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1
Q

Why may patients with haematological malignancy present to ICU?

A
  1. Complications of acute presentation e.g. DIC in APML
  2. Neutropenia and sepsis
  3. Respiratory failure - infection, oedema
  4. TLS
  5. GvHD
  6. Complications of chemo and HSCT
  7. GI dysfunction
  8. AKI
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2
Q

Neutropenia

A

Neutrophils < 0.5 x 10^9/l
neutropenic sepsis < 0.5 + clinical infection or temp > 38

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3
Q

what empirical abx should be used for neutropenic sepsis

A

broad spectrum, anti-pseudomonas - taozcin
add aminoglycoside if gram negative suspected

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4
Q

Chemotherapy complications

A

cytotoxic abx - bleomycin (pulp fibrosis), doxorubicin (cardiomyopathy)
anti-metabolites - methotrexate (pulmonary fibrosis, liver)
alkylating agents - cyclophosphamide (pneumonitis`0
anti-microtubule agents - vincristine (peripheral neuropathy)

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5
Q

Complications of HSCT

A

early < 100 days
-infection
- haemorrhage
- acute GVHD
- aplastic anaemia / graft failure
- venocclusive disease - hepatic sinusoidal obstruction
late > 100 days
- chronic GVHD
- infections
- chronic pulmonary disease

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6
Q

GVHD

A

immune mediate disease following allogenic HCT - complex interaction between donor and recipient
enteritis, dermatitis, hepatitis
clinical / histological diagnosis
seattle staging
1-4
percentage skin rash
bilirubin
GI fluid loss ml/day
chronic gvhd more diverse and presents like other autoimmune disorders

treatment of acute
- high dose seteroids
- immunosuppressants
- TPN

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7
Q

typhilitis

A

neutropenic enterocolitis
GI complication of chemotherapy

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8
Q

Cellular origins of haematological malignancies

A

common myeloid progenitor
- CML
- AML
myeloblast
- AML
common lymphoid progenitor –> lymphoblast
- ALL
lymphoblast –> B / T / NK cell
- Lymphoma
- CLL
B cell –> plasma cell
- myeloma

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9
Q

Sepsis in haematological malignancy

A
  • consider indwelling lines
  • higher risk of opportunistic microbes
  • higher risk of MDR organisms
  • daily micro input
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10
Q

Causes of bleeding in critically unwell patients

A

reduced haemostasis
- coagulation factor deficiency - sepsis, DIC, major haemorrhage, vit k deficiency
- platelet dysfunction. deficiency - sepsis, DIC, extracorporeal circuits, major haemorrhage, BM failure
hyperfibrinolysis
- inherited - haemophilia
- cirrhoss
- truauma
secondary disruption of haemostasis
- hypothermia
- hypocalcaemia
- acidosis
- anticoagulants

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11
Q

inherited bleeding disorders

A

Haemophilia A - F VIII deficiency
Haemophilia B - F IX deficiency
- x-linked recesive
- severity determined by magnitude of factor deficiency
- spontaneous bleeding, haemoarhtrosis, GU bleeding, ICH
VwF disease
- commonest inherited bleeding disorder
- easy bruising

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12
Q

Clotting catastrophes

A

thrombotic storm
- underlying procoagulant state
- trigger to initiate clotting
- rapid development of clots
catastrophic thrombotic syndroms
- APLS
- TTP
- DIC
- HIT
- Covid 19

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13
Q

DIC

A

Systemic activation of clotting pathways
generation of fibrin clots leading to organ dysfunction
consumptive coagulapathy
may present with bleeding or clotting or both
ISTH diagnosis
- underlying cause associated with DIC
- platelets
- fibrinogen
- PT
- FDPs

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14
Q

Treatment of DIC

A
  • stable non-bleeding patients - monitoring
  • platelets < 20, planned intervention or bleeding then transfuse
  • TXA contraindicated
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15
Q

inheritable thrombophilia

A

loss of function
- protein c deficiency
- protein s defieicney
- antithrombin deficiency
gain of function
- factor v leiden
- APLS

suspect in thrombosis < 40, family history

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16
Q

Sickle cell disease

A

point mutation chromosome 11 alteration in B chain
HbA –> HbS unstable, precipitates
acute crises -
- vaso-occlusive - acute chest syndrome, stroke, long bone ischaemia, AKI
sequestration
aplastic (parvovirus)
mx - analgesia, rehydration
transfusion if indicated
prevention of sickling - oxygenation, hydration, temperature, analgesia, venous stasis
top up / exchange transfusions (CVA, MODS)
transfusions - viral screening, specialised, extended antibody screening
IX FBC, blood film, sickledex, electrophoresis

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17
Q

Haemophagocytic lymphohistiocytosis

A

aberrant hyper inflammatory hyperferitinaemic immune réponse syndrome
associated with life threatening cytokine storm
familial HLH / secondary HLH
secondary may be triggered by infection (viral, bacterial) or malignancy. if triggered by autoimmune condition - macrophage activation syndrome (SLE, stills disease)
diagnostic criteria 5 of 8 HLH-2004 / HScore
- fever
- splenomegaly
- cytopenias 2+ cell lines
- ferritin > 500
- high triclygerides, low fibrinogen
- haemophagocytosis on BM biopsy

failure of negative feedback controlling inflammation. failure to clear antigens

steroids anakinra (IL-1 antagonist)
mortality 50% +

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18
Q

Heparin induced thrombocytopenia

A

prothrombotic syndrome after exogenous heparin adminsitration
UFH > LMWH
Type 1 - uncommon, mild thrombocytopenia, non-immune, benign. sepsis / burns. continue heparin
Type 2 - more common, significant thrombocytopenia, immune mediated, thrombosis, heparin cessation

PF4 - following platelet activation, released and binds cell surface, modulates immune response
Type 1 - heparin binds PF4, lower activation threshold, aggregation and mild thrombocytopenia
Type 2 - heparin displaces PF4 and forms complex which triggers immune response. IgG anti-PF4 binds to complexes. platelet activation and thrombosis

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19
Q

thrombocytopenia in ICU

A
  • consumption - DIC, HIT
  • Bone marrow suppression - infection, malignancy
  • autoimmune - ITP
  • thrombotic microangiopathies - TTP, HUS
  • liver disease, splenomegaly

decreased production
- sepsis
- BM failure
- alcohol
Consumption
- DIC
- PE
- Trauma
Diltuonal
- fluid resuscitation / massive transfusion
destruction
- Immune - ITP, HIT, APLS
- Non-immune - DIC, intravascular devices

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20
Q

HIT diagnosis and management

A

4 Ts = pretest probability score
- Thrombocytopenia 50% but > 20
- Timing - day 5-10 or < 1
- Thrombosis - new, skin necrosis
- oTher cause - not present
intermediate / high risk
- stop heparin
- use alternative - argatroban (direct thrombin inhibitor), Xa inhibtior, fondaparinux
- multiplate platelet aggregometer
- antigen ELISA - IgG to PF4
- haematologist

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21
Q

Porphyria

A

Group of disorders caused by disruption in heam syntesis
enzyme deficiency in various stages of pathways lead to accumulation
precipitation of acute attack
- medications - cyp450
- fasting
- pregnancy
- alcohol
- acute physiological stress - infection
Acute
- AIP (porphobilinogen deaminase)
- variegate porphyria
Non-acute
- porphoryia cutanea tarda

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22
Q

porphyria pathophysiolgy

A
  • haem supply multistage synthetic pathway
  • enzyme activity changes lead to accumulation of substrate (porphyrins)
    acute attacks when haem requirements increased
    spectrum of clinical features (neurovisceral attacks)
  • CNS - psychiatric, seizures, altered mental state
  • ANS - abdo pain, HTN, tachycardia, nausea, vomiting, urinary retention
  • PNS - motor neuropathy, pain
    diagnosis
  • random urine for urinary porphobilinogen (protect from light)
    management
  • haem arginate
  • resp weakness - support as indicated
  • cvs instability - propanolol, etc
  • seizures - Benzes
  • psych - olanzepien
  • hyponatreemia, malnutition
  • precipitant
  • advice from porphyria specialist

haem arginate - seizures, progressive neuropathy, hyponatraemia 3mg/kg. may clot lines

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23
Q

Thrombotic microangiopathies

A

group of diseases microvascular and macrovascular occlusion from intraluminal thrombus, alongside MAHA and thrombocytopenia
- TTP
- HUS
- others

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24
Q

TTP

A

Pentad
- MAHA, thrombocytopenia, AKI, CNS, fever
presentations usually neurological sequelae, MOD, bleeding
cause
- reduced activity of ADAMTS-13 enzyme
- cleaves VwF
- reduced cleaving -> large VwF multimers –> platelet aggregates (brain, hart, kidney) organ failure. haemolysis occurs as RBCs sheared by aggregates

situations
- primary ADAMTS-13 impaired e.g. genetic
- secondary - HIV, pregnancy, drugs (quinine, trimethoprim), autoimmune

Ix
- ADAMTS-13 assay
- FBC, film, reticulocytes, fragments
- U+E
- autoantibodies ANA, RF
- coagulation screen

Mx
- PLEX 1.5x plasma volume x 3 then 1/24hr
- FFP if delay
- methylprednisolone
- transfusion
- underlying cause

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25
HUS
spectrum of TTP MAHA, thrombocytopenia, AKI Typical HUS - diarrhoea +ve shigella toxoid Atypical HUS - no diarrhoea - pregnancy, infection s.pneumonia, drugs, malignancy, AI typical = supportive mx atypical = Plexiglas
26
presentation of transfusion reactions
acute < 24hr delayed > 24hr presentations - mild - fever or pruritus only - moderate - fever > 39 and or rash or other non-severe - life threatening - resp distress, airway compromise, CVS compromise, bleeding
27
types of transfusion reaction
acute - febrile non-haemolytic - haemolytic - allergic reaction - TRALI - TACO - Bacterial contamination chronic - infection - HIV, HBV, HCV - TA-GVHD
28
generic management of transfusion reaction
- recheck blood and patient compatibility - inspect blood product - stop transfusion, disconnect - return products and labelling to laboratory - report - MHRA
29
specific life threatening transfusion reaction management
anaphylaxis - per usual TRALI - ARDS like within 6hrs - immune or non-immune - supportive mx TACO - acute pulmonary oedema within 6 hrs - furosemide and supportive mx acute haemolytic reaction - donor antigen and recipient antibodies - haemolysis. fever, pain riggers, urticaria, hypotension - falling hb, elevated bile, LDH, Coombs - supportive mx, RRT
30
Adrenocorticol disorders
hyperaldosteronism - hypertension, low K+ - conns syndrome - primary - secondary - low CO state, cirrhosis Cushings syndrome - hypertension, DM, low K+, altered body habitus - ACTH dependent - cushings disease - ACTH independent - adrenal tumour, exogenous steroid Adrenocortical insufficiency - primary - Addisons - autoimmune neoplastic, haemorrhage, drugs - secondary - ACTH deficiency - hypopituitarism , prolonged steroid - tertiary - hypothalamic - infarct
31
features of acute adrenocortical insufficiency
C - vasoplegia, hypotension, shock D - altered consciousness, lethargy, fatigue E - abdo pain vomiting F - dehydration chronic features - skin crease pigmentation, anorexia, associated AI disease e.g. T1DM Blood results - hyponatraemia, hyperkalaemia, hypoglycaemia, uraemia Investigations - random cortisol + U+E, glucose, ACTH, FBC - short synacthen test , imaging Management - hydrocortisone 200mg + 100mg QDS (will influence short synacthen test) - fludrocortisone if using steroid with weak mineralocorticoid - fluid - management of hypoglycaemia - underlying cause
32
Critical illness related corticosteroid insufficiency CIRCI
hypothalamic impairment leading to relative corticosteroid deficiency - refractory hypotension - delirium, coma - nausea, vomiting
33
Steroid conversion
- Hydrocortisone 100mg - Prednisolone 25mg - methylprednisolone 20mg - dexamethasone 3.75mg
34
steroid trials
Corticus - septic shock. no difference in mortality, significantly quicker reversal of shock Adrenal - septic shock - hydrocortisone 200mg - significantly faster reversal of shock Aprocchs - hydrocortisone + fludrocortisone in septic shock - lower mortality and faster shock resolution Crash - TBI steroids bad DEXA-ARDS - steroids good Recovery - steroids good covid Steroids harmful = TBI, cardiac surgery, spinal injury, GBS, CAP
35
DKA
glucose > 11 ketones > 3 pH < 7.3 death from cerebral oedema, hyperkalaemia, ARDS, precipitants
36
DKA causes
acute event on top of absolute insulin deficiency stress response - infection, trauma, CVA, MI, non compliance with treatment altered carbohydrate metabolism - alcohol, pancreatitis insulin deficiency means glucose can't be utilised by tissues increase in glucagon, cortisol, catecholamines increased glycogenolysis, gluconeogenesis hyperglycaemia increased lipolysis - increased FFA which are metabolised to ketone bodies beta-hydroxybutyrate fluid depletion - osmotic diuresis, vomiting
37
differentials of ketosis
alcoholic ketoacidosis - euglycaemia starvation ketoacidosis - slower onset and better compensated. reduced insulin secretion, increased lipolysis
38
DKA admission to critical care
- age 18-25, elderly, pregnant - cardiac or renal failure - serious comobrodity -severe dka - sats < 92% - systolic < 90 - GCS < 12 - ketones > 6 - pH < 7.1 - HCO3 < 5 - K < 3.5
39
Mx priorities in DKA
0-1hr resuscitation 1-6hr clearance of ketones 6-12hr continued improvement and avoidance of complications 12-24hr resolution fluid - resus to sBP > 90 1, 2, 2, 4 hr bags of n/saline KCl 40mmol unless K > 5.5 insulin 0.1units/kg/hr continue long acting if glucose < 14 add 10% glucose refractory DKA - increase FRII target ketones 0.5mmol/hr, bicarb 3mmol/hr, glucose 3mmol/hr resolution ketones < 0.6 pH > 7.3 give subcut insulin when able to eat
40
avoiding DKA complications
thromboprophylaxis maintain UO > 0.5ml/kg/hr hourly monitoring U+E at 4hr underlying precipitant vomiting
41
Hyperosmolar hyperglycaemic state
BM > 30 - absence of ketones < 3 , pH > 7.3 Osmolarity > 320 insidious onset, high mortality from complications - MI, stroke, ODS, cerebral oedema, seizures severe - osm > 350, pH < 7.1, Na > 160, K < 3.5 / > 6, BP < 90, GCS 12, creat > 200, serious comorbidity - consider icu
42
Mx HHS
restore circulating volume, normalise osmolality treat underlying cause and prevent complications - n.saline, or 0.45 if osmolality not falling - FRII 0.05u/hr only after fluid unless ketones 1-3 pH >7.3. if ketones > 3 pH < 7.3 then 0.1u/kg Aims - reduce osmolality 3-8mosm/kg/hr - reduce Na < 10mmol/24hr - 2-3l +ve balance at 6hrs - 500-1000ml/hr n/saline - if osmolaliryt reducing by sodium rising continue 0.9% - if both increasing check fluid balance and increase rat e0.9% - if fluid balance adequate and both increasing switch to 0.45% - glucose fall of 4-6mmol/hr with fluid only - if adequate fluid balance and glucose not falling start insulin - potassium replacement resolution - osmolality < 300 - hypovolaemia correct UO > 0.5 - cognitive status back to premorbid - glucose < 15
43
Euglycemic DKA
SGLT-2 pregnancy, fasting, bariatric surgery, gastroparesis, insulin pump failure, cocaine intoxication, chronic liver disease and glycogen storage disease relative or absolute carbohydrate deficit, milder degree of insulin deficiency or resistance and increased glucagon/insulin ratio
44
symptoms of hypoglycaemia
autonomic - sweating, palpittions neuroglycopenic - confusion, discordination general malaise severe- coma, seizures, hemiparesis enteral - 15-20g quick acting carbohydrate or glucogel if uncooperative IV 100ml 20% glucose or IM glucagon 1mg
45
causes of hypoglycaemia
- failure of enteral nutrition - insuline overdose - sepsis - liver failure - adrenal insufficiency - drugs - sulphonylureas - hypothyroidism - post gastric bypass
46
phaeo
rule of 10s - 10% familial, 10% bilateral, 10% normotensive, 10% malignant, 10% extra-adrenal associated with MEN
47
catecholamine pathways
phenylalanine tyrosine DOPA dopamine NA Adr dopamine - MAO NA / Adrenaline - MAO / COMT to metanephrine, normetanephrine, VMA
48
general phaeo features
- sweating, headaches, palpitations - hypertension - anxiety, lethargy, weight loss, tremor - incidental finding acute crisis - MODS - Pulmonary oedema - hypertensive emergency , arrhythmias, MI , takutsubo CM - stroke, ICH, seizures - AKI - DIC plasma / urine metanephrins 24 hr urinary VMA , catecholamines, homovanillic acid adrenal CT
49
mx phaeo crisis
supportive care specifics - alpha blockade first - phentolamine (IV), phenoxybenzamine (PO) for BP control - B1 blockade atenolol / metoprolol for arrhythmias - fluid repletion - myocardial optimisation - echo, ecg - glucose and electrolyte management adequate alpha blockade - BP controlled, infrequent ectopics, no st changes, orthostatic hypotension, nasal congestion tumour handling in theatre - phentolamine, GTN, esmolol, magnesium, remifentanil post-op hypotension - NA. IABP monitoring. analgesia. steroid if bilateral. glucose monitoring. hypertension - complete resection
50
carcinoid
liver or post hepatic mets of neuroendocrine tumours release of hormones into circulation - serotonin, catecholamines flushing, wheeze, palpitations, diarrhoea crisis exacerbated but stress or surgery. severe bronchospasm, labie BP, arrhythmias, diarrhoea. octreotide
51
post op trans-sphenoidal surgery
general supportive care avoid cpap / NPA avoid ponv analgesia hormone - glucocorticoids 8hrly post-op. test GH / GTT, endocrine review complications - DI, SIADH, CSF leak, meningitis, bleeding (visual fields)
52
pituitary apoplexy
hypo perfusion of pituitary gland leading to infarction life and sight threatening hormone deficient and mass effect precipitated by hypertension, surgery, anticoagulants, radiotherapy, trauma Sheehans - secondary to hypo perfusion in peurperneium life threatening futures - cvs collapse due to adrenal insufficiency, seizures due to hyponatraema glucocorticoid replacement, specialist input, surgical decompression
53
thyroid storm
rare thyrotoxic crisis with extreme hyper metabolic state. chronic progression of thyrotoxicosis with crises precipitated by acute stressor continuation of spectrum of thyrotoxicosis with end organ dysfunction serum thyroid levels don't correlate with severity of disease
54
thyroid storm diagnosis
clinical burch-wartofsky score assesses decompensation (thermoregulatory, heart failure, AF, CNS, liver)
55
thyroid storm management
general - cooling - fluid - treatment of arrhythmias - CNS support - CO monitoring specific - anti-thyroid medication - propylthiouracil (carbimazole second line) - iodine > 1 hour after - corticosteroid - beta blockade - propanolol, esmolol Refractory - PLEX, thyroidectomy
56
myxoedema coma
life threatening complication of hypothyroidism primary hypothyroidism precipitated by MI, HF, stroke, drugs, cessation of thyroxine
57
myxoedema features
B - respiratory failure C - bradycardia, BBB, cardiac failure D - reduced consciousness E - hypothermia, hypoglycaemia F - rhabdo, hyponatraemia dry skin, obesity, hair loss Enteral T4, IV T3 Steroids supportive care
58
skin important in critical care
provided physical barrier, moisture, temperature regulation, sensation and immunity may be threatened by acute illness primary and secondary skin conditions may require critical care important conditions that may arise during critical are - pressure ulcers - peripheral gangrene - extravasation - intertrigo
59
acute skin failure
total dysfunction of skin primary skin conditions - burns - infection - nec fasc, cellulitis - TSS - TEN / SJS - Erythroderma Severe cutaneous adverse reaction (SCAR) term for severe drug reactions - SJS, TEN, DRESS
60
DRESS
drug reaction, eosinophilia, systemic symptoms antiepletpcis, antibiotics, antivirals, biologics 2-6 weeks from trigger prodromal malaise skin changes - erythematous morbilliform rash, face first, pruritic, confluent, pustulation, painful, widespread lymphadenopathy, eosinophilia, deranged LFTs, AKI, ARDS specialist input, remove cause, steroids
61
SJS / TEN
acute life threatening drug reaction causes skin lesions, with epidermal loss and mucous membrane involvement < 10% TBSA = SJS 10-30% = SJS/TEN > 30% = TEN drugs - sulphonamides, beta lactase, anticonvulsants. rarely CMV, mycoplasma. prodrome painful erythema macules --> targetoid --> blisters --> sheet line skin loss loss of epidermis with direct pressure extensive necrosis
62
TEN / SJS assessment
SCORTEN - age > 40 - bicarbonate < 20 - cancer - urea > 10 - > 10% detachment - HR > 120 - Glucose > 14
63
Mx TEN / SJS
supportive - fluid resus - temp regulation - access through undamaged skin - nutrition - analgesia, sedation - burns centre / MDT specific - remove cause - swab for bacteria - non-adhesive dressings - debridement - steroids - ciclosporin - PLEX / IVIG
64
rheumatological conditions in critical care
encompass inflammatory conditions affecting muscoskeletal system as well as systemic inflammatory conditions - rheumatoid arthritis - seronegative arthropathies - Ank spon, psoriatic arthritis - connective tissue diseases - SLE, polymyositis, dermatomyositis, Marfan - vasculitides - degenerative - oA - other - gout may present to critical care - progression or complications of disease - complications of therapy - unrelated may be complex - multisystem - MOD - complicated pharamcology - broad differentials - unusual complications - MAS, scleroderma renal crisis
65
Vaculitides
ANCA associated vasculitis - autoimmune disease of blood vessels with ANCA +ve - Microscopic polyangitis (glomerulonephritis) - Granulomatosis with polyangitis (alveolar haemorrhage, glomerulonephritis) - eosinophilic granulomatosis with polyangitis (nasal polyps, asthma, cardiac) - behcets syndrome
66
rheumatological effects by system
A - AAI, TMJD, cricoarytenoid dysfunction B - restrictive lung disease, haemorrhage, effusion C - aortic root, valve issues, pHTN, CCF, effusion D - peripheral neuropathy E - thin skin, arthritis, hypermobility F - glomerulonephritis G - weight loss, H - anaemia I - pyrexia HLH / MAS
67
Pharmacotherapies
NSAIDs steroids DMARDS - 5-ASA - Azathioprine - Methotrexate - Ciclosporin Biologics - Anti TNF - infliximab, adalibumab - Anti CD20 -rituximab - IL6 - toxiluzimab
68
Catastrophic APLS
APLS - recurrent venous and arterial thrombosis, pregnancy disorders, APL antibodies catastrophic APLS - rare accelerated disease MOD with TMA following precipitating event heparin, PLEX, immunomodulation
69
scleroderma renal crisis
systemic sclerosis - autoimmune inflammation and fibrosis of multiple organs including skin scleroderma renal crisis - rapid progressive HTN with AKI (10% of patients with SS) reduced renal perfusion after thickening of eateries headache, visual disturbance, encephalopathy. LVF, my-pericarditis, MAHA Mx with ACEi, BP control, PLEX, RRT
70
Oncology referrals to icu
related to cancer - compression - local spread - medical complications - PE, TLS, febrile neutropenia related to treatment - post-operative - chemo - neutropenic sepsis, pneumonitis, TLS - HSCT - sepsis - radiotherapy proctits / pneumonitis - immunotherapy - immune related reaction - CAR-T cell - CRS, neurotoxicity unrelated
71
Chemotherapy agents and complications
bleomycin - lung toxicity doxorubicin - cardiotoxicity platinum compounds - peripheral neuropahty alkalyating agents - renal failure methotrexate - liver, renal, lung toxicity
72
febrile neutropenia
fever > 38.3 nets < 0.5 10% mortality neutropenic sepsis - sepsis 3 + mneutropenia organisms - pseudomonas, staph aureus, prep (PCP, fungus) abx, gcsf source control attention to indwelling lines microbiliogist involvment
73
immunotherapy
imune checkpoint inhibitors - monoclonal antibodies CAR-T cell therapy non-specific - cytokines IL-2 side effects - unfusomn reactions - CRS - sHLH Mabs -pneumontisi, CM, neurotoxicity, TLS
74
CAR-T
chimeric antigen receptor T cell therapy T cells removed from patient, genetically modified with chimeric antigen receptors chimeric cells cloned and returned triggers immune response against malignant cells - cytokine release syndrome - supraphysuological response leading to activation of immune effector cells. may cause ARDS, high dose vasopressor. steroids, IL=6 - immune effector cell associated neurotoxicity - encepahlopathy, neurotot=xicity from car-t - tremor --> seizure --> coma. anakinra
75
Assessment of patients with malignancy
physiology - reserve - disease progression - organ involvement treatment to date proposed treatment effects of treatment acute reversibility vs underlying disease co-morbidities patient wishes
76
challenges of admitting patients with malignancy
benefit vs risk judgement on life expectancy with or without critical care and view of not prolonging dying process - extensive range of diease - limited evidence - scoring systems don't perform well in those with malignancy - unreliable judgements - tertiary cancer centres have different experience - evolving therapies - uncertain prognosis - patient variability in terms of acceptable burden of treatment - improving outcomes individualised, MDT
77
Systemic sclerosis
rare autoimmune, inflammatory, progressive multisystemc CTD small vessel vasculopathy, abnormal collagen deposition hallmark = scleroderma = hardening of skin - limited cutaneous systemic sclerosis - diffuse cutaneous systemic sclerosis (proximal to knee / elbow) may overlap with other CTD e.g. myositis, sjogrens vasculopathy / immune activation / fibroblasts mx - ace for SRC, immunosuppressants, calcium channel blockers for reynauds
78
Limited cutaneous systemic sclerosis
Previous CREST ( calcinosis, reynaouds, oesophageal dysmomitility, sclerodacytyl, telangectasia anti-centromere autoantibody long interval between reynauds and skin
79
diffuse cutaneous systemic sclerosis
shorter onset skin involvement proximal to knee / elbow anti-scl70 antibodt
80
complications of systemic sclerosis
scleroderma renal crisis pulmonary fibrosis myocardial disease (ischaemia) Pulmonary hypertension
81
scleroderma renal crisis
severe HTN, renal failure, hypertensive encephalopathy, cardaic failure and MAHA fibrosis of renal vasculatre decreased blood flow renal vasospasm renal ischaemia, RAAS activation ACEi --> Ca channel blocker --> dialysis
82
ANCA associated vascultides
small-medium sized blood vessels ANCA in circulation - microscopic polyangitis - granulomatosis with polyangitis - respiraotry complciations - DAH, tracehal stensosi, - renal involvement - eosinophilc granulomastosis with polyangitis - nasal polyps, asthma - heart blocks, myocarditis, pericarditis - single organ AAV
83
Types of vasculitis
large vessel - Giant cell arteritis medium - polyarteritis nodosa, kawasaki small - ANCA associated, HSP
84
Ix in vasculitis
exclude differentials - blood cultures - echo - hepatitis - HIV - Antiglomerular basement membrane antibody - antiphospholipid antibody - antinuclear antibody Assess vasculitis - urine - CXR - ANCA - c-ANCA + anti-PR3 = GPA - p-ANCA + anti-MPO = EGPA - cryoglobulin - biopsy
85
SLE
autoimmune CTD auto antibodies (DNA, RBCs, proteines, phospholipids), circulating immune complexes (deposit in brain, kidney, heart etc), widespread tissue damage females exacerbation when more DNA - infection, trauma features - malar rash, photpsensitivites, ulcers, arthritis, pericrditis, CNS, proteinuria, anaemia, Bloods - ANA, anti-dsDNA, Anti-SM complemeent in exacerbations. 10% rheumatoid factor
86
sjogrens syndrome
dry mouth dry eyes joint pain fatigue swollen glands dry itchy skin comlications - ILD, pancreatitis, reynauds lymphoma
87
behcets disease
inflammatory diroder multisysetm common in middle east - skin and mucosa - aphthous ulcers, genital ulcers, erythema nodosum - inflammtory eye disease - GI disease - lungs - haemoptysisi, pleuritis, cough - arhtirits - glomerulnephritis - aseptic meningitis - pericarditis clinical dx ulcers + 2 out of - eye - genitals - pathergy reaction - skin lesions
88
Autoantibodies
Anti-dsDNA - SLE Anti-mitpchondiral - primary biliary sclerosis Anti-SM - active hepatitis pANCA - EGPA cANCA - GPA Anti-centromere - systemic sclerosis Anti-RO / La - Sjogrens Anti - Jo - polymyositis RF - Rheumatoid

FFICM (14 decks)

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