Basics Flashcards
ANCA neutrophil cytoplasmic antibodies - small vessel vasculitidies
granulomatosis with polyangiitis
eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
microscopic polyangiitis
Common findings of ANCA
renal impairment caused by immune complex glomerulonephritis → raised creatinine, haematuria and proteinuria respiratory symptoms dyspnoea haemoptysis systemic symptoms fatigue weight loss fever vasculitic rash: present only in a minority of patients ear, nose and throat symptoms sinusitis
General approach to first-line investigations for pANCA testing
urinalysis for haematuria and proteinuria
bloods:
urea and creatinine for renal impairment
full blood count: normocytic anaemia and thrombocytosis may be seen
CRP: raised
ANCA testing (see below)
chest x-ray: nodular, fibrotic or infiltrative lesions may be seen
cANCA
granulomatosis with polyangitis
pANCA
eosinophilic granulomatosis with polyangiitis+ others
Mainstay for ANCA associated vasculitis
Immunosuppressive therapy
Features of ank-spon
HLA-B27 associated spondyloarthropathy - sex ration (3:1)
Typically a young man who presents with lower back pain and stiffness of insidious onset
stiffness is usually worse in the morning and improves with exercise
the patient may experience pain at night which improves on getting up
Clinical examination for ank-spon
reduced lateral flexion
reduced forward flexion - Schober’s test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
reduced chest expansion
Other features the A’s
Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis and cauda equina syndrome peripheral arthritis (25%, more common if female)
Later radiograph changes in ank-spon
Radiographs may be normal early in disease, later changes include:
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
Management of ank-spon
encourage regular exercise such as swimming
NSAIDs are the first-line treatment
physiotherapy
the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement
the 2010 EULAR guidelines suggest: ‘Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments’
research is ongoing to see whether anti-TNF therapies such as etanercept and adalimumab should be used earlier in the course of the disease
Antiphospholipid syndrome features
venous/arterial thrombosis recurrent fetal loss livedo reticularis thrombocytopenia prolonged APTT other features: pre-eclampsia, pulmonary hypertension
Antiphospholipid happens secondary to which disease
SLE
Associations other than SLE in anti-phospholipid syndrome
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)
Antisythetase syndrome features
myositis
interstitial lung disease
thickened and cracked skin of the hands (mechanic’s hands)
Raynaud’s phenomenon
Adverse effects of azathioprine
bone marrow depression
nausea/vomiting
pancreatitis
increased risk of non-melanoma skin cancer
Behcet’s syndrome features
classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
thrombophlebitis and deep vein thrombosis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum
Diagnosis of Behcet’s
no definitive test
diagnosis based on clinical findings
positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)
What are bisphosponates
Analogues of pyrophosphate, molecule which decreases demineralisation in bone.
Inhibit osteoclasts by reducing recruitment and promoting apoptosis
Adverse effects of bisphosphonates
oesophageal reactions: oesophagitis, oesophageal ulcers (especially alendronate)
osteonecrosis of the jaw
increased risk of atypical stress fractures of the proximal femoral shaft in patients taking alendronate
acute phase response: fever, myalgia and arthralgia may occur following administration
hypocalcaemia: due to reduced calcium efflux from bone. Usually clinically unimportant
What should be corrected before giving bisphosphonates
Hypocalcaemia/ vitamin D deficiency
Benign bone tumours
Osteoma
Osteochondroma- exotosis
Giant cell tumour
Malignant tumours
Osteosarcoma
Ewing’s sarcoma
Chondrosarcoma
Osteoma features
benign ‘overgrowth’ of bone, most typically occuring on the skull
associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)