Medicine Flashcards
Why may patients with haematological malignancy present to ICU?
- Complications of acute presentation e.g. DIC in APML
- Neutropenia and sepsis
- Respiratory failure - infection, oedema
- TLS
- GvHD
- Complications of chemo and HSCT
- GI dysfunction
- AKI
Neutropenia
Neutrophils < 0.5 x 10^9/l
neutropenic sepsis < 0.5 + clinical infection or temp > 38
what empirical abx should be used for neutropenic sepsis
broad spectrum, anti-pseudomonas - taozcin
add aminoglycoside if gram negative suspected
Chemotherapy complications
cytotoxic abx - bleomycin (pulp fibrosis), doxorubicin (cardiomyopathy)
anti-metabolites - methotrexate (pulmonary fibrosis, liver)
alkylating agents - cyclophosphamide (pneumonitis`0
anti-microtubule agents - vincristine (peripheral neuropathy)
Complications of HSCT
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
GVHD
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
typhilitis
neutropenic enterocolitis
GI complication of chemotherapy
Cellular origins of haematological malignancies
common myeloid progenitor
- CML
- AML
myeloblast
- AML
common lymphoid progenitor –> lymphoblast
- ALL
lymphoblast –> B / T / NK cell
- Lymphoma
- CLL
B cell –> plasma cell
- myeloma
Sepsis in haematological malignancy
- consider indwelling lines
- higher risk of opportunistic microbes
- higher risk of MDR organisms
- daily micro input
Causes of bleeding in critically unwell patients
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
inherited bleeding disorders
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
Clotting catastrophes
thrombotic storm
- underlying procoagulant state
- trigger to initiate clotting
- rapid development of clots
catastrophic thrombotic syndroms
- APLS
- TTP
- DIC
- HIT
- Covid 19
DIC
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
Treatment of DIC
- stable non-bleeding patients - monitoring
- platelets < 20, planned intervention or bleeding then transfuse
- TXA contraindicated
inheritable thrombophilia
loss of function
- protein c deficiency
- protein s defieicney
- antithrombin deficiency
gain of function
- factor v leiden
- APLS
suspect in thrombosis < 40, family history
Sickle cell disease
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
Haemophagocytic lymphohistiocytosis
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% +
Heparin induced thrombocytopenia
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
thrombocytopenia in ICU
- 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
HIT diagnosis and management
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
Porphyria
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
porphyria pathophysiolgy
- 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
Thrombotic microangiopathies
group of diseases microvascular and macrovascular occlusion from intraluminal thrombus, alongside MAHA and thrombocytopenia
- TTP
- HUS
- others
TTP
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
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
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
types of transfusion reaction
acute
- febrile non-haemolytic
- haemolytic
- allergic reaction
- TRALI
- TACO
- Bacterial contamination
chronic
- infection - HIV, HBV, HCV
- TA-GVHD
generic management of transfusion reaction
- recheck blood and patient compatibility
- inspect blood product
- stop transfusion, disconnect
- return products and labelling to laboratory
- report - MHRA
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
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
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
Critical illness related corticosteroid insufficiency CIRCI
hypothalamic impairment leading to relative corticosteroid deficiency
- refractory hypotension
- delirium, coma
- nausea, vomiting
Steroid conversion
- Hydrocortisone 100mg
- Prednisolone 25mg
- methylprednisolone 20mg
- dexamethasone 3.75mg
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
DKA
glucose > 11
ketones > 3
pH < 7.3
death from cerebral oedema, hyperkalaemia, ARDS, precipitants
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
differentials of ketosis
alcoholic ketoacidosis - euglycaemia
starvation ketoacidosis - slower onset and better compensated. reduced insulin secretion, increased lipolysis
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
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
avoiding DKA complications
thromboprophylaxis
maintain UO > 0.5ml/kg/hr
hourly monitoring
U+E at 4hr
underlying precipitant
vomiting
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
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
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
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
causes of hypoglycaemia
- failure of enteral nutrition
- insuline overdose
- sepsis
- liver failure
- adrenal insufficiency
- drugs - sulphonylureas
- hypothyroidism
- post gastric bypass
phaeo
rule of 10s - 10% familial, 10% bilateral, 10% normotensive, 10% malignant, 10% extra-adrenal
associated with MEN
catecholamine pathways
phenylalanine
tyrosine
DOPA
dopamine
NA
Adr
dopamine - MAO
NA / Adrenaline - MAO / COMT to metanephrine, normetanephrine, VMA
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
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
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
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)
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
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
thyroid storm diagnosis
clinical
burch-wartofsky score assesses decompensation (thermoregulatory, heart failure, AF, CNS, liver)
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
myxoedema coma
life threatening complication of hypothyroidism
primary hypothyroidism precipitated by MI, HF, stroke, drugs, cessation of thyroxine
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
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
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
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
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
TEN / SJS assessment
SCORTEN
- age > 40
- bicarbonate < 20
- cancer
- urea > 10
- > 10% detachment
- HR > 120
- Glucose > 14
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
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
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
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
Pharmacotherapies
NSAIDs
steroids
DMARDS
- 5-ASA
- Azathioprine
- Methotrexate
- Ciclosporin
Biologics
- Anti TNF - infliximab, adalibumab
- Anti CD20 -rituximab
- IL6 - toxiluzimab
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
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
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
Chemotherapy agents and complications
bleomycin - lung toxicity
doxorubicin - cardiotoxicity
platinum compounds - peripheral neuropahty
alkalyating agents - renal failure
methotrexate - liver, renal, lung toxicity
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
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
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
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
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
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
Limited cutaneous systemic sclerosis
Previous CREST ( calcinosis, reynaouds, oesophageal dysmomitility, sclerodacytyl, telangectasia
anti-centromere autoantibody
long interval between reynauds and skin
diffuse cutaneous systemic sclerosis
shorter onset
skin involvement proximal to knee / elbow
anti-scl70 antibodt
complications of systemic sclerosis
scleroderma renal crisis
pulmonary fibrosis
myocardial disease (ischaemia)
Pulmonary hypertension
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
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
Types of vasculitis
large vessel - Giant cell arteritis
medium - polyarteritis nodosa, kawasaki
small - ANCA associated, HSP
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
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
sjogrens syndrome
dry mouth dry eyes
joint pain fatigue swollen glands
dry itchy skin
comlications - ILD, pancreatitis, reynauds
lymphoma
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
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
