Rheumatology Flashcards
Langerhan histiocytosis
Abnormal prolif of histiocytes
Px - in early childhood —> LYTIC bone lesions
Ft:
- bone pain
- cutaneous nodules
- recurrent otitis media/mastoiditis
- tennis racquet shaped birbeck granules on electron microscopy
Osteoporosis risk factors:
SHATTERED Family
Steroids >5mg/day
Hypercalciuria, hyperthyroidism hyperparathyroidism
Alcohol and tobacco
Thin bmi < 22
Testosterone low
Early menopause
Renal or liver fx
Erosive Ibd Or RA
dietary ca low/malabsorption
Fhx
Osteoporosis - meds that worsen
Steroids
SSRI
PPI
Glitzines
LT heparin
Aroma tase inhibitor
Osteoporosis management
Treatment indicated if:
Post menopausal woman with DEXA (T =< -2.5 S.D) (Don’t have to do if >75) &Sx)
vit D and calc supplementation
ALendronic Acid = 1st line
If GI sx—> risedronate or etidronate
If can’t tolerate bisphosphonates
(T < -3.5)
-strontium Ranelate = increase osteoblasts and reduce osteoclast
- raloxifene = selective oestrogen receptor modulator
- denosumab - mab inhibits RANK ligand which inhibits maturation of osteoclasts
RA in preg
Disease can improve in preg then most flare after delivery
NSAIDs up to 32 weeks - after which causes early PDA CLOSURE
MTX not safe - stop 6/12 prior
Leflunomide not safe
Hydroxychllroquine and sulfasalazine are safe
Refer to
Obstetric anaesthetics —> risk of Atlanto-axial dislocation
Osteoporosis if at risk of
Steroid induced
T-score 0 - nothing
T- score 0 —> -1.5: rpt DEXA 1-3yr
T score
Mcardle disease
AR glycogen storage disease.
Distal muscle cramping on use wirh a second wind.
distinguish between myositis and Mcardles as McArdles has second wind and myositis will have 100x ULN of CK
SLE antibody assoc with congenital heart block
Anti-Ro
Paget’s disease
Old man with bone pain and Normal Ca and nomral PO4- with raised ALP
Classical ft if untx:
- Bowing of tibia
- Bossing of skull.
it is due to high osteoclast activity.
RF:
- Inc ages
- male sex
- nothern latitude
- fhx
Urine Hydroxyproline
Indications for tx - bone pain, skull or long bone deformit, periarticular Paget’s:
- Bisphosphonat - PO risedronate or IV zoledronate
Complications: -Deafness bone sarcoma fractures skull thickening high output cardic failure.
Adult onset stills disease
Triad:
- fever
- joint pain
- salmon rash
Arthralgia Salmon pink rash Pyrexia High ferritin LN
RF negative
Mx:
NSAID - try for one week
Fx —> steroids —> fx—-> MTX/IL1/ anti TNF
Osteogenesis imperfects
Brittle bone disease
A.D
Collagen type 1
Px in childhood m # following minor trauma Blue sclera Deafness Dental imperfections
Osteoporosis dexa scan
> -1 = normal
-1 —> -2.5 = osteopaenia
< -2.5 = osteoporosis
Anyiphospholipid sundrome
Paradoxical prolonged APTT and low platelets
Thrombocytopenia
Recurrent thrombosis
Recurrent foetal loss
Liverdo reticularis
Pre eclampsia
Pulm htn
Assoc SLE
Mx
- warfarin for 6/12 aim 2-3
Recurrent - lifelong warfarin
If event whilst on warfarin - lifelong and aim 3-4
Arterial thrombus = warfarin lifelong 2-3
Ankylosing spondylitis features:
The A’s
Ant uveitis Aortic regurge Apical fibrosis AV block Achillis tendonitis Amyloidosis
Ankylosing spndylitis
- most useful radiological inx
X~Ray lumbar spine most useful for diagnosis and monitorin
Reactive arthritis - joint aspirate
CULTURE NEGATIVE
Occular manifestations of RA
KEratoconjunctiva sicca - most common - sore gritty eyes
scleriti
episcleritis
Keratitis
corneal ulcer
Stronges RF of thrombus in anti-phospholipid
Lupus anticoagulant
CREST with dysphagia
most common complication is malabsorption due to a sclerosed small bowel leading to bacterial overgrowth
Pagets disease
Bone pain + uni lat Hearliung loss + isolated high ALP
Tx: Bisphosphonates
Dermatomyositis
Photosensitive, muscle pain + Weakness, heliotrope rash. Gottrenns papuoles.
ANA
Anti-Jo1
Ant-mi-2 - most specific
SCREEN ALL FOR MALIGNNACY
Behcets sydrrome
Ant uveitis Gental ulcers ORal ulcers Aseptic meningitis DVT
Drug induced lupus
Arthralagia
myalgia
plurisy
malar rash
ANA +
ant-dsDNA - neg
Anti-histone +
Causes:
Procanamide
hydralazine
isoniazid
phenytoin
Penicillamine
Z score
Useful in diagnosing secondary osteoperosis #
- always use in children, young adults, pre-menopausal women and men <50
MTX
Dihydrofolate reductase inhibitor
limtied cutaneous systemic sclerosis
- anti-centromere
- Face + Distal limbs
- CREST
- LT complication - Pulm HTN
diffuse cutaneous systemic sclerosis
- ant-Scl-70
- Trunk+ prox limb
- LUNG INVOLVEMENT - 80% ILD nd PAH
- Renal dx + HTN