Rheumatology Flashcards
Name the type of vasculitis’s there are:
Large:
- Temporal/ Giant
- Takayasu arteritis
Medium:
- Kawasaki
- Polyarteritis nodosa
- Buerger’s disease
Small:
- Henock Scholien
- Granulomatosis with polyangiitis
- Eosinophilic Granulomatosis with polyangiitis (Chaurg Straus)
- Microscopic polyangiitis
- Cryoglobulinemia
What should make one think this may be vasculitis?
When there is multisystem involvement
Rashes
Raised inflammatory markers - especially ESR
Abnormal urinalysis
What are the symptoms of Kawaski disease?
CRASH - Conjunctivitis - Rash - Adenopathy (lymphadenopathy) - Strawberry tongue - Hand/ feet rash - on palsm \+ - Fever
How can Henock - Scholein be differentiated from other syndromes that cause purpuric rashes such as ITP and TTP?
The platelet count will remain normal to high in Henock, where as the others cause thrombocytopenia
What is Felty’s syndrome?
Where in RA there is splenomegaly and Neutropenia
What investigations should be done in suspected vasculitis?
^ESR/ CRP
ANCA test - immunofluorescence followed by ELISA
Renal Status - creatinine, urea
Urine analysis - RBC casts, proteinuria
Angiography
Biopsy
Which vessels do giant cell vasculitis tend to affect?
Temporal
Ophthalmic
External carotid
What vasculitis is hepatitis associated with?
Polyarteritis nodosa
- associated with Hep B* major risk factor
How is polyarteritis nodosa diagnosed?
Angiography
and
Biopsy
Angiography will show the Rosary sign
- skipping of lesions causing a bead like appearance
At diagnosis of Kawasaki disease, what must be done?
Echocardiogram to assess for any damage to the heart
What is needed to diagnose granulomatosis polyangiitis?
c- ANCA levels
+
Biopsy from affected site
Which vasculitis are typically treated with steroids?
Giant cell
Takayasu
polyarteritis nodosa
What is the treatment of ANCA+ and Cryoglobulinemic type vasculitis?
Immunosuppressive medication (such as cyclophosphamide)
+
Steroids
How should Kawasaki vasculitis be treated?
IV immunoglobulins
- within the 1st 10 days
+
Aspirin
- prevent clots
What type of vasculitis is p-ANCA positive? and what are some symptoms of it?
Eosinophilic granulomatosis with polyangiitis
- chronic sinusitis
- asthma
- blood eosinophilia
What is a highly suggestive feature of Lupus?
Multisystem involvement with increased ESR but normal CRP
What disease is associated with Anti- centromere antibodies?
Systemic Sclerosis - limited (CREST) type
What are the symptoms of CREST?
Calcinosis Raynaud's Oespahgeal dysmotility Sclerodactyly Telectangasia
What is a distinguishing feature of polymyalgia rheumatica that helps distinguish it form other muscle diseases?
Normal Creatine kinase
Which drugs can be taken for RA during pregnancy?
Sulfasalazine
Hydroxychloroquine
What is the different areas of diagnostic criteria for RA?
Number of joints affected
Serology markers
Duration of symptoms
Acute reactant proteins. ESR. CRP
Scores of >6 are diagnostic
What are the symptoms of polymyalgia rheumatica?
Subacute <2 weeks pain onset
- bilateral
- should pain
- limb girdle pain
- fatigue
- weight loss
- depression
- Carpel tunnel syndrome
- temporal arteritis
How is the diagnosis of polymyalgia rheumatica made?
Clinical symptoms \+ Response to steroids \+ Ruling out other conditions \+ Rapid onset <2 weeks
- ESR >40
What advice/ additional interventions should be given to anyone on steroids?
STOP
- sick day rules - double
- Ticket - to say steroid dependent
- Osteoporosis treatment
- PPIs
What autoimmune antibodies are associated with Dermatomyositis?
Anti - Jo
What are the skin lesions typically seen with dermatomyositis?
Photosensitivity
Maculopapular rash
Gottron’s papules - on knuckles
Lilac purple rash across eyelids ** very characteristic
Dermatomyositis and Myositis may be due to what serious conditions?
Paraneoplastic effects from:
- lung
- ovarian
- pancreatic cancer
what investigations should be done into suspected dermatomyositis and how is it treated?
Muscle biopsy - diagnostic
- MRI can help identify areas of abnormal muscle for biopsy
CK levels - useful for measuring disease activity
Anti- Jo
CXR/ Spirometry
- *screening for malignancy
- CT chest/Abdo/ Pelvis
Management:
- exercise programme
- prednisolone
Azathioprine/ methotrexate 2nd line
What are the diagnostic tests into Sjogren’s syndrome?
Schirmer tear testing
- 5 mins analysis of amount of tear production
Salivary gland biopsy
Auto antibodies
- RF
- ANA
- Anti - Ro
- Anti - La
Which antibodies are associated with CREST syndrome?
Anti - Centromere
Which antibodies are associated with Diffuse systemic sclerosis?
DNA SCL-70 antibodies
What is the treatment for systemic sclerosis?
Non Medical:
- Stop smoking
- skin stretching
- avoidance of cold
- Physiotherapy
Medical
- nifedipine
- Metoclopramide - motility
- PPIs - gastric reflux
- Analgesia - joint pain
Diffuse disease:
- ACE inhibitors (kidney damage can occur)
- IV cyclophosphamide
What are the diagnostic tests for Sjogrens syndrome?
Schrimer Test
Biopsy of salivary gland
Anti Ro and Anti La antibodies
How does Takayusa present, how is it diagnosed and what is the treatment?
Claudication
Fever
Weight loss
Loss of radial pulses
Angiogram
ESR
treatment:
- High dose steroids
What are the symptoms / clinical features of temporal arteritis?
Headache
- temporal headache
- occipital region pain
Scalp tenderness
Jaw Pain
- brought on by chewing
Amaurosis Fugax
Sudden loss of vision
TIAs
What investigation should be done into temporal arteritis?
Bloods:
- FBC : anaemia, high platelets, WCC
- ESR
- LFTs - often albumin is low
X-rays:
- Ultrasound of temporal artery
- Angiography
Biopsy of temporal artery
What would you see on biopsy of the temporal artery in giant cell arteritis?
Fragmentation of the internal elastic lamina with necrosis of the media
+
Inflammatory infiltrate
What are some differentials for temporal arteritis?
PMR
Amyloidosis
Haematological malignancies
Takayasu’s arteritis
What deformity can occur in granulomatosis polyangiitis?
Saddle nose deformity
List some features found clinically on RA:
Sausage shaped fingers
Synovitis
Prominent radius head
Z-shaped deformity of thumb
*then usually stuff remember symmetry
What are some side effects of methotrexate:
How often is it given and what should be given with it?
Pancytopenia
Pneumonitis
Liver toxicity - need regular LFTs
Stomastiis
Once weekly.
Folic acid
- sub cut
What score is used to monitor disease progress in RA?
Disease Activity Score of 28 joints - DAS28
- joints
- ESR
- General health
<0.6 is poor prognosis
*ultrasound is also usually used
What are some features of poor prognosis indicators in RA?
Multiple joint involvement HLA DR4 positive RF positive Early -x-ray changes Extra-articular features
What investigations do you want to do into Lupus?
Bloods:
- FBC - leukopenia/ thrombocytopenia
- ESR
- U&Es - renal nephritis
- Complement studies - Low C3, C$
- ANA, ds DNA
Orifices:
- Urine analysis
X-rays:
- CXR - pleural effusion
- Joint x-rays - non erosive
ECG
- pericarditis
Special tests:
What is it called when lupus affects the kidneys and how is confirmed?
Lupus nephritis
Renal biopsy
How can infection and Lupus be differentiated?
CRP levels
What are some complications that can occur in birth with lupus?
Miscarriage - PLS
Pre-eclampsia
Worsening of symptoms
Preterm
Neonatal lupus
- transcient discoid rash
What is the diagnostic criteria for RA?
Joints affected
Serology
- Rh Factor
- Anti - CCP
Duration of symptoms
>6 weeks
Acute phase reactant proteins
- ESR
- CRP
What surgical operations may be indicated for RA?
Synovectomy
- finger tendon sheath to reduce contractures
Joint arthroplasty
What is psoriatic arthritis associated with?
nail changes
- Pitting nails
- Onycholysis
- Subungual Hyperkeratosis
- Onychauxis
- colour changes
Synovitis
palmo-pustular changes
Metabolic syndrome
What are the different types of Psoriatic arthritis and how do they present?
Asymmetrical Oligoarthritis
- synovitis
- enthesitis
- dactylitis
Symmetrical Polyarthritis
- symptoms of RA without extra articular
DIPJ arthritis
- Periarticular swelling
- Nail dystrophy
AS arthritis
- resembles AS
Mutlins
- Telescoping of the digits
- shortening and destruction
- opera glass hand
What is the diagnostic criteria and disease monitoring scoring system used for psoriatic arthritis?
Diagnostic criteria: CASPAR
- evidence of psoriasis / family history
- dactylitis / of previously
- Negative Rh
- Radiological keeping
- Nail changes
Disease monitoring: DAPSA-64
- >28 is severe
Which bones are most likely to be affected in paget’s disease?
Skull, spine, pelvic bone
Where does a malar rash spare?
Nasal labia folds
What are the specific antibodies towards lupus?
dsDNA
Anti-smith
What gene is linked to anti-phospholipid syndrome?
HLA DR7
What investigations should be done into lupus?
Bloods:
- FBC - anaemia
- U&Es - kidney involvement
- ESR
- Complement studies - C3, C4 will be low
- Ant- ANA
- Anti - dsDNA
- Anti Smith
Orifices:
- Urine analysis - protein, blood (lupus nephritis)
X-rays:
- Joint’s
ECG:
- pericarditis
Special tests:
- Kidney biopsy
What is the general management of Lupus and what is the scoring system used?
SELENA SLEDIA - scoring system used
Avoid sun exposure
Mild:
- NSAIDS
- hydroxychloroquine
Moderate:
- Hydroxychloroquine
- Steroids
- Belimumab
Severe:
- Cyclophosphamide
- Plasmapheresis
Differentials for Lupus?
RA
Phospholipid syndrome
Systemic Sclerosis
What is the medical management of SA?
1st line:
- NSAIDs
- Steroid injection
2nd line:
- Infliximab
- Etanercept
3rd line:
- Secukinumab = IL-17 Inhibitor
Cardiovascular management
What are the manifestations of Reactive arthritis?
Asymmetrical arthritis
- usually follows a few weeks after infection
Sterile conjunctivitis or anterior uveitis
Urethritis
Dactylitis
Enthesitis
Skin:
- Keratoderma Blennorrhagica
**usually develops within 4 weeks of the infection
What are the risk factors for systemic scleroderma?
Bleomycin
Vinyl chloride
Female - 30 -50 years old
What are the features of limited scleroderma?
Calcinosis
Raynaud’s
Esophageal dysmotility
Sclerodactyly - intense tight fibrosis tissue around hands
Telectangasia
What are the symptoms of diffuse scleroderma?
edematous onset followed by raynaud’s.
Extensive skin thickening
- Heart burn
- Malabsorption for G.I track
- Acute hypertensive renal crisis
- Lung fibrosis
- Myocardial fibrosis
What are the specific antibodies related to scleroderma?
CREST syndrome: anti- centromere
Diffuse:
Anti - SCL-70
What investigations should be done into Scleroderma?
Bloods:
- FBC
- U&Es - kidney involvement
- Antibodies
- Rh factor
Orifices:
- urine microscopy
- Protein: creatinine ratio
X-rays:
- High resolution CT for fibrosis of lung
- Barium swallow
ECG:
- Heart block/ fibrosis
What is the classical onset of polymyalgia rheumatica?
50 year old woman, sudden onset pain and stiffness in: - neck - shoulders - pelvis last for >30 mins
What is Behcet’s disease, where is it common, what is the genetic factor and what is diagnostic test?
Multisystem inflammatory condition characterised by:
- Oral ulcers
- genital ulcers
- also affects many other organs:
- Iran
- Japan
- Turkey
HLA B51
Pathergy test.
What would you see on a FBC of someone with phospholipid syndrome?
Thrombocytosis
What is the recommended treatment for fibromyalgia?
CBT
What are some common drugs that cause lupus?
Procainamide
hydralazine
Isoniazid
What will the optic disc look like in temporal arteritis?
Pale and oedematous
What antibodies are associated with drug induced lupus?
Anti-histone body
What are the risk factors for lupus?
Family history
Genetics
- HLA DR3
Sex hormone status
Drugs
- hydralazine
- Isoniazid
- Procainamide
- Penicillamine
What are some skin manifestations of lupus?
Malar rash
Discoid rash
Livedo Reticularis
Alopecia
What defects can occur in the heart due to lupus?
Pericarditis
Aortic valve lesions
Non - infective endocarditis
What will the FBC results of lupus show?
Anaemia - anemia of chronic disease + Haemolytic anaemia
Leukopenia
Lymphopenia
Thrombocytopenia
Type II hypersensitivity reaction can destroy the blood cells
What is another name for Scl - 70?
Topoisomerase - 1
Name two tests to look for antiphospholipid syndrome:
Anticardiolipin test
- detects IgG that bind to negatively charged phospholipids
Lupus anticoagulant test
- despite being called lupus it is for APL
What is the core triad of lupus?
Fever
Arthralgia
Rash
What is the key radiological featured needed on x-ray to confirm a diagnosis of AS?
Sacroiliitis
Patient is on warfarin and develops gout, what is 1st line medication?
Colchicine
**NSAIDS contraindicated with warfarin
What is the histological changes seen in RA?
Increased villous hyperplasia
Synovial thickness
Lymphocyte and plasma cell infiltration
Name two creams used in psoriasis which have vitamin D analogues in them:
Dovobet
Enstilar
How is Psoriatic arthritis differentiated from RA?
Absence of Anti - CCP
Dactylitis
What are the radiological findings of psoriatic arthritis?
Pencil in Cup deformity
Dactylitis
Fluffy periostitis
Distal joint erosion
What is the treatment for psoriatic arthritis?
1st line:
NSAIDs
Physiotherapy
2nd Line:
- DMARDS
3rd:
- Biologics
- Infliximab
- Apremilast
What findings may be present in psoriatic arthritis?
Dactylitis Reduced ROM Painful Deformity Sacroiliac involvement Psoriasis Nail changes Predominantly distal involvement
What x-ray features are seen with psoriatic arthritis?
Fluffy periostitis
Dactylitis
Distal phalangeal erosion
Pencil in cup deformity
What are the symptoms of AS?
Sacroiliac pain
Morning stiffness
Breathlessness
- apical pulmonary fibrosis
Reduced exercise capacity
- aortic regurgitation
Blurring of vision
- anterior uveitis
Plantar fasciitis
Weight loss
Fever
What are some clinical findings of AS?
Schober’s test positive
- reduced lumbar movement when bending forward
Enthesitis
Diastolic murmur heard left sternal edge
- aortic regurgitation
Hyperextension of the neck
- compensatory mechanism
Loss of lumbar lordosis
Kyphosis
What is the diagnostic criteria for AS?
<50 years
Worse on rest
Improvement with movement
Insidious onset
What is a common bursitis found with polymyalgia rheumatica?
Trochanteric Bursitis
- diagnosed via US
What are the differentials for polymyalgia rheumatica?
Polymyositis
SLE
Fibromyalgia
Hypothyroidism
RA
How is polymyalgia monitored?
ESR and CRP levels
In the treatment of Polymyalgia, what occurs at 4 weeks?
Reassessment
- and started on reducing regime
What investigations are wanted into giant cell arteritis?
FBC ESR CRP CK level Rh factors
Ultrasound of temporal with biopsy
Fundoscopy
What is an adverse effect of hydroxychloroquine?
Retinopathy
Which drug is sulfasalazine similar to and as such if there is a allergic reaction should also be avoided?
Aspirin
Mesalazine
Both are salicylic acid based
