Systemic lupus erythromatosous Flashcards Preview

Internal Medicine > Systemic lupus erythromatosous > Flashcards

Flashcards in Systemic lupus erythromatosous Deck (15):
1

Define

Multisystem
Autoimmune, autoantigens

2

Pathophysiology

Polyclonal B cell secretion of autoantibodies->immune complexes, deposit

3

Epidemiology

+++Women
Reporductive age
10% relatives affected

4

Clinical features

Relapsing and remitting
Malaise, fatigue, weight loss, myalgia, fever
Raynauds
oral ulcers
nose ulcers
alopecia
arthralgia/arthritis
fibromyalgia
poorly localised proximal limb inflammatory pain with weakness
Raynaud's phenomenon
chest pain and shortness of breath
dysrhythmias (e.g., tachycardia), conduction defects, or unexplained cardiomegaly
hypertension
signs of nephrosis (e.g., oedema)
lymphadenopathy
CNS signs: seizures, cranial nerve abnormalities, cognitive defects, psychosis
venous or arterial thrombosis
abdominal pain, vomiting, or diarrhoea
dysphagia

5

Immunology

95% +ve for ANA
anti-dsDNA highly specific, only 60% +ve
Anti-Smith, Anti-Ro, Anti-cardiolipin
Associated with other AI conditions->Sjogrens, thyroid

6

Investigations

Anti-dsDNA antibody titres
ANA
Complement->reduced C3, C4
ESR->elevated
BP
Urine for casts, protein
FBC->normochromic, normocytic anemia, thrombocytopenia, leukopenia
UEC->+urea and creatinine
LFTs
CRP usually normal
aPTT->prolonged if APS
CXR->effusions, cardiomegaly

7

Drug-induced

Isoniazid
Hydralazine
Procainamide
Quinidine
Chlorpromazine
Skin and lung signs++

8

CLOT abnormalities in APS and treatment

Anti-cardiolipin, lupus anticoagulant
Coagulation defect
Livedo reticularis
Obstetric complications
Thrombocytopenia

Use low dose aspirin

9

Management of

Lifestyle: smoking cessation, diet, exercise, sun protection/avoidance for ALL
1. Arthritis
->Naproxen
If inadequate:
->Hydroxychloroquine
->Prednisilone
->Methotrexate + folic acid
2. Mucocutaneous
->Oral hygeine
->Lidocaine gel
->Antiseptic mouth wash
->Prednisilone, hydroxychloroquine, methotrexate + folic acid
3. Nephritis
->Induction therapy (cyclophosphamide) + prednisilone + hydroxychloroquine
->Maintenance with azathioprine and prednisilone

10

Steroid sparing agents

Methotrexate
Mycophenolate
Azathioprine

11

Prognosis

80% survival at 15 years

12

Revised criteria for diagnosis of SLE

A RASH POINTS MD
Any ≥4 of the 11 criteria are required to classify a patient as having SLE. These criteria can be present serially or simultaneously during any interval of observation.
1. Malar rash
->Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds.
2. Discoid rash
->Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions.
3. Photosensitivity
->Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation.
4. Oral ulcers
->Oral or nasopharyngeal ulceration, usually painless, observed by physician.
5. Arthritis
Non-erosive arthritis involving ≥2 peripheral joints, characterised by tenderness, swelling, or effusion.
6. Serositis (one of the following):
->Pleuritis: convincing history of pleuritic pain, pleural rubs on auscultation, or evidence of pleural effusion.
->Pericarditis: documented by ECG, pericardial rub, or evidence of pericardial effusion.
7. Renal disorder (one of the following):
->Persistent proteinuria >0.5 g/day or >3+ if quantification not performed.
->Cellular casts: may be red cell, haemoglobin, granular, tubular, or mixed.
8. Neurological disorder (one of the following):
->Seizures: in the absence of offending drugs or known metabolic derangements; for example, uraemia, ketoacidosis, or electrolyte imbalance.
->Psychosis: in the absence of offending drugs or known metabolic derangements; for example, uraemia, ketoacidosis, or electrolyte imbalance.
9. Haematological disorder (one of the following):
->Haemolytic anaemia: with reticulocytes
->Leukopenia, Thrombocytopenia
10. Immunological
->Anti-DNA: presence of antibody to native DNA in abnormal titre
->Anti-Smith: presence of antibody to Smith nuclear antigen
->Positive findings of antiphospholipid antibodies based on:
An abnormal serum level of IgG or IgM anticardiolipin antibodies
->Positive test result for lupus anticoagulant using a standard method
->A false-positive serological test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilisation or fluorescent treponemal antibody absorption (FTA) test.
->Anti-nuclear antibody
11. An abnormal titre of anti-nuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome.

13

Most important complication of SLE

Renal involvement
Hypertension, CRF, nephrotic, ESRD

14

Main causes of death

Renal
CNS
Infection
Vascular disease

15

Which infection can show a false positive

VDRL->syphillis