Rheumatology Flashcards
Rheumatoid Arthritis
What is the diagnostic criteria for rheumatoid arthritis?
> ACR 1987 requires 4 of the following for 6 weeks or more:
- Morning stiffness > 1 hour
- Symmetrical joint involvement
- Involvement of small joints of hands
- Arthritis affecting 3 or more joints
- Positive rheumatoid factor
- Rheumatoid nodules
- Radiographic evidence
Rheumatoid Arthritis
What are the poor prognostic markers in rheumatoid arthritis?
- Positive RF
- Positive Anti-CCP
- Early radiographic evidence of erosive disease
- Impaired functional status
- Persistently active synovitis
Ankylosing Spondylitis
What are the systemic manifestations of ankylosing spondylitis?
> Mnemonic 6A’s:
- Anterior uveitis
- Atlanto-axial subluxation
- Apical lung fibrosis
- Aortic regurgitation, Mitral valve prolapse, Atrioventricular conduction defects
- Amyloidosis (kidneys)
- (Autoimmune bowel disease - UC)
- Achilles tendonitis, Plantar fasciitis
- (Arthritis)
Psoriatic Arthropathy
What are the indications for TNF-a inhibitor therapy in rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, crohn’s disease?
>Rheumatoid Arthritis: -Not responding to Methotrexate & one other DMARD >Psoriatic Arthritis -Not responding to two or more DMARDs >Ankylosing Spondylitis -Not responding to two or more NSAIDs >Crohn's Disease: -Fistulating disease -Refractory to immune modulators
Psoriatic Arthropathy
What are the contraindications to TNF-a inhibitor therapy?
> Contraindications to TNF-a inhibitor therapy:
- Pregnancy, Breast feeding
- Active bacterial infection, Septic arthritis in previous 12 months
- Active tuberculosis (requires 2 months of anti-tuberculous prior to TNF-a inhibitor therapy)
- Latent tuberculosis (give isoniazid for 6 months alongside TNF-a inhibitor therapy)
- Demyelinating disease
- Congestive cardiac failure
Psoriatic Arthropathy
What are the side effects of TNF-a inhibitor therapy?
> Side Effects of TNF-a inhibitor therapy:
- Serious infection e.g. tuberculosis
- Haematological abnormalities (anaemia, leukopaenia, thrombocytopaenia)
- Haematological malignancy
- Worsening heart failure
Marfan’s Syndrome
What are the causes of high-arched palate?
- Marfan’s syndrome
- Turner syndrome
- Friedrich’s ataxia
- Tuberous sclerosis
Marfan’s Syndrome
What are the causes of lens dislocation?
- Marfan’s syndrome (up & out)
- Homocystinuria (down & in)
- Ehler Danlos syndrome
Osteomalacia and Rickets
What is the difference between Osteoporosis and Osteomalacia?
> Osteomalacia:
- Decreased mineralization
- Calcium, Phosphate or Vitamin D deficiency
- Symptoms of proximal myopathy & bone pain
- Can be associated with secondary hyperparathyroidism
- Treated with calcium, phosphate, vitamin D replacement
Vitamin D deficiency causes secondary hyperparathyroidism and exacerbates osteopenia/osteoporosis. Raised PTH levels induce phosphaturia/hypophosphataemia causing defect in mineralisation of osteoid (osteomalacia). Unlike osteoporosis, osteomalacia causes non-specific bone pain, severe proximal myopathy, impaired balance and raised alkaline phosphatase. (ref: Hall)
Osteogenesis Imperfecta
What is the molecular defect in osteogenesis imperfecta?
What are the clinical findings?
- Symptoms of brittle bones, blue sclerae, deafness
- Due to defects in type I collagen synthesis
- Treat with calcium & vitamin D supplementation, bisphosphonates
Polydactyly
What is the classification of Polydactyly?
What are the causes of Polydactyly?
>Classification -Post-axial: ulnar side of hand -Pre-axial: radial side of hand -Central: on middle three fingers >Causes: -Bardet-Biedl syndrome -Holt-Oram syndrome -Carpenter syndrome -Patau syndrome -Familial (AD inheritance) >Assoc. features include cognitive & cardiac deficits
Scleroderma
What is the diagnostic criteria for scleroderma?
> ARA Diagnostic Criteria requires 1 Major or 2 Minor criteria:
- Major criteria include sclerosis of skin affecting arms, face, neck
- Minor criteria include sclerodactyly, finger pulp atrophy, bilateral pulmonary fibrosis.
Systemic Lupus Erythematosus
What is the diagnostic criteria?
> ACR 1997: 4 of 11 = lupus (or Nephritis+ANA/dsDNA)
- “DUMP CARS HIA”
- Discoid rash
- Ulcers, Oral ulcers
- Malar rash
- Photosensitivity
- CNS, Neurologic disease a. Seizures OR b. Psychosis
- Arthritis - symmetrical, polyarticular; unlike RA - non-erosive; reducible; sometimes asymm.; and pain is out of proportion to swelling
- Renal, Kidney disease a. > 0.5g/day protein OR b. ≥ 3+ dipstick protein OR c. Cellular casts
- Serositis
- Haematological disorders a. Haemolytic anaemia OR b. Leukopenia OR c. Lymphopenia OR d. Thrombocytopenia
- Immunologic abnormalities (a) Anti-‐dsDNA (b) Anti-‐Sm (c) Antiphospholipid
- ANA is positive
Systemic Lupus Erythematosus
What are the causes of drug-induced lupus?
How can you differentiate drug-induced lupus from SLE?
> Causes of Drug-Induced Lupus:
-Procainamide (most common), Hydralazine, Isoniazid, Methyldopa, Quinidine, Phenytoin, Chlorpromazine, Carbamazepine, Sulphonamides, Tetracycline.
> Differentiating Drug-inducted Lupus from SLE:
-Drug-Induced Lupus has M:F ratio of 1:1, Less Renal/CNS involvement & More Lung involvement, Anti-histone antibodies, Resolution within days-weeks of drug discontinuation
Systemic Lupus Erythematosus
What is the WHO classification of lupus nephritis?
> Mnemonic: No Man Feared Diabetes Mellitus
- Class I: Normal
- Class II: Mesangial
- Class III: Focal Proliferative
- Class IV: Diffuse Proliferative
- Class V: Membranous
- Class VI: Sclerosing
What are side effects of methotrexate?
- hepatic toxicity
- pulmonary fibrosis
- haematological: low WCC, thrombocytopenia
Side effects of cyclosporin?
- renal impairment
- HTN
What are side effects and precautions when using Biologics?
- infection
- TB reactivation
- Allergic reaction
- no live vaccines
- CI in active HepB and C
- increase skin cancer
- not good in pregnancy
What is the differential for deforming symmetrical arthropathy?
- RA
- psoriatic arthritis
- SLE (jaccoud arthropathy)
- tophaceous gout
- other seronegative arthropathy
- very severe OA
What are the XR changes of RA?
- soft tissue swelling
- symmetrical joint space narrowing
- marginal joint erosions
- juxta-articular joint osteoporosis
Investigations for RA?
- RF, anti-CCP
- ESR/CRP
- Hb re: anaemia chronic disease
- XR
Management of RA?
Non pharm: Physio, splints, OT Pharm: - NSAID - DMARD: MTX, leflunomide, hydroxychloroquine, cyclo, azathio - Biologics: TNF, non TNF Surgical: joint replacement
What is on the SLE criteria list?
MD SOAP BRAIN Malar rash Discoid rash Serositis Oral ulcers Arthritis Photosensitive rash Blood abnormal: haemolytic anaem, leukopenia Renal disorder: proteinuria, GN ANA Immunologic disorder: dsDNA, anti-Sm, antiphospholipid Neuro disorder: psychosis or seizure 4 or more = dx of SLE
What are the investigations for SLE?
- ANA, dsDNA, anti-Sm, anti-ro (congenital CHBlock)
- anti U1RNP if MCTD suspected rather than pure SLE
- ESR, C3/C4, CH50
- FBC: hb, low WCC, low play
- UA: proteinuria, blood
- renal biopsy
- APS: anti cardiolipin, B2 glycoprotein, lupus anticoagulant
- MRI and LP if CNS lupus
Treatment of SLE?
- NSAID
- hydroxychloroquine: monitor eyes
- Ca blockers for raynaud
- pred for active disease
- anti coagulation if thrombosis/APS
- OP prevention (pred)
- AZA, cyclophosphamide, or MTX
What are features of Wegeners and Ix?
- lung and kidney involvement
- SOB, cough/haemoptysis, nasal congestion
- crackles throughout lungs
- c-ANCA
- urine: casts, dysmorphic red cells
What are features and Ix for Churg-Strauss?
Churg “Struassthma-panca” (EGPA)
- asthma, allergic rhinitis, eczema, peripheral neuropathy
- p-ANCA
- biopsy
What investigations for scleroderma?
- ESR
- folate, B12 - malabsorption
- anti centromere (CREST)
- anti scl70 (diffuse scleroderma)
- gastroscopy, esophageal manometry
- ILD: PFT, hrCT, 6min walk test
- TTE: pulm HTN
What is the treatment for scleroderma?
- avoid smoking
- PPI
- Ca blocker for Raynaud
- Abx for gut bacterial overgrowth
- PAH Rx: endothelin receptor antagonists, PDE inhibitors
- pred for pericarditis or early ILD
- cyclophosphamide for ILD
- ACEi for renal protection
- myeloablative autologous stem cell transplant
Clinical features of Dermatomyositis?
- Grottron papules
- shawl sign
- heliotrope rash
- arthritis
- proximal weakness
What is MTCD?
Overlap features of SLE, scleroderma, polymyositis
What antibodies for Dermatomyositis/polymyositis?
Anti jo1
Anti mi2
Anti U1RNP
Anti synthetase
1) Pattern of weakness in Dermatomyositis and polymyositis?
2) Pattern in inclusion body myosotis?
1) proximal
2) proximal lower limb and distal upper limb
What are the hand XR features of gout?
- tophi
- punched out erosions
- intraosseous lytic lesions
What are XR hand features of psoriatic arthritis?
- pencil in cup deformity
- distal erosive change with bone proliferation
What are extra articular features of RA?
- Raynaud
- dry eyes, scleritis
- neck pain: C1-2 subluxation
- pulmonary fibrosis
- pericarditis, IHD
- peripheral neuropathy
- anaemia
- Vasculitis: ulcers, mononeuritis multiplex
What is Felty Syndrome?
RA + neutropenia + splenomegaly
What are the main causes of death in SLE?
- infection
- renal failure
- lymphoma
- MI
What to test for for anti phospholipid syndrome?
- anti cardiolipin
- lupus anticoagulant
- b2 glycoprotein
Dx:
- Clinical: > 1 VTE or recurrent miscarriage or low platelets
- Lab: antiphospholipid antibodies
SLE lupus nephritis treatments
- Class 1/2/6 - no immunosuppression
- Class 3/4/(+/- 5) - immunosuppression
- Adjuncts - ACEi if ptn>0.5g/d, BP<130/80, statins for LDL<2.6, PLEX if TMA
