (presentation or flare) | ||||
| Monocytosis | 20/47 (43%) | 9/19 (47%) | 11/28 (39%) | 0.58 |
| Eosinophilia | 14/47 (30%) | 8/19 (42%) | 6/28 (21%) | 0.13 |
| Neutropenia | 3/47 (6%) | 2/19 (11%) | 1/28 (4%) | 0.34 |
| Anemia | 7/47 (15%) | 3/19 (16%) | 4/28 (14%) | 0.89 |
| Immunoglobulin (Ig) analyses | ||||
| Low IgG levels | 6/21 (29%) | 1/7 (14%) | 5/14 (36%) | 0.41 |
| Low IgA levels | 5/21 (24%) | 1/7 (14%) | 4/14 (29%) | 0.31 |
| Low IgM levels | 6/21 (29%) | 1/7 (14%) | 5/14 (36%) | 0.21 |
| Any hypogammaglobulinemia above | 11/23 (48%) | 2/9 (22%) | 9/14 (64%) | 0.06 |
| Elevated IgE | 2/14 (14%) | 0/5 (0%) | 2/9 (22%) | 0.08 |
| Low vitamin D | 4/42 (10%) | 2/16 (13%) | 2/26 (8%) | 0.66 |
a
Thyroperoxidase antibodies and/or thyroglobulin antibodies.
*
Difference in proportions evaluated with Pearson χ2 test.
ESR, erythrocyte sedimentation rate; CRP, C-reactive protein; ANA, antinuclear antibodies.