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
Scleroderma
- Tight skin
- Plaques
Antibodies in systemic sclerosis
ANA - 90%
RF - 30%
anti-centromere - Limited cutaneous
anti-Scl-70 - Diffuse cutaneous
ank spond tx
1st line: Ex regime + NSAIDS
2nf - anti-TNF - Etanacerpt
septic Arthritis
Kocher Criteria: Fever >38.5 Non WB Raised ESR Raised WCC
common causes - Staph A + N.gonnorhea (young adults)
Mx:
- Aspirrate
- IV Fluclox or Clinda (pen allergy) - 2-6/52
What medication should be avoided with MTX
Trimethoprim
Combo –> BM failure
minimum steroid use for Osteoperosis mx
equivalant of pred >/= 7.5mg for 3/12
Mx of steroid induced osteoperosis:
1st line allendronate
> 65 or prev fragilty fracture –> Protection
<65 - Then they need T score:
- >0 - nothign
- 0 to -1.5 - rpt scan 1-3yrs
-
Osteoperosis - inx for secondary causes
Hx + physical
FBC/LFT/ Alb / Cr/ Ca/ ESR/ PO4-/TFT
DEXA
Osteoperosis DEXA scan
T score:
- Based on bone mass of young reference oplation
- > -1.0 = Normal
- 1.0 to - 2.5 = osteopaenia
Osteoperosis Mx - post menopausal women w/ osteoperotic #
Tx if postmenopausal + osteoperotic # + T score 75 yrs –> dnt need dexa
Offer Vit D + Ca to all - unless levels normal
1) Alendronate
2) if GI problems tjem Risedronate or etidronate
3) Can’t tolerate any bisphosphonate –> Strontium ranelate and raloxifene. (base on strict T score)
Osteoperosis medication - notes
Bisphosphonates:
- reduce risk of #
- alendronate + Risedronate > etidronate ant reducing # risk
Raloxifene:
- Selective oestrogen receptor modulator
- prevents bone loss + reduce vertebral # risk.
- worsen menopausal sx
- VTE risk !!!!!!
- decrease risk of breast Ca !!!!!
Strontium:
- increase osteoblast abd recrease osteoclast
- Specialisst onlu
- Risk of CVD!!!
- DO NOT USE IF VTE Hx
- Steven Johnson syndrome risk
Denosumab
- MAb –> inhibits RANK ligand –> inhibit maturation ofosteoclast
- S/C 6/12
Hip protectors
- good in NH
Falls risk assessment
- consider in high risk
- no strict evidence of reduced # rate.
Hypomagnesaemuoa
Causes:
- Drugs: diuretics + PPIs
- TPN
- diarrhoea
- EtOH
- Hypokalaemoa/hypocalcaemia
- Ciarrhoea causing conditions - Crohns/UC
- Gitellmans/Barterrs
Ft:
- Similair to hypocalcarima
- Causes decreased PTH –> hypoCa
- ECG similar to hypokalaemia
- Prolonged QT
- Exacerbate dig toxicity
Mx - <0.4:
- IV MgSo40 - 40mmol / 24hr
Mx - >0.4:
- PO mg = 10-20 mmol/day
- Can cause diarrhoea
hypermagnesaemia
Sc:
- lethargy
- drowsy
- paralysis
- low BP
- Heart bock –> cardiac arrest
Causes: - CKD - Adrenal insufficiency - Mg infusion - milk alkali - I Enemas - Tumour lysis syndrome - Theophyllin intoxication - Rhabdomyolysis
Hypophosphataemia
CauseS:
- EtOH
- liver fx
- hyperPTH
- DKA
- Refeeding
- Osteomalcoa
ConsequenceS: - Haemaolysis - WCC / platelet dysfn -Muscle wakness / Rhabdomyolysis (<0.3) CNS dysfn -
Hyperphosphataemia
Causes:
- CKD
- Rhabdomyolysis
- Tumour lysis
- lactic and ketoacidosis
- Exogenous
- Vit D intoxication
- low PTH
- pseudohypoparathyroidism
- Acromegaly
- Thyrotoxicosis
- Bisphosphonates
Mx - Acute :
- Saline infusion + Loop diuretic
Mx - CKD
- low PO4- diet
- PO4- nbinders
- Dialysis.
Obesity - BMI
Healthy 18.5 - 24.9 Overweight - 25 - 29.9 Obesity 1: 30 - 34.9 Obesity II: 35 - 39.9 Obesity 3; >40
Criteria for bariatric surgery
- BMI > 40
BMI 35 - 40 + other sig dx that could be improved with WL. - all appprop non-surgical therapies have been tried and failed for 6/12
- intensive speciailist mx available/accepted
- Fit for anaesthetic
can give as first line under special=st conditions if BMI >50
Severe malnutrition
Marasmus
- deficiency of both protein + calaories
- growth fx more sever e in marasmus
Kewashikor:
- Protein deficiency - I.e. diet of solely carb
Oedema:
- Kwashikor +ve
- Masrasmus -ve
Anion gap
= (Na + K) - (HCO3- + Cl-)
Normal anion gap = 8-14
Metabolic acidosis + Normal Anion agap:
- GI HCO3- loss
- RTA
- Acetozolamide
- AlCl injection - (Cl - replaces HCO3-)
- Addisons
High anion gap acidosis:
- Lactic acidosis (lost HCO3-)
- ketoacidosis (as above)
- AKI and advanced CKD
- LFx
- Toluene ingestion
- Intoxication - methanol, ASA, ethylene glycol
Hypoalbuminaemia and the anion gap
Low albumin does contribute to Anion gap (but is not measured)
Every 1g of albumin decrease –> fall of 2.5 –> 3 mmoles in AG
therefore in a high anion gap acidosis with hypoalabuminaemia –> normal AG
Particular issure in ICU
A lactic acidosis in Ia low olb ICU pt –> normal anion gap.
Metabolic acidosis spec causes
MA with diarrhoea
- GI contents mostly alkaline + high K_
- Hypokalaemia
- low urinary K_
- Low pH
RTA - covered prev
MA wiith ureteric diversion or ileal loop diversion:
- Hyperchloraemix
- Urinary CL- exchange for HCO3-
Metabollic alkalosis
GI hydrogen loss:
- Vomittin/pyloric stenosis
- NG suction
- Antacids.
Intracellular shift of H+
- hypokalaemia
Alkali administratuin
Renal hydrogen loss:
- Cushings
- loop/thiazide diuretic
- Post -hypercapnic alkalosis.
- hyperCa + milk-alkali syndrome
Volume depletion
Spec metabolic alkalosis
Gastic H+ loss:
- Meatb alkalosis
- but aslo get acidic urine - NaHCO3- is reabsrorbed to maintain plasma Vol.
Milk-alkali syndrome:
- Triad of hyperCa + metab alkalosis + inhestion of large amounts of Ca w/ absorbable alkali.
- Hyper Ca –> increased HCO3- reavs –
> worsones alkalosis.
Post - hypercapnic alkalosis:
- Chronic resp acidosis –> compensation via increase urinary H+ excretion –> Raisd HCO30-.
- Rapid lowering of PCO2 (mechanic ventilation) –> fall in HCO3- + CL-.
Hypothermia
Acute (immersive)
Subacute (Exhaustion) hypothermia - unable to generate heat.
Chronic - gradual loss - elderly with inadequate heating
ECG:
- J waves
- PR prolonged
- QT prolonged.
- Arrythmias
Mx:
= >30 –> Surface rewarming
<30 00<> internal warming:
- warmed IVI
- Warm bladdrer washout
- Warm humidified O2:
- Peritoneal lavage
- pleural lavage.
- VF or profound deterioration –> ECMO
Cardiac arrest:
- not dead till warm and dead
- decreased metabolism of drugs - trherefore avoid IV drugs until >30.
- hypothermia protects brain during arrest –> therefore can have full neuyro recovery despite prolonged arrest.
Gout
US of joint:
- Effusion
- DOuble contorur sign = deposition of urate crystal on cartilage surface
Xray:
- Well defined punched out erosions with sclerotic margins juxta-artixular
- joint space preserved
- Soft tissue tophi
RF for Carpal tunnel syndrome
MEDIAN TRAP
myxoedema edema db idiopathic acromegaly Neoplasm
Trauma
RA
Amyloiid
Preg / Obesity
Adult onset stills dx
Ft:
- Arthralgia
- Elevated ferritin
- SALMON PINK RASH
- Pyrexia - late afternoon/early evening.
- RF & AN-
Yamaguchi criteria.
Mx:
1) NSAIDs
2) Steroids
3) If sx persist –> MTX/ IL-1 or anti-TNF
Drug induced lupus
arthralagia
myalgia
plurisy
malar rash
DO NOT GET GLOMERULONEPHRITIS
Which markers used in dx monitoring in SLE
anti-dsDNA
C3/C4
ESR
Bisphosphnate side effects
osteonecrosis of jaw
Oesopphagitis/ulcers
allendronate: - atypical # of femoral shaft
Osteomalacia
Normal bony tissue but less dense
IF growing then it’s called Rickets.
Low Vit D
High ALP
Low Ca/PO4-
Xray - Cupped metaohysis.
- Adults = loosers zones = translucent bands
MX:
- Vit D + Ca
Osteopetrosis
Marble bone disease.
Fx in Osteoclast fn –> reduced resorption of bone – VVV.thick/brittle bone –> prone to fracture
Ca/PO4-/ALP all normal
Poor prognostic factors for RA
RF positive Anti-CCP present Insidious onsen Poor functional capacity at presentation Xray features - Early erosions exra-articular features - nodules
Relapsing Polychondritis
repeated inflammation of cartillage. Therefore affects anywhere with cartilage:
Nose –> Saddle nose
Ears –> Cauliflower ears
Airways –> Resp symptoms
Mx:
Remission –> Steroids
Maintenance –> MTX/AZA (Steroid sparing)
MAnagemen of adult onset stills
NSAIDs 1st line
if fx –> TNF inhib or Il-1 inhib (Anakinra)
Leflunomide
Used in RA
NOT SAFE in preg –> stop 2 years before
Ons topping –> Long washout period –> may require co-admi n of cholecystyramine
Monitor:
- FBC/LFT/BP
S/E:
- Diarrhoea
- HTN
- WL
- Pneumonitis
- myelosupression
- PEriph neuropathy
Newly diagnosed RA - what Mx to slow dx progression
MTX + one other DMARD + steroid
SLE what complement is low
C3 + C4
Initial Mx of BEchets
Prednisolone
Lupus 1st line MX of choice
Hydroxyxhloroquine
then after –> steroids –> Fx —> Belimumab
antiGBM vs vasculitits
anti-GB - normal ESR
OTher vasculitis - raise dESR
