Rheumatology Flashcards
X-ray findings of rheumatoid arthritis
Loss of joint space, periarticular osteoporosis, swelling
Late: periarticular erosions, subluxation
Associations with myotonic dystrophy
Distal weakness initially
Diabetes
auto Dominant
Dyarthria
Features of myotonic dystrophy
20-30yrs... Myotonic facies "haggard" Distal weakness Frontal balding Bilateral ptosis Dysarthria Mild mental impairment Testicular atrophy Heart block, cardiomyopathy
Diagnosis of vitamin d resistant rickets
Increased urine phosphate, in context of normal calcium and cupped metaphyses on XR
X linked dominant
Replace vit d
Symmetrical proximal muscle weakness, Reynauds, no rash diagnosis
Polymyositis
(Anti Jo)
Rash indicated dermatomyositis
Investigations for proximal symmetrical muscle weakness with Reynauds and no rash
Polymyositis
Elevated CK, and other elevated muscle enzymes (LD, AST, ALT) Anti Jo1 (seen in pattern of disease with lung involvement)
What does CREST stand for
Calcinosis
Reynauds
Eosophageal dysmotility and malabsorption
Sclerodactyly
Telangiectasia (small dilated blood vessels
Features of myasthenia gravis
Diplopia
Muscle fatigue
Ptosis
Dysphagia
Management of myasthenia gravis
Long acting anticholesterase inhibitor, pyridostigmine
Immunosuppression with steroids
Thymectomy
Lambert Eaton syndrome management
Treat underlying cancer!
Immunosuppression with pred, aza
Genetic link with multiple sclerosis
HLA DR2…
Monozygotic twin concordance of 30%
Dizygotic 2%
Environmental factors of multiple sclerosis
Higher latitudes
Neurological triad of signs for MS
Charcot’s triad - Dysarthria, nystagmus, intention tremor
Two specific signs for MS
L’hermittes sign -electric shock down spine when head forwards
Urthoffs phenomenon - worse vision when body temp increases
Associations with pANCA and cANCA
pANCA granulomatosis with polyangitis (wegeners)
cANCA churg Strauss syndrome
pANCA also associated with IBS (UC>C), Connections tissue disease and autoimmune hepatitis
Which immunodeficiency predisposes to neisseria meningitidis
C5-9 deficiency
What antibody is suggestive of RA
Anti cyclic citrullinated peptide antibody can be detectable 10 years before! Sensitivity of 70 (similar to RF) but specificity of 90-95%
Investigations for proximal symmetrical muscle weakness with vesicular rash on back and shoulders and heliotrope rash around periorbital region
Dermatomyositis
ANA positive, and 25% anti Mi-2 positive
Immunoglobulins reaction of Rheumatoid Factor
Rheumatoid factor is an IgM antibody against IgG
Autoantibody associated with dermatomyositis
ANA
Autoantibody associated with rheumatoid arthritis
Anti CCP (cyclic citrullinated peptide) antibody is most specific (90%)
also a poor prognostic factor
may be detectable up to 10 years before the development
Autoantibody associated with SLE
Anti dsDNA antibodies…non-specific symptoms such as, fatigue, fever, oral ulcers, joint pain and a rash
Autoantibody associated with diffuse systemic sclerosis
Anti scl 70
Autoantibody associated with primary biliary cirrhosis
AMA…
Often asymp but fatigue, pruritus and jaundice
IgM
Features of adult Stills disease
Bimodal presentation 15-25 35-45 Arthralgia elevated ferritin Salmon pink maculopapular rash LN fever RF and ANA neg
Treat with methotrexate then Anakinra (competitively inhibits the action of IL-1)
Management of myasthenic crisis
Plasmapheresis or IV immunoglobulin
Plasma is quicker but more expensive equipment
Most common target of pANCA
MPO
Myeloperoxidase
Most common target of pANCA
MPO
Myeloperoxidase
Most common target of cANCA
PR3
Proteinase 3
What antibody is usually found in Myasthenia gravis
(nicotinic) Acetylcholinesterase receptor antibodies in 90%
Patient with MS, on Natalizumab, acutely confused, with hemiparesis
That drug can reactivate JC virus causing progressive multifocal leukoencephalopathy PML
Compliment deficiency that predisposes to SLE
Deficiency in C1q, C1rs, C2 and C4 predisposes to immune complex disease such as SLE
Patient with dermatomyositis… What is most important first investigation?
Exclude malignancy.
~25% diagnosed with dermatomyositis have an underlying malignancy (typically ovarian, breast and lung cancer), this prevalence increases in the elderly.
Poor prognostic factors related to RA
Rh F positive
Anti CCP autoantibodies
HLA DR4
Early erosions and extra articular features like nodules
How does Rheumatoid factor correspond with severity / progression in RA
High levels of Rh F are assoicated with severe progressive disease, but are not a marker of disease progression
What type of HLA allele is most associated with Rheumatoid arthritis?
HLA DR4
RA - R4
Myalgia and fatigue, mononeuritis, glomerulonephritis… Diagnosis and antibody
Microscopic polyangiitis
pANCA
Most common complications of diffuse cutaneous systemic sclerosis
Most common cause of death is resp (80%)
ILD and pulmonary arterial hypertension
HLA memory aid
SjogR3n's DR3 RA DR4 (also T1DM) hA3mochomatosis A3 B5hcet's disease B51 CeiliaQ DQ2/DQ8 naRcolepsy / goodpastuRes DR2 Reiters ... 27yr sleeping around b27
B27 also ank spond (presents agreed 20-30)
And acute anterior uveitis because that’s also assx
Features of Adult onset Stills disease
Arthralgia, fever, salmon Mac pap rash, lymphadenopathy
RF and ANA negative
Management of Reynaud’s….
Secondary care
First line CCB like nifedipine
Second line IV prostacycline like epoprostenol
What type of collagen is primarily affected by Ehlers Danlos Syndrome?
Collagen type 3
3hlers-danlos
SLE - antibodies associated with congenital heart block
Anti Ro
Road…block
Poor prognostic features of RA
RF +ve Anti CCP antibodies Slow onset HLA DR4 XR early erosions Extra articular features like nodules
Possibly female
Anti-GQ1b antibodies
Present in 90% of Miller Fischer syndrome
Drugs that exacerbate MG
Beta blockers
Gentamicin
Lithium
Phenytoin
SLE - antibodies associated with congenital heart block
Anti Ro
Ro ro ro your boat
Bony lump at DIP
Heberden’s OA
Bony lumps at PIP
bouchards RA
wegners / granulomatosis with polyangiitis features
Ent features like sinusitis
Goodpastures features
Pulmonary haemorrhage
anti-smooth muscle antibodies (SMA)
Type 1 autoimmune hepatitis
Treat with steroids, / immunosup e.g. azathioprine
liver transplantation
CAN get smooth muscle ab in primary biliary cholangitis but more assx with AMA and IgM
RA scleritis Vs episcleritis
Scleritis is Sore
ePiscLEritis is PainLess
Which immunoglobulin are most common?
GAMDE
Management of SLE
Hydroxychloroquine
Management of MG
pyridostigmine
Diagnosis of ankylosing spondylitis
x-ray of the sacroiliac joints - look for sclerosis,or subchondral cysts
Or MRI if still unclear
Looking for inflam of sacroiliac joints (bone marrow oedema)
Which antibiotics should you avoid when on methotrexate
Trimethoprim and co-trimoxazole
They all work by inhibiting the enzyme dihydrofolate reductase, so can cause myelosuppression
Which cytokine is most important in pathophys of RA
Tumour necrosis factor
Related cramps and myoglobinuria after exercise
McArdle’s disease
Auto R type V glycogen storage disease
Etanercept TNF-alpha adverse effect
Reactivation of TB
Symps and antibodies of mixed connective tissue disease
Anti RNP
Reynaud’s
N - no synovitis but swollen hands
Pain in muscles and joints
mixed connective tissue disease prognosis
1/3 long-term remission
Chronic symps
Develop severe systemic involvement and premature death
What feature is isn’t commonly seen in drug induced lupus
Glomerulonephritis
Medium vessel vasculitis assx w hep B
Polyarteritis nodosa
No Autoantibodies likely to be positive
Fever malaise foot drop, livedo reticularis , background of hep b
Polyarteritis nodosa
No antibody likely positive, raised ESR
Clotting bloods of patient with anti phospholipid syndrome
Paradoxically, prolonged APTT
Clotting bloods of patient with anti phospholipid syndrome
Paradoxically, prolonged APTT
Low platelets
Cubital tunnel syndrome
cUbital tUnnel syndrome - Ulnar nerve - fUnny bone. Tingling in 4th and 5th
NO pain or tenderness over medial epicondyle (that’s medial epicondylitis)
Lower back pain - prolapsed disc L4
on alL 4s - sensory loss anterior of knee
And reduced knee reflex (seen in L3 and l4)
Lower back pain - prolapsed disc L3
Sensory loss over anterior thigh
Reduced knee reflex (seen in L3 and l4)
Polymyositis and dermatomyositis Autoantibodies
Jo and ANA
