MCRP Notes Rheum Flashcards
New name for Wegener’s
Granulomatosis with polyangitis
What is Granulomatosis with polyangitis
Autoimmune condition associated with a necrotizing granulomatous vasculitis
What does Granulomatosis with polyangitis affect?
Affects both the upper and lower respiratory tract as well as the kidneys
Main symptoms of Granulomatosis with polyangitis (GPA)?
Upper resp tract - epistaxis, sinusitis, nasal crusting.
Lower resp tract - dyspnoea and haemoptysis
Kidneys - rapidly progressive glomerulonephritis
Type of glomerulonephritis in GPA
Pauci-immune in 80%. Minimal hypersensitivity seen when staining with immunofluorescence for IgG
Extra symptoms of GPA
Saddle shaped nose deformity. Cranial nerve lesions. Eye involvement - proptosis. Vasculitic rash
Antibodies present in GPA
c-ANCA in over 95%, p-ANCA in 25%
What is seen in investigations in GPA
CXR and CT chest - cavitating lesions
Renal biopsy - epithelial crescents in bowmans capsule
Management of GPA
Steroids, cyclophosphamide (response in 90%), plasma exchange
Mean survival GPA
8-9 years
Similarities between GPA and Churg-Strauss syndrome
vasculitis, sinusitis, dyspnoea
Differences between GPA and Churg-Strauss syndrome
Churg-strauss = asthma, eosinophilia + pANCA GPA = c-anca, renal, epistaxis/haemoptysis
New name for Churg-Strauss syndrome
Eosinophilic granulomatosis with polyangitis
Organs affected by Churg-strauss
Skin, lungs, bowels, heart, nerves and kidneys
Features/stages of churg-strauss
Allergic phase - occurs 3-9years before the rest, asthma/allergic rhinitis, can get nasal polyps
Eosinophilic phase - high levels of eosinophils, esp in lungs and bowels. Get weight loss, night sweats, cough, abdo pain, GI bleeding
Vasculitic phase - worsening of all previous symptoms plus vessel disease/vasculitis. Can get blood clots and infarction of organs including severe heart disease
Treatment of churg-strauss
prednisolone, azathioprine, cyclophosphamide
Age of churg-strauss
Around 40’s. Rare for children or over 65 to be affected
What is goodpastures syndrome
Rare condition associated with pulmonary haemorrhage and rapidly progressive glomerulonephritis
What causes goodpastures syndrome
Anti-glomerular basement membrane antibodies against type IV collagen
Associated with HLA DR2
Features of goodpastures
Male:female is 2:1. Bimodal age distribution - peaks age 20-30 and age 60-70
What increases risk of pulmonary haemorrhage in goodpastures syndrome
Pulmonary oedema, smoking, LRTI, inhalation of hydrocarbons, young males
Investigations in goodpastures syndrome
Renal biopsy linear IgG deposits along basement membrane
Raised transfer factor after pulmonary haemorrhages
Management of goodpastures
Plasma exchange, steroids, cyclophosphamide
What is Behcets
Complex multisystem disorder characteristed with presumed autoimmune inflammation of arteries and veins
Main 3 symptoms of Behcets
Oral ulceration, genital ulceration, anterior uveitis
epidemiology features of behcets
M>F - more common and worse. Associated with HLA B5 More common in eastern Mediterranean Young adults 30% family history
Other symptoms of behcets
Arthritis, thrombophlebitis, neuro (aseptic meningitis), Gi (Abdo pain, diarrhoea + colitis), erythema nodosum, DVT
diagnosis of behcets
no definitive test, clinical diagnosis
+ve pathergy test is indicative (puncture site following needle prick becomes inflamed with small pustule forming)
