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