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Me d Ed Y3 Wri tte n Exa m L e ct ure s 2 02 5 Liz z ie Varaksin a | ev3 21@ic. ac . uk Rheumatology and V asculidities L EC T U R E OV E RV IE W : VACU L IT IS CON NEC T IV E T IS S U E D IS EA SE CONDIT IONS : • Polymyalgia rheumatica • Behcet’s Disease • Polyarteritis Nodosa • Churg Strauss • Wegener’s • Microscopic Polyangiitis • GCA • Sarcoidosis • SLE • Sjogren’s Syndrome • Systemic Sclerosis • Poly/dermatomyositis R ea ct ive a r th rit is C PPD A nk y lo sin g s po nd y litis G ou t Ps or iat ic a rt hr it is E nte r op ath ic a rt hr it is crystal arhtropathies seronegative spondylo arth ropathies me tabo lic bone dise as es sept ic arthrit is Os te o m ala cia / r ick et s R HE U M Os te o po ro sis fi bromyalg ia Page t ’s d ise a se OV E RV I E W : osteoarthriti s connect ive t issue dis eases rheumatoid arthriti s S cle r od e r m a PM R vasculi tis S LE S m a ll v e ss e l Me d ium ve s s el La rge v e s se l PM / D M R ea ct ive a r th rit is C PPD A nk y lo sin g s po nd y litis G ou t Ps or iat ic a rt hr it is E nte r op ath ic a rt hr it is crystal arhtropathies seronegative spondylo arth ropathies me tabo lic bone dise as es sept ic arthrit is Os te o m ala cia / r ick et s R HE U M Os te o po ro sis fi bromyalg ia Page t ’s d ise a se OV E RV I E W : osteoarthriti s connect ive t issue dis eases rheumatoid arthriti s S cle r od e r m a PM R vasculi tis S LE S m a ll v e ss e l Me d ium ve s s el La rge v e s se l PM / D MVA S C U L IT IS: Definition: • Autoimmune • Inflam of vessels → vascular lumen compromise and ischaemia of the end-organ • ARTERIES MOST COMMONLY AFFECTEDVA C U L ITI S PAT HO PH YS : 1. DIRECT = Endothelial layer (large/medium) 2. INDIRECT = Cells closely involved with endothelium (small) • damage to endothelium = weak walls = aneurysms • ischaemia: ⚬ exposed collagen & TF → ↑coagulation → restrict blood flow ⚬ fibrin deposits in healing → stiffening walls → mechanical reduction in size of lumen GIA N T C EL L ART E RI TIS : Perfect patient: MRS SMITH • >60 (preferably 70) • PC: sudden visual loss • HPC: has been suffering with headaches (new) past 1/52, ass. w/ jaw claudication, also has morning stiffness in proximal limbs • FLAWS: low grade fever, lethargy, anorexia, night sweats investigations: • ↑ESR, ↑(CRP) • Fundoscopy: swollen pale disc and blurred margins (anterior ischameic optic neuropathy) TREATMENT: • Temporal artery biopsy • ↑↑↑ corticosteroids! ASAP ⚬ ↑ prednisolone if no visual loss • CK, EMG norm ⚬ IV methylprednisolone if visual loss then ↑pred • refer to ophthalPO LYA RT ER IT IS N O DO S A : Perfect patient: MR SMITH • man in 50s • PC: unspecific FLAWS signs, haematuria, arthralgia investigations: O/E: • fibrinoid necrosis of all layers of endothelium = • livedo reticularis aneurysm prone • ↑BP • blood in urine sample • hep B +ve • ANCA +ve (only 20%) TREATMENT: • ↑↑↑ corticosteroids ⚬ + Cyclophosphamide if severe organ- threatening disease • remission with azathioprine / methotrex + continued corticosteroids • ANCA associated: (anti-neutrophil- S M A L L V ES S E L VAS C UL I TI S cytoplasmic antibodies) M IC RO SC O PIC W EG EN E R ’ S C H U RG - S TR AU S S • granulomatosis with polyangiitis • glomerulonephritis → renal • eosinophilic granulomatosis with failure polyangiitis • diffuse alveolar haemorrhage → Upper resp tract: haemoptysis • epistaxis • severe asthma, allergic rhinitis, • heaing loss sinusitis Ix: ↑creatinine, haematuria, • sinusitis proteinuria O/E: saddle shaped nose (nasal bridge Ix: ↑eos! pANCA ↑pANCA (against MPO), cANCA collapse) (against PR3) Associations: Lower resp tract: • cough, wheeze, haemoptysis • UC • PSC cytoplasmic = cANCA = serine proteinase 3 ab • Crohn’s perinuclear = pANCA = myeloperoxidase ab Ix: cANCA, epithelial crescents • Anti-GBM Bowman’s capsule (renal failure)VA C U L IDIT IES SU M M ARY: Large: • Temporal arteritis (giant cell arteritis) • Takayasu arteritis (<40y F, Asian, arm claud) Variable vessel: • Behcet’s - HLAb51 Medium: ⚬ oral ulcers (red halo) • Polyarteritis nodosa ⚬ genital ulcers ⚬ anterior uveitis • Kawasaki disease ⚬ GI, neuro, arhtritis, erythema nodosum, thrombophlebitis, DVT Small: • Immune complex associated: ⚬ IgA (Henoch-Schonlein purpura) Ix: pathergy test (small pustule after puncture) → erythema with induration ⚬ Goodpasture's syndrome (anti-glomerular basement membrane disease) ⚬ Cryoglobulanaemic arteritis • ANCA associated: ⚬ Granulomatosis with polyangiitis (Wegener’s) ⚬ Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) ⚬ Microscopic polyangiitis PO LYM YA LGIA R H EU M AT IC A Perfect patient: MR GREEN • NECK • SHOULDER GIRDLE • >60s • HIP GIRDLE ⚬ pain and stiffness (no weakness o/e) • PC: aching morning stiffness in proximal limbs (NO ￭ morning (>45mins) WEAKNESS), depression, FLAWS, ￭ after rest or inactivity ￭ poor sleep investigations: • ↑ESR • NORMAL CK AND EMG TREATMENT: • ↑↑↑ corticosteroids ⚬ PREDNISOLONE 15mg/OD → if not resolving then likely alt diagnosis S J O GR EN ’ S Perfect patient: MRS BROWN • exocrine glands affected! (sicca sx) • 40yo • PC: DRY EYES, dry mouth, vaginal dryness, arthralgia, Raynaud’s, parotitis, sensory polyneuropathy • PMH: RA diagnosed 10y ago, recently developed lymphoid malignancy investigations: • RF +ve (50%) • ANA +ve (70%) • anti-Ro (SSA) (70%) • anti-La (SSB) (30%) TREATMENT: • artificial saliva and tears • Schirmer's test: filter paper near conjunctival sac ⚬ pilocarpine to measure tear formation (<10mm) • vaginal lubricants • Hypergammaglobulinaemia, low C4S YS TE MIC SC L ERO SI S :S YS TE MIC SC L ERO SI S : DI F F U SE : L I M I TE D C U TAN EO U S ( C R ES T) : • anti-Scl 70 (anti-topoisomerase) • anti-centromere • trunk and proximal limbs predominately • face and distal limbs predominately • Alveolitis and ILD • Pulm HTN • Scleroderma renal crisis (SRC) potentiated by • Renal sparing glucocorticoids CREST! • RAPID • PUFFY SKIN changes (fibrosis)M YO S I TIS • muscle inflammation → proximal muscle weakness • ass. w/ malignancy (breast, ovarian, lung) → screening post diagnosis PO LYM YO SIT IS DE R M ATO M YO S IT IS • T cell mediated cytotoxic • O/E: ⚬ Gottron’s papules (knuckles, elbows, knees) • O/E: ⚬ no skin features! ⚬ Heliotrope rash ⚬ prox muscle weakness +/- tenderness ⚬ Periorobital oedema ⚬ Raynaud’s ⚬ Photosensiivie erythematous rash ⚬ resp muscle weakness (fibrosing alveolitis) ⚬ prox muscle weakness +/- tenderness ⚬ Raynaud’s ⚬ resp muscle weakness (fibrosing alveolitis) investigations: • ↑CK, ↑LDH, ↑AST, ↑ALT investigations: • anti-Jo-1 ab TREATMENT: • ANA +ve • ↑↑↑corticosteroids • anti-synthetase ab: • EMG, MRI, muscle biopsy ⚬ histidine-tRNA ligase (Jo-1) • immunosuppresants (methotrex, azathioprine) • IV ig ⚬ anti SRP (signal recognition • biologics (infliximab, etanecerpt) particle) ⚬ anti-Mi-2S L E Perfect patient: MRS MOORE • 35yo, Afro-Caribbean • type 3 hypersensitivity - immune complex formation PC: fatigue, mouth ulcers, fever, lymphadenopathy, • HLA B8, DR2, DR3 skin changes, arthralgia, cough • neuro, renal (diffuse prolif glomerulonephritis), cardio (pericarditis) , MSK involvement investigations: • ANA +ve • RF +ve • anti-dsDNA highly specific • O/E: • anti-Smith • malar rash (sparing nasolabial folds) • Anti-U1 RNP, anti-RO, anti-La TREATMENT: • anti-phospholipid antibiodies • hydroxychloroquine • discoid rash (antiphospholipid syndrome) • photosensitivity • if other organ involved = pred, • ulcers cyclophosphomide • anaemia, lymphopenia, • serositis (pleuritis, pericarditis) thrombocytopenia • Raynaud’s • livedo reticularis • ↑ESR • alopecia • ↓C3, C4S ARC O ID O SIS CXR changes!!! • stage 0 = normal • stage 1 = bilateral hilar lymphadenopathy • non-caseating granulomas (US guided (BHL) mediastinal lymph node biopsy) = • stage 2 = BHL + interstitial infiltrates hypercalcaemia • stage 3 = diffuse interstitial infiltrates only LOFGREN’s = bilat hilar lymphadenopathy, • stage 4 = diffuse fibrosis erythema nodosum, fever, polyarthralgia HEERFORDT = parotid enlargement, uveitis, fever • dry cough • SOB TREATMENT: • erythema • steroids indication: nodosum ⚬ stage 2 or 3 symptomatic • lupus pernio ⚬ hypercalc • FLAWS ⚬ eye, heart, neuro involvementT HA N K YO U ! 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