Long case spiels Flashcards
Heart failure/Cardiomyopathy
Differentials: Idiopathic Ischemic Hypertension Valve related Myocarditis Alcohol Rate related Takusubo Post partum Haemochromatosis/amyloidosis/sarcoidosis Endocrinopathies: Hyperthyroidism/acromegaly HCM/ARVC
Investigations:
CXR - pumonary odema, cardiomegaly
ECG - arrythmias, q-waves to suggest previous ischemia
ECHO - LV function, regional wall motion abnormalites, valve disease
ETT/ESE/Angiogram - to exclude ischemia
Bloods - renal, liver, iron studies, TFTs, anaemia, SFLC
Cardiac MRI
Endomyocardial biopsy
Management: Non-pharmacological - education - daily weights (greater than 2kgs over target weight, should prompt action) - fluid restriction in severe CHF less than 1.5L - low salt diet - heart failure nurse - cardiac rehabilitation - avoid alcohol - smoking cessation - vaccinations - driving (no driving with ICD or symptomatic CHF) - advanced care planning Pharmacological - diuretics - loop and thiazides (symptoms only) - ACE-I (survival) - B-blockers (survival) - Aldosterone antagonist (survival) - Digoxin (controversial, increase in mortality) - Long acting nitrates (symptoms) - Others - Ivabradine, angiotensin neprilisn inhibiton Devices - CRT (survival) - ICD (survival benefit) Surgery - heart transplant
Parkinson’s disease
Key features:
Motor: Tremor, akinesia, rigidity, postural instability
Non-motor: cognitive dysfunction and dementia, psychosis and hallucinations, mood disorders, sleep disorders, autonomic dysfunction (postural hypotension/constipation/sexual dysfunction/urinary incontinence)
Differentials: Idiopathic parkinson's disease Dementia with lewy bodies Atypical parkinsons syndromes (PSP, MSA, corticobasal degeneration) Drug induced Normal pressure hydrocephalus Wilson's disease (young patients) Vascular parkinsons
Investigations:
Clinical diagnosis
Confirmed with response to levodopa therapy
MRI can exclude other causes
Management:
Non-pharmacological
- education
- support groups
- physiotherapy
- speech and language therapist
- dietitian
- social worker (carer stress)
- occupational therapy
- cognitive behavioural therapy/counselling
- orthostatic advice
- increase fluid and fibre intake (constipation)
- asessment of cognition
- advanced care planning
Pharmacological
- laxatives
- consider cholinesterase inhibitor for cognitive impairment
- psychosis - only evidence is for Clozapine but quetiapine can be used (others make much worse)
- Levodopa combined with peripheral decarboxylase inhibitor = best therapy
- Dopamine agonists (risk of impulse control disorder)
- Mao-B inhibitors, anticholinergics, amantadine, COMT inhibitors
Surgery
- deep brain stimulation (subthalamic nucleus/globus pallidus)
Monitor for on/off phenomenon and dyskinesias
- best treatment is to fractionate L-dopa - give low doses more frequently
- add COMT inhibitor (entacapone etc.)
- DBS early
Cognitive impairment
Differentials: Delirium (illness, drugs, alcohol withdrawal etc.) Mild cognitive impairment Alzheimer Vascular Lewy body demenita Frontotemporal Normal pressure hydrocephalus Depression
Investigations:
Remember clock drawing and AMT in long case
Collateral history
Review medication chart
Bloods: electrolyte derangement, inflammatory markers, B12/folate, TFTs
Urinalysis, bowel chart
CT head: vascular change, NPH
Geriatric depression scale
MOCA/ACE-III
Functional assessment (important in distinguishing from MCI and dementia)
Management: Non pharmacological - Involve family and main supports - EPOA - Education - Consider safety, communication, environment - Manage pain - Assess hearing and vision - Support groups e.g Alzheimer society - Dietitian - high risk of malnutrition - SLT - dysphagia - Social worker - supports, respite, carer stress - OT and PT - advanced care planning - sleep hygiene
Pharmacological
- treat precipitating causes (infections, remove offending drugs)
- consider cholinesterase inhibitor (except for in Frontotemporal dementia)
- anti psychotics for management of aggression/behaviours (try to avoid if possible due to increased stroke risk)
- treat depression with SSRI’s
Palliative management in end stage disease
Diabetes
Investigations: BSLs HBA1c Blood pressure, lipids Results of retinal screening Urine protein creat ratio Creatinine
Management: Type 1 - education - medical alert bracelet - driving - employer supportive? - diabetes nurse - BSL testing at least 4x a day - insulin therapy (usually basal bolus) - role of carbohydrate counting, insulin sensitivy factor and insulin pumps - unwell advice - take insulin, liquid glucose, test for ketones, present early for medical attention - hypoglycemic education - exercise (reduce insulin or supplement with glucose) - regular assessment for complications (see below) - smoking cessation - limit ETOH - pregnancy planning - mental health - screen for other autoimmune disorders - perioperative management
Type 2
- education
- weight loss
- diabetes nurse
- testing and control
- diet advice/alcohol/smoking
- diet controlled, metformin, sulphonylurea, insulin
- newer GLP-1 analogues, DPPIV inhibitors
- statin
- aspirin controversial (can be considered in those with more than one risk factor)
Complications
Microvascular
- nephropathy
- 3 monthly PCR and creat
- blood pressure control (goal less than 130/80) ACE-I
- assess for dialysis or transplant
- PD better for diabetics
- retinopathy
- opthalmology every 2 years
- panphotocoagulation vs avastin
- peripheral neuropathy
- annual review with monofilament
- inspect feet daily
- 4 monthly podiatry
- careful nail care
- footwear
- early medical assessment of skin ulcers
- autonomic neuropathy
- orthostatic advice
- screen for erectile dysfunction
Macrovascular
- PVD/CVA/CVD
Targets:
HbA1c less than 53 indicates good control
Less than 40 in a patient on insulin indicates likely hypoglycemic episodes
ACCORD trial showed increase mortality in elderly with tight control
Maintaining independance
PT - structured excercise programme to maintain strength and balance, walking aids
OT - vocational OT, equipment around the house
Driving assessments
SW - assessments for supports
Driving support agencies - driving miss daisy
Maintain good relationship with GP, possibility of home visits
Support groups - information sharing
Engaging family
Stop smoking and reduce alcohol
Screening for depression
Blisterpack for medications
Osteoporosis
History of fractures and pain
Consider secondary causes: steroids, hypogonadism, thyroid disease, chronic heparin treatment, drugs (lithium, enzyme inducing anti-convulsants) malabsorbtion, malnutrition, connective tissue disease
Investigations
Bloods: ALP, Calcium, Vit D, PTH
DEXA scan - T score less than 2.5
Management: Smoking cessation Alcohol reduction Excercise - weight bearing excercise Calcium - ideally 2 servings of calcium per day Vit D Bisphosphonates Other option: Teriparatide (T score less than -3), Denosumab, Raloxefine
COPD
Non-pharmacological
- education
- written action plan
- nutrition
- smoking cessation
- vaccination (flu vaccine - annually, pneumovax 5 yearly)
- pulmonary rehab (education/optimising medical management, PT, psychosocial support)
- flying advice
- home oxygen (paO2 less than 55, or less than 60 with pul hypertension)
- home BiPap
- lung volume reduction surgery
- lung transplantation
- advanced care planning/hospice/palliative care
Pharmacological
- no evidence for prophylaxic antibiotics
- some role for macrolide as anti-inflammatory
- Step 1: Short acting bronchodilator (b-agonist, anticholinergic)
- Step 2: Add long acting bronchodilator (b-agonist, anticholinergic)
- Step 3: Add inhaled glucocorticoid (indicated for severe COPD with exacerbations)
- Consider adding tiotropium for severe COPD
Treat exacerbations - steroids, oral antibiotics, NIV
Things that improve survival: smoking cessation and oxygen Things that improve QOL: pulmonary rehab - also reduces hospitalisations Things that reduce exacerbations: inhaled corticosteroids LABAs
Falls
Education - home - footwear - alcohol use - diet Consider TaiChi for balance MDT - rehabilitation - OT: environment, free from clutter, rails, ramp, lighting - PT: transfers, education around excercise daily - SW: home alarm, medicalert bracelet Medically - Vision - Hearing - Continence - Hip protectors - Osteoporosis - Vit D, bisphosphonate - Postural hypotension - Medication review (greater than 4 medications = bad)
Long-term corticosteroid use
side effects: MANY!!! immunosuppression adrenal suppression Cushing's syndrome hyperglycaemia and diabetes cardiovascular disease fluid retention osteoporosis (>5mg/day associated with sig reduction in BMD) osteonecrosis cataracts and glaucoma psychiatric disturbance GI bleeding myopathy impaired wound healing
corticosteroids have both glucocorticoid and mineralocorticoid effect:
glucocorticoid - anti-inflammatory, immunosuppression, vasoconstrictive effects
mineralocorticoid - electrolyte and fluid balance
prior to starting long-term steroids
- lowest dose for shortest time
- consider steroid sparing agents where possible
- assess for infection e.g. active shingles, Hepatitis, TB
- assess BMD (DEXA) then 1 year F/U then 2-3 yearly
- assess baseline cardiovascular risk including lipids, HbA1c
adrenals:
- screen for adrenal suppression with early morning cortisol if suspected, if low performed short synacthen (insulin tolerance test is definitive test)
eyes:
annual ophthalmology review if > 6/12 treatment
bones (see osteoporosis speil)
greatest bone loss in first 6-12 months of treatment
adequate dietary calcium intake
Vit D supplementation
all patients on >7.5mg/day of Pred for > 3/12 are at risk
in general those > 65 should be on bisphosphonate
also consider if 15 - insulin
* adjust diabetes medications and increase BSL monitoring if steroid dose changed
GI:
consider PPI for GI protection
Education:
- increase dose when unwell
- don’t stop suddenly
- inform medical professionals of long-term steroid use
- medic-alert bracelet
- symptoms of development of hyperglycaemia
- test BSLs more freq and contact GP/practice nurse if change in dose if diabetic
Lifestyle:
- regular exercise, limit sugar in diet - to prevent excessive weight gain
Transplant
Pre-transplant:
- underlying disease
- function prior to transplant (ie. hospital admissions, requirement for home O2, LVAD, dialysis)
- work up for transplant (cardiovascular, respiratory, CMV, Tb etc.)
Transplant:
- Type of donor (live, deceased, related, unrelated)
- Match of donor
- CMV status
- immediate operative and post operative complications
- Duration since transplant
Post transplant:
- immunosuppression
- prophylaxis (viral/bacterial/fungal)
- ongoing screening (diabetes, hypertension, skin cancers, other malignancies)
- monitoring of graft function
- episodes of rejection
- monitoring for disease recurrence
- current graft function
- social impact of transplant (e.g displacement from home)
- follow up at transplant unit
- understanding of survival of graft
Immunosuppression:
- interactions
- drug monitoring (levels, side effects)
- infective complications
- importance of compliance
Splenectomy
Either elective splenectomy, emergency splenectomy or hyposplenic due to underlying disease (e.g. coeliac disease)
Vaccination pre-splenectomy (or 2 weeks after emergency procedure):
2 weeks before
- meningicoccal
- pneumococcal
- Hib
- influenza
Splenectomy patients are able to have live vaccines
Management post-splenectomy:
- notify all medical practitoners about hyposplenism
- medic-alert bracelet
- seek medical attention early if unwell
- liaise with primary care practitioner with clear instructions for maintenance of vaccinations
- give supply of amoxicillin to take if unwell and unable to immediately access medical care
- daily antibiotics for first 2 years (at least) after splenectomy (highest risk period) - amoxicillin or penicillin V
- avoid travelling to countries with malaria
- advise of high risk of infection after animal bites
- carry splenectomy card with you at all times
Follow up vaccines:
Meningococcal - every 5 years
Pneumococcal - every 5 years
Yearly influenza vaccine
Cirrhosis
Compensated cirrhosis
- asymptomatic, no varices/ascites
- median survival greater than 12 y
Decompensated cirrhosis
- symptomatic
- development of jaundice, ascites, variceal haemorrhage or hepatic encephalopathy
- 1 year survival 61%
Management and monitoring of complications
General:
- vaccinations
- abstinence
- avoid hepatotoxins
- weight loss
- screen for HCC every 6 months with USS
Ascites (1 y survival = 50%)
- SAAG greater than 11 = due to portal hypertension
- salt restriction
- diuretics; spironolactone better than frusemide
- large volume paracentesis
Varices (mortality 20% with each bleed)
- endoscopy at diagnosis of cirrhosis
- no varices = repeat 2-3 y intervals
- varices = risk assessment; small + red wale mark or large (greater than 5mm), then prophylaxis
- primary prophylaxis: non selective beta blocker or endoscopic variceal ligation
- secondary prophylaxis (prev haemorrhage): both non selective beta blocker + endoscopic variceal ligation
- acute bleeding: non aggressive resus, terlipressin, antibiotics, endoscopic banding, balloon tamponade, TIPS (transjugular intrahepatic portosystemic shunt)
Spontaneous bacterial peritonitis (mortality 20% with each episode)
- ascitic tap = PMN greater than 250, positive micro
- ceftriaxone
- recur in 70%; prophylaxis with norfloxacin
Hepatorenal syndrome
- type 1 = acute onset with associated drop in BP, poor prognosis (median survival 2 weeks)
- type 2 = slow progression of renal impairment (6 month survival)
- consider terlipression
- dialysis as bridge to transplant
Hepatic encephalopathy (1 y survival 40%)
- treat precipitant: infection, bleeding, constipation, diarrhoea, drugs, metabolic derangement
- lactulose
- rifaximin
- do not protein restrict (increases mortality)
Chronic kidney disease
General management
- avoid nephrotoxins
- target BP 130/80 or 125/75 if proteinuria
- ACE-I or ARB for proteinuria
- lifestyle modifications: alcohol, smoking, exercise
- control glucose (target HbA1c less than 53)
- reduce drug doses as appropriate
- monitor GFR 3 monthly
Complications
- fluid overload: need higher doses of diuretics
- hyperkalaemia: low K diet, avoid drugs (NSAIDs)
- CVD: leading cause of mortality
- anaemia (seen in Stage 3-4 CKD, typically less than 30ml/min); consider EPO if Hb less than 100, must be iron replete (ferritin greater than 200), consider inflammation, hyperparathyroidism, aim Hb 100-120
- haemostasis: DDAVP, cryoprecipitate, blood tx
- uraemic peripheral neuropathy
- Ca/PO4: dietary PO4 restriction, phosphate binders (calcium containing if hypocalcaemia), activated Vitamin D, consider cinacalcet and parathyroidectomy
- calciphylaxis: wound care, high dose oxygen, sodium thiosulphate with dialysis, increased time on haemodialysis, stop warfarin and iron
Non-pharamacological
- diet: get a dietician involved
- low sodium and K+ diet
- fluid restriction
Common drug interactions to remember in transplant patients
Drugs for infection:
Azole antifungals - inhibit CYP3A4 = increase CNI levels
Macrolides (except Azithromycin) also inhibit CYP3A4
Rifampicin induces 3A4 = lower drug levels
Hypertension:
Non-dihydropyridine calcium channel blockers (Diltiazem/Verapamil) inhibit 3A4 = increase CNI levels (can be used as a CNI sparing agent)
Antidepressants:
SSRIs have mild inhibiton of 3A4 - Sertraline and Ecitalopram considered best of antidepressants
Statin:
Metabolised through CYP3A4, CNIs inhibit this and risk of developing statin myopathy and liver failure
- use pravastatin (low potency steroid and monitor LFTs and CK)
Alcohol
Guidelines
Women - no more than 10 drinks per week, no more than 2 drinks per day
Men - no more than 15 drinks per week, no more than 3 every per day
2 alcohol free days per week
CAGE questions:
Are you Concerned about your drinking?
Do you get Angry if anyone criticizes your drinking?
Do you feel Guilty about drinking?
Do you need an drink early in the morning (Eye opener)?
If no signs of alcohol abuse or dependence then patient can still have “at risk drinking”
Signs of alcohol abuse:
- risk of bodily harm
- relationship affect
- role interference (Work/family)
- run-ins with the law
Signs of alcohol dependence:
- craving alcohol
- loss of control (inability to cease drinking)
- withdrawal symptoms
- tolerance
Brief intervention
- state drinking is medically unsafe
- ask willingness to reduce alcohol intake
- negotiate goal (abstience is best)
- offer support
- screen often
Readiness to change: Pre-contemplation Contemplation Planning Action Maintaince
Support
- information
- addiction specialist (if dependance)
- support groups (AA)
- counselling
- medications (e.g. Disulfiram, Naltrexone)
Treat associated disorders (nicotine dependence, medical and psychiatric)
Medical manifestations of alcohol use:
- atrial fibrillation
- reflux
- macrocytosis
- peripheral neuropathies
- chronic pancreatitis
- chronic liver disease
- hypertension
- cardiomyopathy
- malignancy
- wernickes (ataxia, confusion, opthalmoplegia), Korsakoffs
Adolescents
HEADS assessment
HOME
- living situation
- health of family members
- family relationships
- domestic violence/substance abuse in household
EDUCATION/EMPLOYMENT
- schooling, doing well?, relationship with teachers
- future plans
- bullying
- work
ACTIVITIES
- peers/friends
- hobbies
- interests
- religion
- sport
- gang/involvement with police
- driving
DRUGS
- smoking/alcohol/other drugs
- drink driving
- payment for drugs
SEXUALITY
- sexually active
- relationship
- contraception
- STD/pregnancy/termination
- abuse
SUICIDE AND DEPRESSION
- eating disorders
Main causes of morbidity/mortality:
- unintentional injuries (e.g. MVA)
- alcohol and drug abuse
- unwanted pregnancy
- STD
- eating disorders
- mood disorders
Sexual dysfunction
Screen for associated disorders
- diabetes
- alcohol use
- peripheral vascular disease
- cardiovascular disease
- hypogonadism
- depression
- spinal cord disease
Discuss affect on relationship
Psychological contribution
Medications that could be contributing
Modify risk factors
Offer counselling
Sildenafil (1 hour before sex, PDE5 inhibitors)
Vacum devices/rings etc.
Domestic violence
Types of violence:
Physical, sexual, psychological, financial, neglect
Populations to consider:
Elderly, migrants, disabled
Assess for imminent danger - police Support networks - womans refuge, youthline etc. Providing education and resources Counselling Anger management Social work
Depression
Screening (2 questions)
In the last month have you
- felt low, depressed or hopeless?
- had little interest or pleasure in doing things?
Management:
Assess risk (suicide) -> urgent psychiatric referral
Frequent follow up
Guided self help resources, internet
Web based CBT
Cognitive behavioral therapy/interpersonal therapy
Relaxation techniques
Excercise/diet/sleep hygiene/avoid alcohol
Drug therapy
- SSRIs recommended first line
- Fluoxetine for children and adolescents
- Citalopram, E-citalopram, Sertraline for those on other medicaitons as lower risk of drug interactions
Follow up
- monitor frequently (increased risk of suicide with initiation for therapy)
- if no response at 4-6 weeks consider compliance, increasing dose or change to alternative drug
- duration of therapy should be at least 6 months to reduce relapse
- when stopping decrease dose gradually, remember washout period
Refer to psychiatry service if high risk, possible other diagnosis, failed treatment, recurrent depression
Sleep
Evaluating sleep
- time to bed, time to sleep, awakenings, daytime sleeps
Assess psychological - anxiety, depression
Sleep environment
Physical (OSA, heart failure, pain, restless legs, urinary frequency)
Management:
Sleep diary
A - avoid health S - sleep/sex only use of bed L - leave laptops out of the bedroom E - excercise reguarly, not close to sleep E - early rising, avoid daytime naps P - plan for bedtime, bath or warm drink
Relaxation therapy
Drug treatment
- avoid if possible
- melatonin (good for adjustment e.g jetlag)
- short acting benzos for short amounts of time if required
Ladies, babies, transplants and immunosuppression
- Fertility can be restored from 1-2 months post transplant
- Must use effective contraception
- Ideal time for pregnancy post transplant is after 2 years when graft function and immunosuppression is STABLE
- Mother, fetus and graft are all at risk
Pre-conception counselling
Regular visits with obstetrician, specialist, neonatologist in high risk clinic
Vaccinations UTD - Hep B, Influenza, Pneumococcus
*Rubella should be given prior to transplant as this is a live vaccine (if not already vaccinated)
Maternal complications:
- Most common is pregnancy-induced HTN which is strongly associated with pre-conception renal dysfunction
fetal complications:
- higher rates of prematurity, IUGR, low birth weight, perinatal infections
- similar rates of congenital anomalies to general pop
Factors associated with good pregnancy outcomes:
- Good general health for about 2 years after Tx
- No graft rejection in the last year
- Good, stable graft function
- No acute infections that might affect the foetus
- Maintenance immunosuppression at stable doses
- Patient compliance with treatment and follow-up
- Well controlled BP
- basically everything is stable and functioning well*
Delivery
- Vaginal delivery preferred
- Increased steroid dose when labouring due to stress
- Antibiotic cover if any procedures required
Drugs safe in pregnancy:
-corticosteroids, Azathioprine, Cyclosporine
Not safe in Pregnancy:
- MMF, Sirolimus, MTX
- change to alternative at least 6/52 prior to conception
Prednisone safe in BF but others not
Other considerations:
- referral to genetics if hereditary condition (e.g. PCKD) is the reason for transplant
- check smears UTD in all ladies
Initiating insulin
in type 2 diabetes insulin should be used in conjunctive with or following
- diet and lifestyle management
- initiation of Metformin
- initiation of Sulphonylurea
consider in those with HbA1c > 65% (or if other measures are not achieving a previously agreed upon target)
patient must measure BSLs for a week prior to initiating insulin
- pre meals and 2 hours after
Isophane is usually first line (intermediate acting insulin) usually once a day initially, 8-10 units
If required BD then stop sulphonylurea
Long acting insulins - Glargine, are indicated when there are concerns re hypos
doses need to be titrated up weekly
Education: administration, storage of insulin BSL monitoring and meter use must continue to exercise as insulin results in weight gain managing hypos - education re symptoms - take BSL - eat 6 jellybeans - re-check after 10-15 mins and repeat until BSL > 4 then eat a more complex snack
if employed as a vocational driver and taking insulin then should be referred to diabetes clinic
other drivers should know their BSL prior to driving and carry glucose in their car in case of hypos
involve diabetes CNS
Coeliac disease
Consider in those with: malabsorption - steatorrhoea - unexplained iron deficiency (Courts) - osteoporosis/osteomalacia Other autoimmune disease
Test: must be done whilst eating gluten
tissue transglutaminase
- IgA deficiency is tested for and an IgGtTG level performed instead
If equivocal can perform HLADQ2 and DQ8 - if negative then highly unlikely to have CD
If positive serology then confirm with small bowel Bx
- must be eating gluten
- villous atrophy evident
Management: gluten-free diet replace deficiencies seek and treat osteoporosis can re-scope on 3 months
symptoms resolve in weeks
histology resolves in months
tTG Abs normalise in 3-6 months
Complications:
“your spleen disappears” - P. Roberts (splenic atrophy)
T cell lymphoma (consider if enlarged spleen)
slightly increased risk of bowel Ca
ulceration of small bowel
osteoporosis
Immunise as per splenectomy
- pneumococcus
- Hib
- meningococcus
IBD general principles
peak age of onset 20-40 years
Symptoms: diarrhoea +/- blood, mucous faecal urgency, tenesmus abdo pain/cramps fistula, abscesses mouth ulcers
non-bowel manifestations: joints: arthraligias, sacrolitilitis, Ank spons skin: erythema nodosum, pyoderma gangrenous liver disease: autoimmune, PSC urinary: renal stones malabsorption anaemia osteoporosis/osteomalcia
10-20% have FH
Ix: FBC, U&E, LFTs, CRP iron, B12, folate coeliac serology (Ddx) - anti tTG ABs stool cultures (Ddx infectious colitis)
colonoscopy
AXR if acute pain
CT/MRI enteroclysis
signs of severe disease:
- Severe abdominal pain + tenderness
- Severe diarrhoea (>8/day)+/- without bleeding
- Dramatic weight loss
- Fever or severe systemic illness
General management of IBD:
- Initial management of relapse
- Recognising complications
- Providing ongoing medication and monitoring for adverse effects
- Providing education and support - IBD support groups, IBD nurse, stoma nurse, dietician
goals:
bring active disease to remission
maintain remission
surveillance:
colonoscopy after 8 years of disease (not required if only proctitis)
then 2-3 yearly
* annually if UC associated with PSC
Management of UC
corticosteroids:
- used in acute flares and tapered off
- no role in maintenance
5-ASA (Pentasa)
- if proctitis only can use enemas/suppositories
SEs: blood dyscrasias
Azathioprine or 6-mercaptopurine - check TPMT level before starting - monitor FBC weekly then 2 weekly then monthly - monitor LFTs monthly SEs: leukopaenia, pancreatitis, lymphoma
Anti-TNFs: Infliximab, Adalimumab prior to starting: - update imms - pap smear - Hep B and C serology - CXR and Quantiferon gold SEs: infusion reaction, infections, lymphomas incl hepatosplenic T cell lymphoma, demyelinating disease, CHF
Surgery: can be curative in UC - up to 40% will require
indications:
- Lack of response or intolerance to medications
- Acute complications e.g. toxic megacolon or haemorrhage
- Precancerous or cancerous changes in the colon
* can get pouchitis after surgery
Acute severe flare:
- fluids, correct electrolytes and anaemia
- antibiotics
- IV steroids - if no improvement then salvage therapy with Infliximab or Cyclosporin or surgery
if raised ALP > 3 x ULN consider associated PSC
- associated with pANCA
Crohn’s disease
5-ASA
- more effective for colonic disease
Budesonide
- ileocolic disease
Azathioprine or 6-MP or MTX
- steroid sparing agents to reduce relapse
MTX
before starting
- start folic acid
- baseline LFTs, Hep B and C serology, CXR
- avoid alcohol
monitoring:
- monthly LFTs for 3 months then 3 monthly
SEs: hepatotoxicity, bone marrow suppression, interstitial pneumonitis
- contraindicated in pregnancy
TNF-alpha inhibitors
- heals fistulas well
- refractory disease
Severe disease flare:
3 days IV corticosteroids then either salvage therapy with Infliximab or surgery
Surgery:
- for complications such as fistula, abscesses, obstruction not responding to medical management
- recurrence rate of disease is unchanged by surgery
note: ileoanal pouch formation reduces fertility
Vasculitis
Granulomatosis with polyangiitis - small-medium vessels - URT, LRT and renal - cANCA positive GCA - medium vessels - assoc with PMR - 60-70's - headache, jaw claudication, visual loss Polyarteritis nodosa - medium vessels - multiple systems - coronaries, mesenteric (abode pain), renal, mono neuritis multiplex - assoc with Hep B Chung-Strauss - small vessels - pulmonary-renal syndrome - asthmatic phase -> eosinophilic -> vasculitic - peripheral neuropathy, mononeuritis multiplex - pANCA (50%) - tissue Bx - eosinophilia Microscopic polyarertitis - small vessels - pulmonary-renal syndrome - pANCA - renal biopsy confirms diagnosis
General principles of management:
Investigations:
ANCA - cANCA (anti PR3), pANCA (anti MPO)
ESR typically > 70
FBC - normocytic anaemia and neutrophilia common, eosinophilia in C-S
Renal function (GPA, PAN, MPA)
LFTs
Urine - casts, dysmorphic RBCs
CXR - GPA, C-S
Biopsy - most reliable for making Dx
exclude infection, malignancy or autoimmune disease
Treatment:
agressive immunosuppression to prevent permanent injury
- steroids
- Cyclophosphamide for renal or lung disease (GPA, PAN) - need monitoring of blood count, monitoring for haematuria (bladder cancer)
Issues to discuss around:
- long-term steroids
- osteoporosis
- immunosuppression and infection risk
- HTN, diabetes
- cardiovascular risk increased
Scleroderma
establish whether limited (to elbows +/- face) or diffuse (above elbows and involving chest/organs)
SKIN: Calcinoshs, Raynaud’s, Sclerodactyly, Telangiectasia
- ischaemia of digits
JOINTS: asthropathy in rheumatoid distribution, CTS
GI: dysphagia, heartburn, diarrhoea (malabsorption, bacterial overgrowth), incontinence
RENAL: HTN, CKD, renal crisis
RESP: ILD (diffuse), Pulm HTN (limited), pleurisy
CARDIO: pericarditis, arythmies, CHF
other: erectile dysfunction, hypothyroidism
ASSESS FUNCTION
Investigations:
ESR/CRP raised
FBC - anaemia chronic disease, bleeding oesophagi’s
Iron/B12/folate - malabsorption
Renal
Anti-Scl 70 - positive in a MINORITY of diffuse
- associated with ILD
Anti-centromere AB - 70% with limited (CREST)
Ix of malasoprtion and dysphagia
- OGD, oesophageal manometry
Monitoring for ILD and pulm HTN as early Mx prevents complications
- PFTs, HRCT for ILD
- Echo, R heart cath, 6 min walk test for pulm HTN
Treatment
Raynauds:
- smoking cessation
- CCB (Nifedipine), alpha blockers, topical nitrates
- iloprost infusions for ischaemia
Oesophageal symptoms
- PPI
Malabsorption/bacterial overgrowth
- antibiotics
ILD
- may improve with Cyclophosphamide (9 months)
Pericarditis/ inflammatory myopathy, early ILD - steroids
Renal
- ACE inhibitor to manage BP aggressively to prevent crisis
Pulm HTN
- phosphodiesterase 5 inhibitors - Sildenafil
- endothelia recepto antagonists - Bosenten
- Prostanoids - inhaled Iloprost
PT - hand exercises to improve mobility
OT - aids to asses function
Scleroderma
establish whether limited (to elbows +/- face) or diffuse (above elbows and involving chest/organs)
SKIN: Calcinoshs, Raynaud’s, Sclerodactyly, Telangiectasia
- ischaemia of digits
JOINTS: asthropathy in rheumatoid distribution, CTS
GI: dysphagia, heartburn, diarrhoea (malabsorption, bacterial overgrowth), incontinence
RENAL: HTN, CKD, renal crisis
RESP: ILD (diffuse), Pulm HTN (limited), pleurisy
CARDIO: pericarditis, arythmies, CHF
other: erectile dysfunction, hypothyroidism
ASSESS FUNCTION
Investigations:
ESR/CRP raised
FBC - anaemia chronic disease, bleeding oesophagi’s
Iron/B12/folate - malabsorption
Renal
Anti-Scl 70 - positive in a MINORITY of diffuse
- associated with ILD
Anti-centromere AB - 70% with limited (CREST)
Ix of malasoprtion and dysphagia
- OGD, oesophageal manometry
Monitoring for ILD and pulm HTN as early Mx prevents complications
- PFTs, HRCT for ILD
- Echo, R heart cath, 6 min walk test for pulm HTN
Treatment
Raynauds:
- smoking cessation
- CCB (Nifedipine), alpha blockers, topical nitrates
- iloprost infusions for ischaemia
Oesophageal symptoms
- PPI
Malabsorption/bacterial overgrowth
- antibiotics
ILD
- may improve with Cyclophosphamide (9 months)
Pericarditis/ inflammatory myopathy, early ILD - steroids
Renal
- ACE inhibitor to manage BP aggressively to prevent crisis
Pulm HTN
- phosphodiesterase 5 inhibitors - Sildenafil
- endothelia recepto antagonists - Bosenten
- Prostanoids - inhaled Iloprost
Lung transplant
Indications: COPD (most common) IPF CF Alpha1antitrypsin Pulmonary hypertension
Median survival:
single lung - 4.5 years
double lung - 6.5 years
Causes of death:
Early - primary graft failure (ARDS, DAD)
Late - chronic allograft dysfunction caused by bronchiolitis obliterans (occurs in 50% at 5 years)
Infection
Cancer (skin, PTLD, colon, breast)
Lung function improves over 1-3 months then stabilises
Immunosuppression is typically 3 drug regime with CNI, anti-metabolite and prednisone
Prophylaxis
- cotrimoxazole for PCP
- voriconazole/amphotericin B for aspergillus/fungal (not universal)
Causes of SOB in transplant:
- infection
- bronchial stenosis
- tracheobronchomalacia
- anastomotic site infection
- underlying lung disease
- bronchiolitis obliterans (can use azithromycin to treat)
Heart transplant
Median survival - 11 years
Causes of death:
- Graft failure
- Opportunistic infection
- Acute allograft rejection
- Cardiac allograft vasculopathy
- Lymphoma/malignancy (most frequent after 5 years)
Immunosuppression
- typically 3 drug regime but can wean steroids
Surveillence
- annual angiography or dobutamine stress ECHO for first 5 years to assess for cardiac allograft vasculopathy
then ongoing depending on risk
To treat cardiac allograft vasculopathy
- statin
- diltiazem (controversial)
- sirolimus
- PCI (not usually amenable)
Liver transplant
Survival: 70% at 5y
Immunosuppression:
Typically with steroid, anti-metabolite, and CNI but steroid can be weaned
Prophylaxis
- Cotrim for PCP
- Acyclovir for HSV, VSV
- Hep B - immunoglobulin and antivirals
Monitoring
- monthly LFTs
Reasons for raised LFTs post transplant:
- rejection
- ischemic insult
- biliary complications
- infections (viral heptatitis and sepsis)
- drug toxicites and hypersensitivites
Complications:
- primary non function of graft
- hepatic artery stenosis
- portal vein stenosis
- vena cava stenosis
- anastamotic leak
- biliary stenosis
- infection
- rejection (chronic bile duct destruction)
Diseases that can recur in graft
- hepatitis B and C
- cholestatic diseases (PSC, PBC)
