Table 1.
Cohort characteristics at the time of diagnosis
| N = 102 | |
|---|---|
| Age at diagnosis, y | |
| Mean (SD) | 35.9 (16.4) |
| <18, n (%) | 19 (18.6) |
| Deceased, n (%) | 8 (7.8) |
| Sex∗, n (%) | |
| Female | 44 (43.1) |
| Male | 58 (56.9) |
| Race∗, n (%) | |
| American Indian/Alaska Native | 1 (1.0) |
| Asian | 14 (13.7) |
| Black/African American | 12 (11.8) |
| Native Hawaiian/Pacific Islander | 1 (1.0) |
| White | 66 (64.7) |
| Other/not stated | 8 (7.8) |
| Histopathological subtype, n (%) | |
| Hyaline vascular | 1 (1.0) |
| Hypervascular | 62 (63.9) |
| Mixed | 27 (27.8) |
| Plasmacytic | 7 (7.2) |
| Unknown | 5 |
| Time from diagnostic biopsy to pathologic diagnosis, d | |
| Median (interquartile range) | 4 (2-8) |
| Clinical subtype, n (%) | |
| TAFRO | 61 (59.8) |
| NOS | 41 (40.2) |
| Clinical symptoms, n (% of those assessed) | |
| Constitutional symptoms | 92 (91.1) |
| Organomegaly | 73 (79.3) |
| Cherry hemangioma/violaceous papules | 2 (2.6) |
| Lymphocytic interstitial pneumonitis | 0 |
| Fluid retention | 79 (84.0) |
| Laboratory features | |
| CRP, mg/L | 80.0 (22.0-180.0) |
| ESR, mm/h | 73.0 (43.0-107.0) |
| Platelets, 103/μL | 134.0 (64.0-275.8) |
| Hemoglobin, g/dL | 10.0 (8.4-11.6) |
| Albumin, g/dL | 2.7 (2.3-3.3) |
| Creatinine, mg/dL | 1.1 (0.9-1.7) |
| eGFR, mL/min per 1.73m2, n (%) | |
| 0-20 | 6 (9.5) |
| 20-40 | 9 (14.3) |
| 40-60 | 12 (19.0) |
| ≥60 | 36 (57.1) |
| Not documented | 39 |
| IgG, mg/dL | 1150 (780-1727) |
| Gammaglobulin, g/dL | 1.22 (0.9-1.8) |
Data represent closest information to the date of diagnosis within 90 days before, through 15 days after, diagnosis date; laboratory data presented in median (interquartile range) unless otherwise stated.
eGFR, estimated glomerular filtration rate; ESR, erythrocyte sedimentation rate; IgG, immunoglobulin G; SD, standard deviation.
∗
Patient reported.