Systemic Lupus Erythematous (SLE)

Exam Cram

• Systemic auto-immune condition

• Presentation: photosensitive rash, arthralgia, nephritis

• ANA highly sensitive, anti-dsDNA highly specific

• Management depends upon site:

  • • • Arthralgia- hydroxychloroquine

      • Nephritis- MMF

Diagnosis

Systemic Lupus

Clinical & Immunological: total 4, including one from each 

Clinical 

• Cutaneous- malar rash, toxic epidermal necrolysis, photosensitive rash, discoid rash

• Oral ulcers

• Alopeci- non scarring

• Synovitis: > 1 joint swelling, tenderness, > 30mins morning stiffness

• Serositis: inflammation of pleural, pericardium, peritoneum

• Renal: membranous lupus nephritis - proteinuria +++, RBC casts

• Neurology: seizures, psychosis, mononeuritis multiplex, myelitis, peripheral or central neuropathy, acute confusion

• Anaemia- haemolytic

• Leukopenia

• Thrombocytopenia: PLT <100

Immunological

• ANA- c. 90% sensitivity, poorly specific

• Anti-dsDNA-  poorly sensitive, highly specific

• Anti-SM

• Anti-phospholipid/ lupus anticoagulant

• Low complement (C3 or C4)

• Positive direct Co-ombs test (absence of haemolysis)

*

Investigations II

• Anti Ro- associated with neonatal heart block

Management

Vital Organ Involvement (heart, kidneys, lungs, eyes)

Renal

1. Biopsy

2. MMF

3. Cyclophosphamide

Heart, lungs, eyes

1. ? Methotrexate/ steroids

Non Vital Organ involvement: 

Lupus Arthralgia

1. Hydroxychloroquine

2. Methotrexate

Malar Rash

1. Hydroxychloroquine

Membranous Lupus Nephritis

Considered a nephrotic syndrome. 

Diagnostic criteria (SLIC 2012)- 2/2

• Biopsy 

• ANA or anti-dsDNA +ve

Management

1. Biopsy

2. MMF

3. Cyclophosphamide

Drug Induced Lupus

Assessment

History: 

• Recent drug started

• Rash

Examination: 

• Erythematous photosensitive rash over skin exposed areas

Investigations: 

• Anti-histone antibodies positive

Diagnosis: 

• Symptoms + antihistone positive

Drug Causes

• • Lamotrigine, phenytoin, carbamazepine

  • Hydralazine, Isoniazid

  • Chlorpromazine

  • Procainamide, primidone

Discoid Lupus

Presentation

• Patchy erythematous rash

• Sun sensitive

• 30F

Management

1. Photoprotection- UVA & UVB

2. Topical steroids

3. Hydroxychloroquine [1]

References

1. Kuhn, A., Aberer, E., Bata-Csörgő, Z., Caproni, M., Dreher, A., Frances, C., Gläser, R., Klötgen, H.-.-W., Landmann, A., Marinovic, B., Nyberg, F., Olteanu, R., Ranki, A., Szepietowski, J.C. and Volc-Platzer, B. (2017), S2k guideline for treatment of cutaneous lupus erythematosus – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol, 31: 389-404. https://doi.org/10.1111/jdv.14053 

Page written in 2024.