| Literature DB >> 35186817 |
Zhijuan Kang1,2, Wentao Wu2, Mai Xun1,2, Yunfeng Ding1,2, Zhihui Li1,2.
Abstract
Henoch-Schönlein purpura (HSP)/ IgA vasculitis (IgAV) is the most common form of systemic vasculitis in children and often involves the skin, gastrointestinal tract, joints, and kidneys, though cardiac involvement rarely occurs. We report on a 6-year-old male child with HSP/IgAV who had renal and cardiac involvement at the initial stage of the disease and in whom we found an extremely rare coronary artery aneurysm. After administration of glucocorticoid combined with mycophenolate mofetil, the renal involvement improved, but the coronary artery aneurysm remained. Pursuant to this case, we retrieved information on other cases of HSP/IgAV complicated with cardiac involvement from the PubMed database, and excluded cases of cardiac involvement accompanied by Kawasaki disease, polyarteritis nodosa, rheumatic fever, Takayasu arteritis, systemic lupus erythematosus, poststreptococcal glomerulonephritis, or sepsis. We then analyzed gender, age, cardiac involvement, renal involvement, treatment, and prognoses. To date, 24 cases of HSP/IgAV complicated with cardiac involvement have been reported. Among them, there were 22 male and 2 female patients, with the onset age ranging from 3 to 71 years old. A total of 10 children (including the child we examined) and 14 adults were identified, and 17 patients (70.8%) had HSP/IgAV complicated with renal involvement. The majority of patients were treated with glucocorticoid and/or immunosuppressants or biological agents, 4 patients died (16.7%), 8 patients were completely relieved (33.3%), and 3 patients had unknown prognoses. This article suggests that HSP/IgAV complicated with cardiac involvement may result in a poor prognosis and early treatment may therefore be essential. Our case revealed that glucocorticoid does not prevent the occurrence of renal and cardiac involvement in HSP/IgAV patients. If HSP/IgAV is complicated with coronary artery dilation, the therapeutic effect of glucocorticoid combined with immunosuppressants is not satisfactory, and early administration of biological agents or IVIG may be an effective therapeutic regimen.Entities:
Keywords: Henoch-Schönlein purpura; IgA vasculitis; cardiac involvement; children; coronary artery dilation; renal involvement
Year: 2022 PMID: 35186817 PMCID: PMC8850625 DOI: 10.3389/fped.2021.781106
Source DB: PubMed Journal: Front Pediatr ISSN: 2296-2360 Impact factor: 3.418
Laboratory examination results of the HSP/IgAV child after hospitalization.
| Blood routine test | Pathogenic examination | ||
| White blood cell (×109/L) | 24.77 | PPD-IgG/IgM | Negative |
| Neutrophils ratio (%) | 0.680 | SPOT-TB test | Negative |
| Lymphocyte ratio (%) | 0.253 | Mp-Ab | Negative |
| Hemoglobin (g/L) | 143 | HIV-Ab | Negative |
| Platelet (×109/L) | 640 | CMV-DNA(0–400 Copies/mL) | <400 |
| Red blood cell (×1012/L) | 5.09 | EBV-DNA(0–400 Copies/mL) | <400 |
| Blood biochemical test | ASO(0–100 IU/mL) | <25 | |
| ALB (35–55 g/L) | 38.8 | Autoimmune antibody | |
| AST (0–40 IU/L) | 119.1 | Anti-nuclear antibodies (ANAs) | Negative |
| ALT (0–40 IU/L) | 45.8 | P-ANCA | Negative |
| Creatinine(μmol/L) | 33.0 | C-ANCA | Negative |
| Urea nitrogen(mmol/L) | 4.32 | ACA IgA/ IgG/ IgG | Negative |
| CK (38–174 U/L) | 976 | GBM/MPO/PR3-IgG | Negative |
| CK-MB (0–24 IU/L) | 71.6 | Coagulation function tests | |
| LDH (0–450 IU/L) | 965 | PT (10–14s) | 12.9 |
| Troponin I (<0.01 ug/mL) | 9.77 | INR (0.8–1.5) | 0.99 |
| Acute-phase reactants | APTT (28–48s) | 30.4 | |
| C-reactive protein (0–8 mg/L) | <0.5 | AT3 (80–120%) | 126 |
| ESR (0–15 mm/h) | 29 | TT (14–20s) | 16.6 |
| Immunoglobulins | FIB (170–450 mg/dL) | 226 | |
| Ig A (0.14–1.38 g/L) | 2.01 | D-Dimer (0–0.55ug/mL) | 0.38 |
| Ig E (<90 IU/mL) | 2,780 | FDP (0–5 ug/mL) | 2.38 |
| Ig G (3.6–10.6 g/L) | 7.69 | Urine analysis | |
| Immunoglobulin M (0.38–1.44 g/L) | 1.50 | Proteinuria (Negative) | 3+ |
| Complements | Urine red blood cell count (0–22.8/ul) | 143.5 | |
| C3(0.79–1.52 g/L) | 1.02 | Proteinuria-24 h (0–150 mg/24h) | 1,210 |
| C4(0.16–0.38 g/L) | 0.16 | urine protein/creatinine ratio (mg/mg) | 3.35 |
Mp-Ab, Mycoplasma Pneumoniae Antibodies; TB, tubercle bacillus; ANCA, anti-neutrophilic cytoplasmic antibodies; PR3-IgG, proteinase 3-IgG; MPO-IgG, myeloperoxidase-IgG; GBM-IgG, glomerular basal membrane-IgG; ACA, anti-cardiolipin antibody.
Figure 1Dilation of the left coronary artery showed by cardiac color Doppler ultrasound.
Figure 2Renal biopsy shows: (A). The number of cells in the glomerulus increased, focal and segmental hyperplasia of mesangial cells and stroma, and multiple crescents (HE, X100); (B). Cellular crescents (PAS, X400); (C). IgA deposited in glomerular mesangial area (IF, X400).
Follow-up of proteinuria, troponin I, and cardiac color Doppler ultrasound of the HSP/IgAV child after discharge.
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| 24-h urinary protein quantification (mg) | 1,210 | 260 | 230 | 190 | 90 | 70 | |
| Troponin I (μg/mL) | 9.77 | <0.01 | - | - | - | - | - |
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| Backbone (Z-value: score) | 3.6 mm (+3.2) | 3.7 mm (+3.32) | 3.6 mm (+2.98) | 3.9 mm (+3.82) | 3.5 mm (+2.62) | 3.3 mm (+1.9) | 3.7 mm (+3.0) |
| Anterior descending branch (Z-value: score) | - | 6.8 mm (+16.13) | 11 mm (+30.68) | 6.7 mm (+15.67) | 11 mm (+30.63) | 9.6 mm (+25.7) | 10.6 mm (+29.03) |
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| Backbone (Z-value: score) | 3.0 mm (+2.6) | 3.3 mm (+3.36) | 2.8 mm | 4.1 mm (+5.7) | 2.6 mm (+1.20) | 2.6 mm (+1.20) | 2.7 mm (+1.37) |
| Distal wider inner diameter (Z-value: score) | - | 4.3 mm (+6.37) | 4.2 mm (+6.051) | - | - | - | - |
Figure 3Flowchart of the literature search.
Comparison of reported pediatric and adult HSP/IgAV patients with cardiac involvement.
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| Age of onset (y) | 9.5 (7.5–13.0) | 60.0 (21.0–63.0) |
| Male ( | 8 (80) | 14 (100) |
| Renal involvement ( | 6 (60) | 11 (78.5) |
| Death ( | 1 (10) | 3 (21.4) |
| Complete remission ( | 3 (30) | 5 (35.7) |
| Treatment record ( | 9 | 12 |
| glucocorticoid use ( | 7 (77.8) | 10 (83.3) |
| Immunosuppressant use ( | 3(33.3) | 3 (25) |
| Use of biological agents ( | 1 (11.1) | 1 (8.3) |
Pediatric patients with HSP/IgAV accompanied by cardiac involvement in the literature.
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| Our patient, M/6 | Chest tightness, crushing pain in precordial area | Coronary artery aneurysm (Echocardiogram) | CK/CK-MB?troponin I elevation | Kidney, Liver | MMF, MP/ Prednisone, Aspirin, Warfarin | Coronary arteries aneurysm (22 months) | Proteinuria disappeared; liver function normal (22 months) |
| M/9 Bloom et al. ( | No | The left main and left anterior descending coronary artery dilation (Echocardiogram) | No | No | Aspirin, IVIG, Infliximab | Complete resolution (3 months) |
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| M/3 Veetil et al. ( | Persistent tachycardia | Periluminal coronary artery thickening (Echocardiogram) | No | No | Prednisone, IVIG | Complete resolution (1 month) |
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| M/17 Zaidi et al. ( | Shortness of breath and chest pain | Left ventricle dilatation and prominent coronary arteries (Echocardiogram) | No | Kidney, Liver, Pancreas | MP/ Prednisone, ACEI | Unknow | Renal function normal, proteinuria decreased; liver and pancreas unkown (3 months) |
| F/8 Yilmaz et al. ( | Tachycardia | Pericardial effusion, right atrium thrombus, myocarditis (Echocardiogram and MRI) | BNP elevation | Kidney | Hemodialysis, MP/Prednisolone, CTX, AZA, ACEI | Complete resolution (8 months) | Kidney function normal, proteinuria decreased (8 months) |
| M/8 Cimaz et al. ( | Cardiac murmur | Pericardial effusion; inversion T waves with repolarization abnormalities (Echocardiogram and ECG) | No | No | Symptomatic treatment | Complete resolution (a few weeks) |
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| F/10 James et al. ( | Apical III/VI holosystolic murmur | Severe mitral regurgitation, left atrium dilatation, diastolic dysfunction, mitral valve prolapses (Echocardiogram) | BNP elevation | Pulmonary hemorrhage | Mechanical ventilation, IVIG, Prednisone, ACEI | Mitral regurgitation (12 months) | Asymptomatic (12 months) |
| M/16 Shah and Hata ( | Palpitations | Arrhythmia (ECG) | The troponins elevation | Kidney, CNS, GI bleed | MP/ Prednisone, Labetalol, Amiodarone, Metoprolol, CTX, MMF, ACEI | Complete resolution (9 months) | No recurrence of GI and neurological complications; kidney function normal, proteinuria decreased (9 months) |
| M/12 Migita et al. ( | Carotid artery engorgement, hypotension, and narrow pulse pressure | Pericardial tamponade (Echocardiogram) | No | Kidney, small intestine, nervous system | Prednisone/MP, Surgery, Pericardiocentesis | Unknow | Unknow |
| M/11 Lecutier ( | Systolic apical murmur | No | Myocardium necrosis with calcification (Necropsy) | Kidney | Unknow | Death (1 month) | Death (1 month) |
M, male; F, female; Electrocardiogram, ECG; MRI, Magnetic Resonance Imaging; BNP, Brain natriuretic peptide; MMF, Mycophenolate mofetil; CTX, Cyclophosphamide; MP, Methylprednisolone; AZA, azathioprine; CNS, Central Nervous System; ACEI, Angiotensin Converting Enzyme Inhibitor; GI, Gastrointestinal.