Table 2.
Clinical features of GPA at presentation in the largest pediatric cohorts reported.
| Cabral et al. (ARChiVe) (6) | Bohm et al. (31) | Sacri et al. (3) | James et al. (33) | Calatroni et al. (37) | Akikusa et al. (36) | |
|---|---|---|---|---|---|---|
| Type of study | Retrospective and Prospective | Retrospective | Retrospective | Retrospective | – | Retrospective |
| No of patients | 183 | 56 | 28 | 28 | 31 | 25 |
| M/F | 70/113 | 18/38 | 7/21 | 68% | 11/22 | 5/20 |
| Median age at diagnosis | 12 | – | 12.8 | 14.7 | 14 | 14.5 |
| Ethnicity | Predominant Caucasians (59%), unknown (44%) | Caucasians | Caucasians | Caucasians (75%) and Asian (21%) | Caucasian | Caucasian |
| Classification used | MD diagnosis | EULAR/ PRINTO/ PRES |
EULAR/ PRINTO/ PRES |
EULAR/ PRINTO/ PRES or ACR1990 |
Unclear | ACR Criteria |
| CLINICAL FEATURES (%) | ||||||
| Constitutional symptoms (fever, malaise and weight loss) | 88 | 89 | 82 | 79 | 68 | 96 |
| Upper respiratory involvement (ear, nose, throat) | 70 | 91 | 75 | 93 | 78 | 84 |
| Lower respiratory tract involvement (hemoptysis, nodules, pulmonary hemorrhage and pulmonary infiltrates) | 74 | 78.5 | 68 | 82 | 52 | 80 |
| Renal (elevated creatinine, biopsy proven GN, abnormal urinalysis) | 83 | 68 | 78.5 | 71 | 65 | 88 |
| Ocular (scleritis, conjunctivitis) | 43 | 34 | 21.4 | 21 | 45 | 52 |
| Gastrointestinal | 36 | 16 | 17.8 | 29 | 6 | 12 |
| Musculoskeletal (arthralgia, myalgia, arthritis) | 14 | 59 | 57.1 | 61 | 45 | 96 |
| Mucocutaneous | 47 | 64 | 53.5 | 33 | 26 | 32 |
| Nervous system (headache) | 20 | 14 | 3.5 | 4 | 6 | 8 |
| Cardiovascular (venous thrombosis) | 5 | 0 | 0 | 11 | 6 | 20 |