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T
                                           OF SYSTEMIC LUPUS ER
                                   MANAGEMENT
                                                     YTHEMA
 YTHEMA
 T
     QUICK REFERENCE FOR HEAL
 MANAGEMENT
 QUICK REFERENCE FOR HEAL
  OF SYSTEMIC LUPUS ER
 QUICK REFERENCE FOR HEALTHCARE PROVIDERSTHCARE PROVIDERS  MANAGEMENT OF SYSTEMIC LUPUS ERYTHEMATOSUSOSUS  QUICK REFERENCE FOR HEALTHCARE PROVIDERSTHCARE PROVIDERS  MANAGEMENT OF SYSTEMIC LUPUS ERYTHEMATOSUSOSUS
                      CLINICAL MANIFESTATIONS
 TREATMENT           PRE-PREGNANCY & PREGNANCY
 KEY MESSAGES
 1.  Systemic lupus erythematosus (SLE) is a chronic autoimmune
 •  Principles of SLE treatment are to achieve:  •  It is important  to ensure  that patients with  SLE who plan  to get
                                         Neuropsychiatry
 multisystem disorder with diverse & complex clinical manifestations
   disease remission  pregnant achieve the following:   • Polyneuropathy   •  Acute confusion state
 characterised  by  inflammation  in  a  variety  of  organs.  It  has  a     remission or low disease activity for ≥6 months
                                   • Cerebrovascular  • Headache/Migraine
   disease flare prevention
 relapsing-remitting course with a very unpredictable prognosis &     disease/Stroke   •  Transverse myelitis
   organ damage prevention    well-controlled blood pressure
 considerable morbidity.           • Seizure   •  Cognitive impairment
   quality of life improvement
          estimated glomerular filtration rate (eGFR) >60 mL/min/1.73 m
                                                        2
 2.  Diagnosis of SLE should be based on clinical manifestations   Mucocutaneous   • Psychosis
   minimisation of drug side-effects  •  Malar rash
          proteinuria <1 g/day (proteinuria 2+)
 supported by laboratory findings following exclusion of alternative
 •  If complete remission cannot be achieved, the lowest possible   •  All pregnant SLE patients:  Eye
       •  Oral ulcers
 diagnoses.  • Photosensitivity              •  Dry eyes
 disease activity in all organs involved should be targeted.
          especially those with positive aPL should be referred to the
 3.  All patients with SLE should have clinical assessments of disease   •  Discoid rash   •  Retinal vasculitis
         rheumatologist at antenatal booking
 activity using validated assessment tools.  • Alopecia  •  Optic neuritis
          should be under combined care of rheumatologist/physician,
 4.  Patients with SLE should practise sun avoidance &, use protective   Cardio-respiratory
 FREQUENCY & PARAMETERS FOR MONITORING
         feto-maternal specialist/obstetrician & family medicine specialist
 clothing & broad-spectrum sunscreen with at least sun protection   • ILD  Haematology
 factor (SPF) 50.  Patients with  Patients with stable/  • Pericarditis   • Leukopenia
 active disease
 5.  Corticosteroids should be used for acute flare in SLE; the dose should   • PAH  • Thrombocytopenia
 low disease activity
 At first
                                                • AIHA
 Assessments  visit  should be reviewed  should be reviewed  • Pleurisy   REFERRAL  • Lymphopenia
 be minimised accordingly & discontinued whenever possible.
    • Serositis
 at least every
 6.  All patients with SLE should be on hydroxychloroquine (HCQ) unless   • Myocarditis  • APS
 every 6 - 12 months
      •  All cases with clinical suspicion of SLE should be promptly referred
 intolerant or contraindicated.  1 - 3 months  • Libman-Sacks
        to
 Clinical
 7.  Immunosuppressants should be considered as add-on therapy to     endocarditis rheumatologists for  confirmation of the  diagnosis  & further
        management.
 History  Renal                                 Gastrointestinal
 patients with SLE not responding to HCQ alone or in combination with
 Vital signs  • Proteinuria                     • Pancreatitis
 corticosteroids, or when corticosteroids doses cannot be tapered.
 Clinical examination
       • Microscopic
 8.  Infection in patients with SLE should be identified early & treated   Indications   • Enteritis
         haematuriafor  referral  to  rheumatologist  includes  to  confirm  diagnosis,
                                                •  Lupoid hepatitis
 Drug review  assess disease activity & severity, provide general disease management,
 accordingly.
 Blood tests
 9.  All  women  with  SLE  in  the  reproductive  age  group  should  receive   manage organ involvement or life-threatening disease & manage/prevent
 FBC  treatment toxicities. Other specific circumstances that require referral
                                             • Raynaud’s
 pre-pregnancy counselling.
 RP
 10. In SLE with pregnancy, HCQ, azathioprine, calcineurin inhibitors & low   include APS, pregnancy & perioperative management.   phenomenon
         Vasculitis
 LFT
 dose corticosteroids should be continued.
 CRP  a  a
                                           Musculoskeletal
 ESR  This Quick Reference provides key messages & summarises the main  For moderate to severe organ involvement, patients with SLE will require
                                           • Arthritis/Arthralgia
     multidisciplinary care involving various subspecialties.
 Bone profile   a  a  a  Constitutional    • Myositis
 recommendations in the Clinical Practice Guidelines (CPG) Management of Systemic
 -
 Vitamin D3  Lupus Erythematosus.  a  symptoms
 a
           • Fever
 Immunology/serology   Indications for urgent referral are as listed below:
           • Fatigue
 ANA Details of the evidence supporting these recommendations can be found in the   •  for patients not diagnosed with SLE yet -
 -
           •  Weight loss
 -
         clinical suspicion of SLE with major or multisystem organ involvement
 Anti-dsDNA  above CPG, available on the following websites:  a  •  for patients diagnosed with SLE -
 a
 C3/C4 levels  Ministry of Health Malaysia: www.moh.gov.my  a    disease flare of major organ or multisystem organ involvement
 a
 aPL   Academy of Medicine Malaysia: www.acadmed.org.my  a,p    pregnancy (at booking)
 a
 ENA   Malaysian Society of Rheumatology: msr.my  a,p    severe infection
 a
 Immunoglobulin A, G, M  a  a  a  AIHA = autoimmune haemolytic anaemia; APS  = antiphospholipid syndrome; ILD =
 CLINICAL PRACTICE GUIDELINES SECRETARIAT
 Direct Coombs’ test  a  a  interstitial lung disease; PAH = pulmonary arterial hypertension
 Malaysian Health Technology Assessment Section (MaHTAS)
 Urine   Medical Development Division, Ministry of Health Malaysia
 UFEME   Level 4, Block E1, Precinct 1,
 Urine random protein:
 Federal Government Administrative Centre
 creatinine ratio OR   62590 Putrajaya, Malaysia  a
 a
 a
 24-hour urine protein  Tel: 603-88831229
 E-mail: htamalaysia@moh.gov.my
   =  indicated;     =  when indicated;     =  when indicated during pregnancy; -  =  not
 a
 a,p
 indicated; anti-dsDNA = anti-double stranded deoxyribonucleic acid; ENA = extractable
 nuclear antigen
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