Corticosteroids for Autoimmune/Inflammatory Disorders in Children: Clinical Aspects


DCI

Anti-inflammatory activity

Mineralocorticoid effect

Dose equivalence (mg)

Half-life (h)

Hydrocortisone

1

1

20

8–12

Cortisone

1

0.8

25

8–12

Prednisone

4

0.8

5

12–36

Prednisolone

4

0.8

5

12–36

Methylprednisolone

5

0.5

4

12–36

Betamethasone

25–30

0

0.75

36–54

Triamcinolone

5

0

4
 




Conditions of Prescription and Main Indications (Table 2)





Table 2
Main indications of corticotherapy for child inflammatory diseases


























































Disease type

Etiology

Remarks

Rheumatology, systemic disease

Juvenile idiopathic arthritis (JIA)

Systemiconset JIA, macrophage activation syndrome, uveitis, polyarticular JIA

Acute rheumatic fever
 

Juvenile dermatomyositis
 

Systemic lupus erythematosus

Pulse therapy: cardiac, nephrologic, neurological form

Scleroderma
 

Sarcoidosis
 

Systemic vasculitis, refractory Kawasaki disease, complications of Henoch–Schönlein purpura
 

Autoinflammatory

PFAPA syndrome

Treatment of crisis

Digestive

Inflammatory bowel disease, autoimmune hepatitis
 

Hematological

Thrombocytopenic purpura, autoimmune hemolytic anemia
 

Nephrologic

Nephrotic syndrome, glomerulonephritis
 

Neurological

Systemic sclerosis, inflammatory demyelinating polyradiculoneuropathy
 

Ophthalmological

Uveitis
 


PFAPA periodic fever, aphthous stomatitis, pharyngitis, adenitis

The prescription of GCs over a long period is an important decision in terms of consequences, considering the risks of a diagnostic error (i.e., prescription in case of infections or neoplasia) and inappropriate or delayed initiation that could impact vital or functional prognosis. The effect is suspensive, which exposes patients to quick relapses when interruptions are attempted. The side effects of GCs represent a major worry, which justifies their use only after a definitive diagnosis has been made. According to the indication, prescription can be short or long term, preferably orally administered prednisone or prednisolone and sometimes with intravenous boluses [1]. Short treatments last less than 10 days and typically aim for an anti-inflammatory effect with high doses (2 mg/kg/day). Examples of such treatment are complicated Henoch–Schönlein purpura, Kawasaki syndrome resistant to immunoglobulins, and idiopathic thrombocytopenic purpura. Long-term treatments aim for lower doses (maximum 60–80 mg/day), with a quick taper. The initial dosing, preferably in the morning, is generally maintained 2–4 weeks, and carefully tapered in gradual steps from 1 to 4 weeks, depending on the pathology, the protocol, and the initial response. This decrease will occur faster for dosages higher than 1 mg/kg/day, with the objective of reaching a maintenance dose of 0.5 mg/kg/day and then the minimal effective dose or the discontinuation. Alternate-day dosing as used for nephrotic syndrome may attenuate the effects on growth but in other indications represents a risk of disease flare. GCs should never be interrupted abruptly, since the risk of adrenal insufficiency must be considered when the administered dose is greater than the replacement dose and when treatment exceeds 15 days. High-dosage “pulse” therapy allows one to obtain a quick anti-inflammatory effect, possibly with a corticosteroid-sparing effect. Intravenous boluses are usually recommended daily over 3 consecutive days, particularly in critical situations regarding vital or functional prognosis (severe organ involvement, hemophagocytosis, resistant Kawasaki disease). Methylprednisolone is given via an intravenous route at a dose of 30 mg/kg (max. 1 g) over a period of at least 4 h and while carefully monitoring vital signs. Intra-articular injections can be part of the therapeutic strategy for juvenile arthritis. Triamcinolone hexacetonide is the most used form because of its long-lasting effect [2].

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Nov 17, 2016 | Posted by in PEDIATRICS | Comments Off on Corticosteroids for Autoimmune/Inflammatory Disorders in Children: Clinical Aspects

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