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Rheumatology Flashcards

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1
Q

Langerhan histiocytosis

A

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
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2
Q

Osteoporosis risk factors:

SHATTERED Family

A

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

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3
Q

Osteoporosis - meds that worsen

A

Steroids

SSRI

PPI

Glitzines

LT heparin

Aroma tase inhibitor

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4
Q

Osteoporosis management

A

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

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5
Q

RA in preg

A

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

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6
Q

Osteoporosis if at risk of

Steroid induced

A

T-score 0 - nothing

T- score 0 —> -1.5: rpt DEXA 1-3yr

T score

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7
Q

Mcardle disease

A

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

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8
Q

SLE antibody assoc with congenital heart block

A

Anti-Ro

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9
Q

Paget’s disease

A

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.
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10
Q

Adult onset stills disease

A

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

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11
Q

Osteogenesis imperfects

A

Brittle bone disease

A.D
Collagen type 1

Px in childhood m
# following minor trauma 
Blue sclera 
Deafness 
Dental imperfections
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12
Q

Osteoporosis dexa scan

A

> -1 = normal

-1 —> -2.5 = osteopaenia

< -2.5 = osteoporosis

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13
Q

Anyiphospholipid sundrome

A

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

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14
Q

Ankylosing spondylitis features:

The A’s

A 
Ant uveitis 
Aortic  regurge 
Apical fibrosis 
AV block 
Achillis tendonitis 
Amyloidosis
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15
Q

Ankylosing spndylitis

  • most useful radiological inx
A

X~Ray lumbar spine most useful for diagnosis and monitorin

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16
Q

Reactive arthritis - joint aspirate

A

CULTURE NEGATIVE

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17
Q

Occular manifestations of RA

A

KEratoconjunctiva sicca - most common - sore gritty eyes

scleriti

episcleritis

Keratitis

corneal ulcer

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18
Q

Stronges RF of thrombus in anti-phospholipid

A

Lupus anticoagulant

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19
Q

CREST with dysphagia

A

most common complication is malabsorption due to a sclerosed small bowel leading to bacterial overgrowth

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20
Q

Pagets disease

A

Bone pain + uni lat Hearliung loss + isolated high ALP

Tx: Bisphosphonates

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21
Q

Dermatomyositis

A

Photosensitive, muscle pain + Weakness, heliotrope rash. Gottrenns papuoles.

ANA
Anti-Jo1
Ant-mi-2 - most specific

SCREEN ALL FOR MALIGNNACY

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22
Q

Behcets sydrrome

A 
Ant uveitis 
Gental ulcers 
ORal ulcers 
Aseptic meningitis 
DVT
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23
Q

Drug induced lupus

A

Arthralagia
myalgia
plurisy
malar rash

ANA +
ant-dsDNA - neg
Anti-histone +

Causes:
Procanamide
hydralazine

isoniazid
phenytoin
Penicillamine

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24
Q

Z score

A

Useful in diagnosing secondary osteoperosis #

  • always use in children, young adults, pre-menopausal women and men <50
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25
MTX
Dihydrofolate reductase inhibitor
26
limtied cutaneous systemic sclerosis
- anti-centromere - Face + Distal limbs - CREST - LT complication - Pulm HTN
27
diffuse cutaneous systemic sclerosis
- ant-Scl-70 - Trunk+ prox limb - LUNG INVOLVEMENT - 80% ILD nd PAH - Renal dx + HTN
28
Scleroderma
- Tight skin | - Plaques
29
Antibodies in systemic sclerosis
ANA - 90% RF - 30% anti-centromere - Limited cutaneous anti-Scl-70 - Diffuse cutaneous
30
ank spond tx
1st line: Ex regime + NSAIDS 2nf - anti-TNF - Etanacerpt
31
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
32
What medication should be avoided with MTX
Trimethoprim Combo --> BM failure
33
minimum steroid use for Osteoperosis mx
equivalant of pred >/= 7.5mg for 3/12
34
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 -
35
Osteoperosis - inx for secondary causes
Hx + physical FBC/LFT/ Alb / Cr/ Ca/ ESR/ PO4-/TFT DEXA
36
Osteoperosis DEXA scan
T score: - Based on bone mass of young reference oplation - >-1.0 = Normal - 1.0 to - 2.5 = osteopaenia
37
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)
38
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.
39
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
40
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 ```
41
Hypophosphataemia
CauseS: - EtOH - liver fx - hyperPTH - DKA - Refeeding - Osteomalcoa ``` ConsequenceS: - Haemaolysis - WCC / platelet dysfn -Muscle wakness / Rhabdomyolysis (<0.3) CNS dysfn - ```
42
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.
43
Obesity - BMI
``` Healthy 18.5 - 24.9 Overweight - 25 - 29.9 Obesity 1: 30 - 34.9 Obesity II: 35 - 39.9 Obesity 3; >40 ```
44
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
45
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
46
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
47
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.
48
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-
49
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
50
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-.
51
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.
52
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
53
RF for Carpal tunnel syndrome MEDIAN TRAP
``` myxoedema edema db idiopathic acromegaly Neoplasm ``` Trauma RA Amyloiid Preg / Obesity
54
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
55
Drug induced lupus
arthralagia myalgia plurisy malar rash DO NOT GET GLOMERULONEPHRITIS
56
Which markers used in dx monitoring in SLE
anti-dsDNA C3/C4 ESR
57
Bisphosphnate side effects
osteonecrosis of jaw Oesopphagitis/ulcers allendronate: - atypical # of femoral shaft
58
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
59
Osteopetrosis
Marble bone disease. Fx in Osteoclast fn --> reduced resorption of bone -- VVV.thick/brittle bone --> prone to fracture Ca/PO4-/ALP all normal
60
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 ```
61
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)
62
MAnagemen of adult onset stills
NSAIDs 1st line if fx --> TNF inhib or Il-1 inhib (Anakinra)
63
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
64
Newly diagnosed RA - what Mx to slow dx progression
MTX + one other DMARD + steroid
65
SLE what complement is low
C3 + C4
66
Initial Mx of BEchets
Prednisolone
67
Lupus 1st line MX of choice
Hydroxyxhloroquine then after --> steroids --> Fx ---> Belimumab
68
antiGBM vs vasculitits
anti-GB - normal ESR | OTher vasculitis - raise dESR

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