The misfits Flashcards
Antisynthetase syndrome
Rare inflammatory muscle condition that is closely associated with dermatomyositis and polymyositis. The kicker –> Interstitial lung disease, and the give away in questions. Anti-Jo 1 antibody
Prognosis is based on pulmonary disease
ECOG Scale
0 = fully active 1 = restricted but ambulatory and light work 2 = ambulatory and capable of all self care - but requires rest ~50% of the day 3= limited self care: bed or chair more than 50% 4= completely disabled, cannot self care 5 = dead
Granuloma annulare
Mediated by TNF alpha. Often occurs in areas that are knocked.
Disseminated form with mauve coloured patch associated with HIV.
Necrobiosis lipoidica
Rare disorder affecting the shin of insulin dependant diabetics. Can be round oval shaped or irregular
Hypersensitivity syndromes
Type 1 = Allergy, Allergen - IgE interaction with degranulation of mast cells
Type II = cytotoxic hypersensitivity
Type III = immune complex hypersensitivity
Type IV = Cell mediated
Hypersensitivity syndromes primary mediators
1 = IgE, 2 = IgG/M, as with 3, 4 CD4 and CD8
Type 1 hypersensitivity mechanism
Allergen-specific IgE antibodies bind to mast cells via their Fc receptor. When the specific allergen binds to the IgE, cross-linking of IgE induces degranulation of mast cells.
Type 2 Mechanism hypersensitivity
IgG or IgM antibody binds to cellular antigen, leading to complement activation and cell lysis. IgG can also mediate ADCC with cytotoxic T cells, natural killer cells, macrophages, and neutrophils.
Type 3 hypersensitivity mechanism
Antigen-antibody complexes are deposited in tissues. Complement activation provides inflammatory mediators and recruits neutrophils. Enzymes released from neutrophils damage tissue.
Type 4 hypersensitivity mechanism
Th2 cells secreted by cytokines which activate macrophages and cytotoxic T cells.
Type 1 hypersensitivity examples
Asthma, rhinitis, atopic eczema
Type 2 hypersensitivity examples
Rhesus incompatibility, transfusion reactions, cell destruction due to autoantigens, drug induced haemolysis
Type 3 hypersensitivity examples
GN, SLE, Farmers lung, vasculitis
Type 4 hypersensitivity
Tuberculin reaction, granuloma formation, allergic contact dermatitis, type 1 diabetes
Hypersensitivity reactions mnemonic
“ACID”
The “A” stands for Allergic/Anaphylactic (Type I), the “C” stands for Cell-triggered (Type II), the “I” is for Immune Complex Deposition (Type III), and the “D” stands for ‘Delayed’ (Type IV).
hypersensitivity examples mnemonic
AnGST –(as in, you’d probably feel angst about someone pouring acid on your skin.) A is for “anaphylaxis, asthma, atopy”, G for “Goodpasture’s disease, Grave’s disease, and myasthenia Gravis”, S for “Systemic lupus erythematous and serum sickness”, and T for “T-cell mediated diseases like the TB test and transplant rejection”.
Cardio/circulatory changes in pregnancy
Blood volume increases to 50% of that of normal circulating volume (by week 32)
Stroke volume initially increases to compensate, but later in the pregnancy it is in fact the heart rate that augments.
Pulse pressure widens as systemic vascular resistance falls
Purpose of gemfibrozil
Increase HDL levels and decrease plama triglyceride levels
MOA is unclear ? Stimulates lipoprotein lipase activity via peroxisome proliferator activated receptor alpha (PPARa)
Targets for cholestrol/LDL levels
total Chol <4 HDL >1 LDL <2 Non-HDL-C <3 TG < 2 `
Statin MOA
Competitively inhibits enzyme COA-reductase
Increase hepatic uptake of cholesterol from blood, reduce LDL, modestly reduce triglycerides and increase HDL
Ezetemibe MOA
Reduces absorption of dietary and biliary cholesterol.
This leads to upregulation cellular LDL receptors on the surface of cells and increased LDL cholesterol uptake into cells.
NAGMA
HCO3 loss and replaced with Cl- -> anion gap normal
if hyponatraemia is present the plasma [Cl-] may be normal despite the presence of a normal anion gap acidosis -> this could be considered a ‘relative hyperchloraemia’.
USEDCRAP
Ureteroenterostomies Small bowel fistula Excess Chloride Diarrhoea Carbonic anhydrase inhibitors Renal tubular acidosis Addisson’s disease Pancreatoenterostomies
What is a normal anion GAP
4-10
Commonest organism implicated in septic arthritis
Although Staphylococcus aureus is the most common cause of monomicrobial septic arthritis overall, among sexually active adults, N. gonorrhoeae is the most common causative organism
Congenital deficiency leading to recurrent Neisseria infections
Complement deficiency of C5,6,7,89, i..e can’t form MAC
How to differentiate inflammatory vs. infective synovial fluid
WBC count - typically >2000 in inflammatory but greater than 20,000 in septic
Non-inflammatory will be less than 2000
How to differentiate gout from pseudogout on crystal analysis
Monosodium urate crystals are brightly birefringent and are needle shaped
Calcium pyrophosphate crystals have a rhomboid shape or rectangle they are WEAKly birefringent.
I.E. the same as ‘pseudo’ gout they are ‘pseudo’ refringent
As a random tidbit - if lipids are seen ‘a maltese cross sign’ then suspect fracture
Place to do knee joint aspirate as per UpToDate
Medial epicondyle and patella as landmarks then aim postero-inferiorly
Primary signs of hyperaldosteronism
Hypertension and hypokalaemia +/- metabolic alkalosis
Low Renin and High aldosterone doesn’t clinich the diagnosis of primary hyperaldosteronism unless one of three factors is also present
The exception to the requirement for confirmatory testing is the patient with:
- Spontaneous hypokalemia
- Undetectable PRA or PRC
- PAC 555 pmol/L
Ratio of Plasma aldosterone concentration to plasma renin activity
PAC/PRA ratio greater than 20 (depending upon the laboratory normals)
Secondary hyperaldosteronism such as renovascular disease pattern of renin aldo
PRA (or PRC) and PAC are increased and the PAC/PRA ratio is <10
Liddle’s syndrome
The underlying defect is a gain-of-function mutation in the collecting tubule sodium channel.
Autosomal dominant: presents with hypernatraemia and hypokalaemia
HLA-A3 association
Haemachromatosis
HLA B51 association
Bechets disease (positive pathergy test)
HLA B27 positive association
Ank spondylitis
Reiters syndrome
Acute anterior uveitis
HLA Dq2-DQ8 association
Coeliac disease
HLA-DR2 association
Good pastures and Narcolepsy
HLA DR3 association
Dermatitis herpetiformis
Sjogrens syndrome
Primary biliary cirrhosis
HLA DR4
Type 1 diabetes
Rheumatoid arthritis in particular DR B1
Burkitts lymphoma summary
8,14 mutation associated with c-myc. B cell malignancy with a strong association with EBV. Lymphoma with high risk of tumour lysis syndrome - therefore rasburicase given pre-chemotherapy. Its MOA is increasing the solubility of uric acid to allantonin
Procedure that specifically do not require antimicrobials when you think they may
Genitourinary and gastrointestinal procedures. Cesarean and normal delivery also not required.
Oral regimens for endocarditis prophylaxis
Amoxicllin 2 g if not penicillin allergic
Cephlex, clinda or azithro if allergic
Typical history for acute closed angle glaucoma
Painful red eye, halos around lights, decrease in vision, headaches
Usually triggered by rapid pupillary dilatation i.e. in dark room.
Aqueous humour can’t escape and builds up between the iris and the cornea.
Iritis aka anterior uveitis typical history
‘Ciliary flush’ may be seen - this is a red ring around the iris.
Consensual light reflex exhibits pain. i.e light in opposite eye.
Infection, autoimmune disease and inflammatory conditions can all predispose.
Keratitis (i.e. corneal iritation) typical history
Can be bacterial or viral. Bacterial typically use contacts. Typically there is a corneal opacity. Herpes simplex keratitis appears as a serpigenous or dendritic ulcer on fluroscein
Scleriritis typical history
Severe boring pain to eye. Red, inflamed. Worse with movement. Nodular sclera. COMMON WITH RHEUMATOID
IBD
Granulomatosis with polyangiitis
Causes of a painless red eye
Blepheritis, subconjunctival haemorrhage, conjunctivitis, episcleritis, keratoconjuntivitis sicca
Alcohol cessation drugs and reasoning
A brief intervention - even as short as 5 minutes has valid data in reducing alcohol use.
If pharmacotherpy is needs: “Remember Alcohol N Drugs”
A = Acamprosate: assists with anxiety, use in cirrhosis (except C), can use in heart disease
N = Naltrexone: Once daily, can use in heart disease and caution in renal disease (seek renal advice if Cr >120)
Disulfiram = Once daily, can’t use if complex medically (heart disease, liver disease, renal disease)
Site of action of all diuretics
Carbonic anhydrase - NaHCO3 reabosorption in prox tubule
Thick ascending loop Na + K + Cl = loop diuretics
Thiazides in distal tubule on Na - Cl
K sparing/aldosterone blockers Na + K + H collecting duct.
4 Main renal stone types and causes
Calcium stones = hyperparathyroid, renal calcium leak, hyperoxaluria (short gut or rhubarb), hypomag, hypocitraturia
Uric acid stones: pH less than 5, high intake of purine foods. Associated with gout
Struvite stones: gram neg urase positive organisms (proteus, klebsiella– NEVER E.Coli)
Cystine - intrinsic metabolic defect causing failure of renal tubules to absorb Cystine, lysin, ornithin, arginine.
Best test for renal stones
CT non contrast KUB/abdo pelvis
Over 5mm unlikely to pass on their own
Medical therapy post stones
Uric acid : lower with allopurinol is implicated. Also can make urine more alkaline with sodi bic.
Thiazide diuretics are recommended for patients with recurrent stones.
Cystine with D penicillamine and alkalinization.
Which stones are radiolucent
Cystine are faint
Urin acid - completely radiolucent.
Likewise stones formed by indinanir (ICU is the mnemonic)
Thiamine deficiency overview
Wet or dry beri beri:
Lack of thiamine or Vitamin B 1
Stores typically only last 1 month.
Important co-enzyme in the formation of glucose, acetyl-CoA and decarboxylation of alpha ketoacidosis
Typical of alcoholics, starvation. Can be from decrease absorption, severe liver disease and also with folate deficiency.
Dry beri beri: bilateral sensory and motor neuropathy and weakness with hyporeflexia.
Wet beri beri: tachycardia, vasodilatation, high cardiac output, fluid overload
Wernicke’s : alterned mental state, opthalmoplegia (horizontal nystagmus), ataxia and altered vestibular function
Korsakoff: chronic, memory loss and confabulation
Rheumatoid arthritis and lung disease: most common finding
Pleural effusion
Lung disease is second to infection as a cause of death in patients with RA.
Predictor of lung disease development in Scleroderma
Systemic sclerosis typically is associated with a pulmonary fibrosis ion a UIP pattern. The presence of SCL-70 antibodies is a strong predictor of lung fibrosis/lung involvement
Anti-centromere antibodies are protective.
Typical cause of lung damage in polymyositis/dermatomyositis
Lung involvement does not primarily involve the pleura but instead typically is secondary to aspiration pneumonia from pharyngeal muscle weakness.
There is often also diaphragmatic elevation and decreased lung volumes.
If possessing anti-Jo-1 antibodies however this points toward chronic bronchioilitis /antisynthetase syndrome.
Sjögrens syndrome lung involvement
Upper airways are bried. Often mucous plug inspiration can not be mobilised for ejection. Most commonly a pulm. fibrosis also.
Mixed connective tissue disease definition and lung involvement
Typified by overlaps with SLE and SSc and PM.
Anti-U1-RNP is typical of this disease. Often lung disease is not a strong feature
Churg-strauss lung disease
Also now called Eosinophilia granolomatosis with polyangiitis
Almost exclusively affects patients with a history of asthma.
Lungs are the most common organ involved. Allergic rhinits, eosinophilia and fever all point towards it.
Upper lobe predominant bilateral pulmonary pathologies
SET CAP Silicosis and sarcoid Extrninsic alveolitis and Eosinophilic granuloma TB/fungal CF Ank spond PCP
Lower lobe predominant bilateral pulmonary pathologies
Bronchiectasis Aspiration DIP Rheumatoid Asbestosis Scleroderma Hamman-Rich syndrome aka acute interstital pneumonia
Which immunosuppressive drug most increases your risk of skin cancer ?
Azathioprine
Rasburicase MOA
Catalyses Poorly soluable uric acid into allantoin with CO2 and H2O2
Allopurinol MOA
Xanthine oxidase inhibitor
Purine Catabolism pathway
Purine –>hypoxanthine –> xanthine –> uric acid –> allantoin (via urate oxidase)
Mnemonic for seizures/first seizures
SICK DRIFTER
Substrates (Sugar, oxygen)
Isoniazid OD
Cations (Na, Ca, Mg)
Kids (Eclampsia)
Drugs (Crap = Cocain rum (alcohol), amphetamines, PCP)
Rum
illness (Chronically well mx seizures)
Fever
Trauma
Extra (TAIL = Theophyline Aspirin Isoniazid Lithium, anti-histamine, antidepressant, anticonvulsant (level low or high) Withdrawal (benzo)
Rat Poison (Organophosphate poinsoning - not actually rat poison)
Clozapine side effects
Agranulocytosis, seizure, myocarditis
Mirtazepine important side effect
Agranulocytosis
Lithium side effects
Nephrogenic DI, tremor, hypothyroid, hyper PTH
EBSTEINS ANOMALY if taken in the first trimester
Valproate important side effects
Transaminitis, thrombocytopenia, neural tube defects
Two different types of pneumococcal vax and timing in asplenia
PCV13 (conjugate) and PPSV23 (polysaccharide) are both given 8 weeks apart (13 first then 23)
Vaccinations recommended in asplenia and restrictions
Hib, Pneumococcus (S.Pneumo) and meningocuccs (N.Meningiditis) + Seasonal influenza
No restrictions on other vaccines (live)
Two types of meningococcal vaccination
MenACWY (like YMCA but upside down back to front) and MenB
Live attenuated vaccines
My Big Monster 'Ruck TIRES, Your crazy chicken Measles, BCG Mumps Rubella Typhoid Influenza Rotavirus Endemic typhus Sabin (oral polio) Yellow fever Chicken pox Cholera
