小兒科topic three: SLE and children

SLE and children

The following notes are simplified for quick review. If there are mistakes, please comment and point out!

1. Initial presentation: 

-- adults: rash

-- children: arthritis, nephropathy, ITP, diarrhea, seizure, psychosis 

2. Diagnosis

-- by clinical presentation 

-- by lab data

***clinical presentation***

--Mucocutaneous: skin, mouth, nasopharynx

--Musculoskeletal: joint

--Heart/lung: serositis

--Kidney: proteinuria>500 mg/dl or 3+

--Neuro: seizure, psychosis

--GI

--Hema: hemolytic anemia, WBC<4000, lymphocyte<1500, plt<100000

--AIR

***lab data***

--ANA: for diagnosis, not for long-term F/U

--ds-DNA

For high risk pts:

--anti-Sm
--anti-SSA. anti-SSB, ribonucleoprotein(U1-RNP)

--antiphospolipid: anti-cardiolipin, lupus anticoagulant,  beta-2 glycoprotein antibody 

May be (+) for ANA(-):

--anti-Ro

--anti-La

Others:

--anti-histone

--mixed Coomb's test: coagulation↓--> antibody(+); coagulation normal --> coagulation factors deficiency

3. Pregnancy and SLE

-- The mother:

                        higher spontaneous abortion possibility, 

                        check antiphospolipid, 

                        cannot use Cellcept (MMF)

--The child:

                        check anti-SSA/SSB for SLE risk if the mother has SLE

                        may lead to permenant heart block

4. Biopsy's role: for prognosis, before taking aggressive treatments( If the damage has become irreversible, there's no much need for aggressive treatment. )

5. Lupus nephritis

6. Drugs for SLE

--HCQ(hydroxychloroquine): ex. Plaquenil, for flare up, arthritis

--Corticosteroids: low dose for arthritis, high dose for major diseases

-- MTX: for rash, arthritis

--Belimumab: for rash , arthritis

--CYC(cyclophosphamide): for severe nephritis(type 4),  CNS 

--MMF(Mycophenolate): for nephritis

--CsA(cyclosporine): for renal disease

--Rituximab: for refractory SLE, ITP, AIHA

--ACEI, NSAID

7. Prognosis :

--Worse if course beginning at youth

--Renal failure, infections are major causes of morbidity and mortality