Tuesday 22 September 2015

SLE


Ø  Lupus means wolf ,manifestation like hungry wolf

Ø  It is autoimmune multisystem disease

Ø  Etiology-Unknown

Ø  Pathogenesis-Ab against every component of cell

Ø  Asian 0.03%

Ø  Black 0.2%

Ø  Onset-20-30 years ,gradual

Ø  F:M =8:1


C/F
Ø 
Fever ,malaise ,mayalgia ,arthralgia

Ø  Photosensitivity-Butterfly rash under sun(Discoid rash in DLE)
Ø  Fall of scalp hair
Ø  Signs of arthritis are less common(Jaccoud's deformity)
Ø  Spontaneous abortion

Ø  Pericarditis ,effusion
Ø  Pleurisy ,effusion ,consolidation
Ø  Red urine
Ø  Anemia/leukopenia/pancytopenia
Ø  Psychosis ,seizures ,convulsion
Ø  General lymphadenopathy

Examination-Anemia ,lymphadenopathy ,loss of hair ,rash ,oral ulcer over hard palate

Systemic-Acc. to systemic features

Diagnostic criteria/ARA(11)

Ø  Butterfly rash
Ø  Discoid rash
Ø  Oral ulcer
Ø  Photosensitivity
Ø  Arthritis(Non erosive ,2/more peripheral)
Ø  Serositis
Ø  Renal-Proteinuria >0.5g/day ,3+ ,cellular cast
Ø  Hematologic-Anemia/leukopenia ,4000/cmm/thrombocytopenia <100000/cmm
Ø  Neurologic -
Ø  +ve ANA Ab ,
Ø  +ve Anti DS DNA Ab

[4/more-Definite SLE]

Complication

Ø  Renal-AGN ,NS ,ARF
Ø  CVS-Pericarditis ,HF ,premature MI/due to vasculitis
Ø  CNS-Psychosis ,convulsion
Ø  DVT ,pul embolism

Investigation

Ø  BLD-TC ,DC  WBC ,HB ,ESR ,total bilirubin
Ø  Routine-Total urine protein(>3.5 g/1.3 sq.m/day in adult)
Ø  Urine RME-RBC cast
Ø  CXR
Ø  ECG-IHD/HB
Ø  ANA-80% +ve(So here Anti DS DNA Ab +ve 30%)
Ø  Anti phospholipid Ab(In abortion pt/chance of thromboembolism)

[Prognosis depends on renal system/vital organ involvement]


Histological change of renal involvement
Ø  Every glomerular disease may occurs





DD/+ve ANA

Ø  Dermatomyositis
Ø  Polymyositis
Ø  Mixed connective tissue disease/MCTD
Ø  Sarcoidosis
Ø  Rh arthritis

DD of butterfly rash

Ø  SLE ,DLE
Ø  Dermatomyositis
Ø  MCTD
Ø  Sarcoidosis
Ø  PKDL

Rx

Ø  Nature of disease  must be explained

Ø  Mild to moderate/no renal involvement-For pain give NSAIDs ,hydroxychloroquine,if not controlled  then give prednisolone 30-60mg/day

Ø  If renal involvement
                          Methylprednisolone 10mg/kg/day IV for 3 days
                               Followed by Oral prednisolone 30-60mg/day
                                   Methotrexate 20-30mg/wk

                                          If side effects>Slowly decrease the dose and add chemotherapy