Systemic lupus erythematosus Flashcards
what is the definition of lupus?
SLE is a chronic multisystem disorder that most commonly affects women during their reproductive years. It is characterised by the presence of antinuclear antibodies. In addition to constitutional symptoms, it most frequently involves the skin and joints, although serositis, nephritis, haematological cytopenias, and neurological manifestations may occur during the course of the disease.
what is the epidemiology of lupus?
Rare
Higher in afro caribbean ethnicity
Mainly women
what is the aetiology of lupus?
Unknown
Genetic:
Predisposing genes (HLA) may activate the innate or adaptive immune response, or have a potential role as self-antigen for autoreactive B cells
Environmental:
The most important non-infectious causative agents are drugs. The first reported association was with procainamide, but other commonly implicated drugs include minocycline, terbinafine, sulfasalazine, isoniazid, phenytoin, and carbamazepine
what are the risk factors for lupus?
Female Over 30 African decent Drugs Sun exposure, family history, smoking
what is the pathophysiology of lupus?
Autoimmune Inflammation leads to tissue damage
Thrombosis - due to phospholipid antibodies
NETosis, cell death in which neutrophil extracellular traps are released, is increasingly recognised as a source of nuclear antigens and bioactive molecules that may facilitate autoimmunity in SLE
Several mechanisms have been proposed, by which T-cell dysregulation of B cells may arise, resulting in autoimmunity.
what are the key presentations of lupus?
Malar (butterfly) rash Photosensitive rash Discoid rash Similar symptoms to RA Kidney involvement = glomerulonephritis CNS involvement = seizures and psychosis GI - mouth ulcers
what are the signs of lupus?
Malar rash Photosensitive rash Discoid rash Fibromyalgia Raynauds Thrombosis Nephritis - hypertension, proteinuria, renal failure Lymphadenopathy Neuro-psychiatric features
what are the symptoms of lupus?
Rash Fever Weight loss Fatigue Oral ulcers Alopecia Chest pain Abdo pain and vomiting Arthritis (similar to RA)
what are the first line and gold standard investigations for lupus?
FBC - low haemoglobin, low WBC, neutropenia, pancytopenia (QUEREY CANCER), anaemia, thrombocytopenia
Activated partial thromboplastin time - prolonged
Urea and electrolytes - elevated urea and creatinine
ESR - elevated
CRP - normal
Antinuclear antibodies - positive in 95%
Urinalysis - haematuria, casts, proteinuria (NEPHRITIS)
CXR
ECG - rule out other chest pain causes
what are the differential diagnoses for lupus?
Acute pericarditis, RA, antiphospholipid syndrome, systemic sclerosis
how is lupus managed?
Reduce sun exposure Topical treatments NSAIDs Antimalarial Steroids - prednisolone Cytotoxic - azathioprine, mycophenolate, ciclosporin, methotrexate, cyclophosphamide Anticoagulants Biological Stem cell transplant
how is lupus monitored?
There is as yet no consensus on the value of serological tests and complement levels to follow up patients. In general terms, changing levels should prompt more vigilant monitoring.
In assessing a patient with a presumed flare of symptoms, it is important to exclude infection and comorbidity as an explanation.
Patients with lupus nephritis should have their renal function monitored (renal biopsy, kidney function tests, urinalysis).
what are the complications of lupus?
Anaemia, leukopenia, thrombocytopenia, corticosteroid related cataracts
what is the prognosis of lupus?
Patients with SLE have higher rates of death from all causes, regardless of sex, ethnicity, renal disease, cardiovascular disease, or infection. However, the risk of death due to malignancy is not increased
