Connective, Vasculitis, Amyloid, Sarcoid Flashcards
SLE
Autoimm, Type III hyperseinsitivity. RF: Afrocab, women, drugs, classical complement def. HLA: DR3. Ab: a-dsDNA, ANA, a-Smith. (Drug-induced: a-Histone.) Histology: - LE bodies, - kidney, - CNS, - Spleen: onion skin lesions - Heart: Libman-Sack Endocarditis.
SLE - S/Sx
4 of Soap brain MD:- Serositis Oral ulcers Arthritis Photosensitivity Blood disorders (AIHA, ITP, luekopaenia) Renal ANA +ve Immune phenomena: a-dsDNA, a-Smith, a-phospholipid; Neuro Sx Malar rash Discoid rash.
Limited scleroderma
= CREST
HLA: DR5 and DRw8
Ab: a-Centromere
Histology: Increased collagen in skin and organs; onion skin thickening of arterioles.
Limited scleroderma - S/Sx
Skin changes on face and distal to elbows/knees. Calcinosis, Raynaud's, Esophageal dysmotility, Sclerodactyly, Telangectasia.
Assoc pulmonary hypertension.
Diffuse scleroderma
HLA: DR5 and DRw8 (same)
Ab: a-Scl-70, a-Topo.
Histology: Inflam in / around muscle fibres.
Skin can change anywhere. Organ involvement (pulmonary fibrosis).
Polymyositis and Dermatomyositis
assoc. underlying malignancy.
Ab: a-Jo-1 (=tRNA synthetase).
Histology: Endomysial (in muscle) inflam infiltrate.
Polymyositis and Dermatomyositis - S/Sx
Proximal muscle weakness, ^CK, abnormal EMG.
DM has heliotrope rash and Gottron’s papules.
Takayasu’s arteritis
Large vessel vasculitis.
Japanese women.
Pulseless, bruits, claudication.
Temporal arteritis
Large vessel vasculitis.
Elderly, scalp tenderness, headache,jaw claudication, blurred vision.
^ESR.
Overlap with polymyalgia rheumatica.
Polyarteritis nodosa (PAN)
Mainly renal.
Never lungs.
30% have Hep B.
Microaneurysms on angiography.
Kawasaki’s disease
Children 5 days, rash, desquamation, strawberry tongue, cervical LN.
Coronary arteries may have aneurysms.
Thrombangitis obliterans
Buerger’s disease
Heavy smokers.
Tibial and Radial artery inflam: pain, ulcers.
Corkscrew appearance on angiogram.
Wegener’s granulomatosis
Granulomatosis with polyangitis.
Triad: - saddle nose, epistaxis, sinusitis; - pulmonary haemorrhage; - crescentic glomerulonephritis (subtype 3). cANCA
Churg-Strauss
Asthma, hay fever, eosinophilia.
pANCA
Microscopic polyangitis
Pulmonary renal syndrome:
- Pulmonary haemorrhage,
- Glomerulonephritis.
pANCA
Henoch-Schonlein Purpura
IgA mediated vasculitis.
Children
Amyloidosis - causes
Primary (AL) is most common; usually Bence Jones protein due to things like Multiple Myeloma.
Secondary:
- AA, acute phase protein, so any chronic inflam/infection;
- Haemodialysis, beta2-microglobulin deposition;
- Familial, several kinds, all rare, most seen is Familial Mediterranean Fever: IL-1 causes fever and inflam.
Amyloidosis - Clinical features
- Kidney: nephrotic syndrome;
- Heart: conduction defects, heart failure;
- Liver/spleen: both big;
- Tongue: Macroglossia in 10%;
- Neuropathies: carpal tunnel.
Amyloidosis - Dx
Congo red stain shows apple green birefringence under polarised light
Sarcoidosis
Non-caseating granulomas; Schaumann and asteroid bodies.
More common, and worse in Afrocabs; F>M.
Lungs.
Seen on CXR - bilateral hilar lymphadenopathy.
Pulmonary infiltrates: fine nodular shadowing in mid zones.
Presents with insidious SOB, cough, chest pain, night sweats.
Ix: ^Ca, ^ESR, ^ACE.
Sarcoidosis -
extra-pulmonary manifestations
- Skin: erythema nodosum, lupus pernio;
- LNs: painless rubbery;
- Eyes: anterior uveitis (misting, pain), or posterior; (Uveoparotid fever = b/l uveitis, big parotids, facial N palsy)
- Hepatosplenomegaly;
- Low WCC, low Hb;
- ^Ca, calculi;
- Heart: arrhythmia, cardiomyopathy.
