Table 3 Main characteristics of the studies on childhood-onset microscopic polyangiitis and features of the patients at presentation
First author | Iudici [11] | Sacri [12] | Bakkaloglu [25] | Peco-Antic [27] | Basu [30] | Sun [29] | Hattori [26] | Yu [28] |
|---|---|---|---|---|---|---|---|---|
Speciality | Adult Internal Medicine | Paediatric Nephrology and Rheumatology | Paediatric Nephrology and Rheumatology | Paediatric Nephrolology | Paediatric Nephrolology | Paediatric Nephrology | Paediatric Nephrology | Paediatric Nephrology |
Year of publication | 2015 | 2015 | 2001 | 2006 | 2015 | 2014 | 2001 | 2006 |
Years of enrolment | 1965–2014 | 1986–2011 | 1990–1999 | 1998–2003 | 2011–2014 | 2003–2013 | 1990–1997 | 1998–2004 |
Study design | Retrospective | Retrospective | Retrospective | Retrospective | Retrospective | Retrospective | Retrospective | Retrospective |
Number of patients | 4 | 38 | 10 | 7 | 11 | 20 | 21 | 19 |
Ethnicity | 3 Caucasian, 1 Asian | 26 (68 %) Caucasian | NA | NA | NA | NA | NA | NA |
Female, number (%) | 0 | 34 (89) | 6 (60) | 6 (86) | 6 (54) | 16 (80) | NA | 18 (95) |
Median age at onset | NA | NA | NA | 12 (7–15) | 7.6 (4.3–11.8) | 10 (1.9–16.8) | NA | 10.8 ± 2.8 |
Median age at diagnosis | 13 (3–16) | 11.2 (8.9–12.3) | 12 (8–17) | NA | NA | NA | NA | NA |
Classification/diagnostic tool used | Revised Chapel-Hill | According to Watts et al. [40] | Typical biopsy findings for renal or non-renal tissues | Inclusion criteria: 1) symptoms suggestive of MPA; 2) Biopsy-proven GN; 3) MPO-ANCA+ | Revised Chapel-Hill CC | Revised Chapel-Hill CC | Chapel-Hill | Chapel-Hill |
cANCA positivity (ELISA) | 1/1 | 4/38 | 0 | NA | 0 | 2/17 | NA | 0 |
pANCA positivity (ELISA) | 0/1 | 33/38 | 10 (100) | 7 (100) | 11 | 16/17 | NA | 19/19 |
cANCA positivity (IFI) | 1/2 | 0 | 0 | NA | 0 | 2/20 | NA | 0 |
pANCA positivity (IFI) | 1/2 | NA | 9 (90) | NA | 10/10 | 18/20 | NA | 19/19 |
Clinical manifestations | ||||||||
Systemic | 4 (100.0) | 29 (78.0) | 10 (100.0) | 7 (100.0) | 8 (72.7) | 10 (50.0) | 18 (85.7) | 12 (63.1) |
Mucocutaneous | 2 (50.0) | 13 (34.2) | 7 (70.0) | 7 (100.0) | 3 (27.2) | 3 (15.0) | 8 (38.0) | 6 (31.5) |
Musculoskeletal | 2 (50.0) | 11 (28.9) | 6/9 (66.7) | 7 (100.0) | 11 (100.0) | 2 (10.0) | 7 (33.0) | 0 |
Ocular | 0 | 3 (7.8) | 0 | 0 | 0 | 1 (5.0) | 2 (9.5) | 1 (5.2) |
Ear, nose, and throat | 0 | 0 | 0 | 0 | 0 | 0 | 2 (9.5) | 2 (10.5) |
Respiratory | 0 | 11 (28.9) | 3 (30.0) | 4 (57.1) | 5 (45.4) | 3 (15.0) | 13 (61.9) | 10 (52.6) |
Cardiovascular | 1 (25.0) | 0 | 0 | 0 | 1 (9.0) | 0 | 0 | 0 |
Gastrointestinal | 2 (50.0) | 4 (10.5) | 2/9 (22.2) | 4 (57.1) | 2 (18.1) | 3 (15.0) | 7 (33.3) | 9 (47.3) |
Neurological | 2 (50.0) | 2 (5.2) | 2/9 (22.2) | 6 (85.7) | 1 (9.0) | 3 (15.0) | 1 (4.7) | 1 (5.2) |
Renal | 4 (100.0) | 36 (94.7) | 7 (70.0) | 7 (100) | 11 (100.0) | 16 (100.0) | 21 (100.0) | 19 (100.0) |