Systemic Lupus Erythematosus

Who can see the pain within my heart?

COMPARE……..

Epidemiology

Clinical feature

Manifestation Prevalence(%)

Fatigue/malaise/fever 95Cutaneous 80hematologic 85Neurologic 60Cardiopulmonary 60Renal 30Gastrointestinal 40

Thrombosis 15

Investigation:

Screening: ANA

Specific:Anti-ds DNA

Diasease activity;Complement, Anti ds DNA, active

sediment in urine

SlICC clinic criteria for SLEClinical manifestation Immunologic

manifestation Acute cutaneous Chronic cutaneous Oral ulcer Serositis Alopecia Synovitis Renal Neurologic Hemolytic anemia Leucopenia thromcytopenia

ANA Anti-ds DNA Anti-Sm Antiphospholipid Low serum

complement Positive DCT4 CRITERIA AT ALEAST 1

CRITERIA EACH FROM CLINICAL AND IMMUNOLOGICAL

BIOPSY PROVEN LUPUS NEPHRITIS+ANA/ANTI DS DNA

Treatment

Not life or organ

threatening

Life or organ threatening

High dose glucocorticoids

Addition of immunosuppressive

Mycophenolate/cyclophosphamide

What about ?

hydoxychlroquinine

Scleroderma

Definition

Connective tissue disorder, involve multisystem

Characterised by skin thickening

Epidemiology

Female predominant(4.6:1)

Peak age of onset: 30-50 years

Types of systemic sclerosisCharacterstics Limited

cutaneousDiffuse cutaneous

Skin involvement Limited distal to elbow

Diffusely involve

Raynauds phenomenon

Precedes the skin involvement

Coincident

ILD Mild Common and severe

Renal crisis Rare Common

Antibodies Anticentromere Ab Anti-topoisomerase Ab.

Clinical features

Raynaud phenomenon

INVESTIGATION

Diagnosis SupportiveACA: limited cutaneous

SSC

Anti-toposisomerase I: Diffuse cutaneous SS

Full thickness skin biopsy

ECHO:

PFT:

UGI endoscopy:

Nail cappillaroscopy:

Treatment

Definative supportive Disease modifying

therapy

Glucocorticoids:only if indicated

Cyclophosphamide:for ILD

Mehtotrexate/mycophenolate mofetil:little benefit

Antifibrotic therapy:D-penicillamine

Vascular therapy:Calcium channel blocker

gastrointestinal :

Pulmonary artery hypertension:

Renal crisis:

Treatment

No any treatment to significantly alter the course the natural history of disease.

Thank you