5s: Immune Disorders* Flashcards
SOAP BRAIN MD for SLE (Systemic Lupus Erythematosus)
- Serositis
- Oral ulcers
- Arthritis
- Photosensitive rash
- Blood (all counts low)
- Renal (proteinuria)
- ANA = used to screen for ANY nuclear antigens
- Immunological (anti-dsDNA)
- Neurological (psych, seizures)
- Malar rash
- Discoid rash
What are the three auto-antibodies in SLE
dsh
Anti-dsDNA (MOST SPECIFIC) = most specific (30%), not very sensitive
Anti-smith (Sm) = specific (20%), not very sensitivie
- NB: all anti-ENAs (RNPs: anti-Ro, La, Sm, U1RNP) found in SLE
Anti-histone (drug-related e.g. hydralazine)
- patients taking hydralazine for HTN may develop SLE
How do we measure anti-dsDNA?
Incubate patient’s serum with Crithidia Luciliae (a protozoa)
- has big mitochondrion with dsDNA (kinetoplast)
- if the patient has anti-dsDNA antibodies, it will bind to the DNA
Measured using ELISA
NB: old test looks for LE cells (neutrophils that have taken up denatured nuclei)
How do we measure anti-dsDNA?
Incubate patient’s serum with Crithidia Luciliae (a protozoa)
- has big mitochondrion with dsDNA (kinetoplast)
- if the patient has anti-dsDNA antibodies, it will bind to the DNA
Measured using ELISA
NB: old test looks for LE cells (neutrophils that have taken up denatured nuclei)
Skin histology of SLE (3)
- lymphocytic infiltration of dermis
- vacuolisation (cell dissolution) of basal epidermis
- Extravasation of RBCs → rash
- IF (antibody to IgG) will show immune complex deposition of the epidermis-dermis junction
Renal histology of SLE (1)
Thick glomerular capillaries (wire-loop)
IF to visualise immune complex deposition (dark areas on EM)
Renal histology of SLE (1)
Thick glomerular capillaries (wire-loop)
IF to visualise immune complex deposition (dark areas on EM)
What heart problem is associated with SLE?
Libman-Sacks Endocarditis
- non-infective form of endocarditis
- emboli, HF, murmurs
- Vegetations = lymphocytes, neutrophils, fibrin stands etc.
Investigation of connective tissue disease
Investigation of connective tissue disease
Lupus can affect complement
Active lupus can cause secondary deficiency of classical pathway (C1,2,4) complement due to production of immune complexes depleting complement
What susceptibility allele is associated with SLE?
HLA-DR⅔
How do we treat SLE?
Mainly analgesia, steroids
Cyclophosphamide (chemo)
SLE summary
What is Scleoderma (systemic sclerosis)? and its types?
fibrosis and excess collagen (localised form is called morphoea in the skin)
Diffuse = involves the trunk (CREST + GIT + interstitial pulmonary disease and renal problems), F>M
Limited = NOT the trunk (CREST) → high risk of lung fibrosis and renal crisis
- Calcinosis (i.e. calcium deposit on tip of thumb)
- Raynaud’s phenomenon
- Eosophageal dysmotility
- Sclerodactlyly
- Telangiectasia
What are the autoantibodies in systemic sclerosis (diffuse vs limited) which one has a specific pattern
Diffuse
- Topoisomerase/Scl70
- RNA Pol I/II/III
- Fibrillarin (NUCLEOLAR PATTERN)
Limited
- Anti-centromere antibody
Skin histology in SS
Increased depth and amount of collagen → reduced skin elasticity
- difficulty swallowing and stomach dysmotility due to excess collagen within the lining
Vascular histology
- Top picture is a normal artery with 3 layers
- Bottom picture is a small artery in a patient presenting to A&E with a renal crisis (characterised by very high blood pressure)
- There is intimal proliferation giving an onion skin appearance
- Lumen effectively obliterated and some small thrombi form
Vascular histology
- Top picture is a normal artery with 3 layers
- Bottom picture is a small artery in a patient presenting to A&E with a renal crisis (characterised by very high blood pressure)
- There is intimal proliferation giving an onion skin appearance
- Lumen effectively obliterated and some small thrombi form