RHEUMATOLOGY Flashcards

Question

How might SLE present Think: Multi system ``` Cutaneous SLE. Neuropsychiatric SLE. GN SLE ? transplanted / PD scars? Heart failure - libman sacks endocarditis ``` Assoc: Autoimmune ovaarian failure - secondary amenorhhoea recurrent miscarriages -antiphospholipid

Answer 1

-Arthritis non deforming jaccouds arthritis -alopecia + mouth ulcers scarring or non scarring -opthalmoplegia or CN lesions mononeuritis multiplex -Malar butterfly rash or systemic discoid rash -Haematuria Lupus glomerulonephritis -VTE + PE / stroke / MI / Retinal A occlusion early onset Antiphospholipid syndrome RECURRENT MISCARRIAGE + PRE-ECLAMPSIA -Anaemia haemolytic anaemia / leucopenia / thrombocytopenia -Cardiac NON INFECTIVE ENDOCARDITIS LIBMAN SACKS ENDOCARDITIS - AR MR CCF -Neuro Psychosis / GBS / seizures -Myositis MIXED CONNECTIVE TISSUE DISEASE OVERLAP ``` - SOB Pericarditis Pleural effusion (transudative) PE pulm fibrosis GBS Reaction to biologics - as ARDS ``` -Cushingoid steroids

Answer 2

APICAL work: silicosis coal pneumoconiosis Autoimmune Sarcoid and other connective tissue disease ank spond infective Allergic aspergillosis TB metabolic Histiocytosis X iatrogenic Radiation BASAL Autoimmune RA MCD - sjorgens / SLE / scleroderma / dermatomyositis / polymyositis Idopathic IPF - AIP / DIP / infective bronchiectasis

Answer 3

Primary or Secondary - usually Secondary AI process which destroys exocrine glands Usually secondary to another AI process like RA or SLE Primary Sjorgens is associated with a 40fold increased risk of Lymphoma Due to parotid involvement differentials of swelling should include: - Parotid cancer - Lymphoma - Sjorgens - Mumps - parotitis - sarcoid

Answer 4

``` Dry eyes - XEROPHTHALMIA Dry Mouth - XEROSTOMA Bilateral parotid enlargement (blocked) -LOOK FOR EXCISION SCARS UNDER JAW LINE non erosive small joint arthropathy ```

Answer 5

Test for lacrimation when sjorgens suspected litmus paper crimped and hooked inside lower eyelid for for 5mm expect to see tear migration >15mm = normal

Answer 6

1. History 2. Hx of CTD - Ra or SLE 3. Abs - anti Ro / La RF and anti-Ds DNA may also be positive 4. Salivary gland biopsy is conclusive

Answer 7

Anti nuclear antigen is +ve in 95% at >1:320 Anti phospholipid may be present Anti-Ds DNA if present good for monitoring activity Anti-ENA may also be present -Sm / Ro / La (THINK IS 2dary SJORGENS PRESENT)

Answer 8

1. Pred long term - may be cushingoid as in RA / PsA - steroid induced DM2 ``` 2.DMARDS MTX hydroxychloroquine MMF Cyclophosphamide (esp lupus nephritis) ``` 3, biologics Rituximab - anti cd20 IVIg for flares plasmaphoresis 4. anticoag if antiphospholipid Warf - antag vit K Rivarox / apix / 5. renal biopsy ACEi for protein urea transplant 6. TAVI 7. iron for haemolytic anaemia 8. physio for jaccouds arthropathy 9. specialist stroke mi / vte pulm fibrosis - apical OR if secondary to MTX basal pleural effusion - Percutaenous drain / pleurodesis Pericardial effusion - pericardiocentesis 10 cosmetic Wigs if scarring alopecia 11. counselling support groups PT and OT input

Answer 9

Conveying exact genes for SLE is rare increased risk compared to background population medication counselling STOP MTX as teratogenic STOP WARF as teratogenic STOP Carbemazepine as teratogenic azathioprine and hydroxychloroqione ok

Answer 10

1. Urine - protein and blood / casts / biopsy 2. Anti-Ds DNA - levels correlate to disease activity 3. FBC - cytopenias 4. LFTs - Transaminitis 5. Low C3 low C4 6. haematuria

Answer 11

1 Immunosuppresion Reactivation of latent TB / HCV / HBV / CMV / EBV Acceleration of undiagnosed HIV Opportunistic infection due to BM suppression PCP CMV or EBV or HTLV-1 THEREFORE PROPHYLACTIC COTRIMOX 2.Small risk of increased cancer. 3. Hemorrhagic cystitis TAKE ORAL MESNA PRIOR TO INFUSION Conjugates toxic urinary metabolites to reduce the risk. 4. infertility

Answer 12

Systemic form of scleroderma Multi organ involving CTD characterised by skin thickening and joint deformity CREST no longer really applies therefore: 1. systemic sclerosis - CREST 2. limited cutanoues - not all CREST fulfilled - e.g. gastroenteropathy + pulm fibrosis + morphoea Look for - small tight mouth / beaked nose - tight shiny skin - sclerodactyly deformity - morphoea - Limited cutaenous SS or DcSS - calcium deposition - CREST - ulcers over fingers - raynauds - hair loss and scarring alopecia - telangiectasia (CREST) artificial tears / saliva = Sjorgens Remember CTD do cross over -SLE may also feature here (RASH G-ANA) rash malar discoid photo / apthous / serositis / haemolytic / GN / ANA / Abs / Jaccouds arth) -E.g MCTD - SLE + scleroderma + myositis 3 presentations: Raynauds Localised scleroderma aka morphoea linear scleroderma well circumscribed Tight hard skin en coup de sabre - scalp scerloderma Systemic Sclerosis - 3 types -Limited cutaeonus systemic sclerosis =any non CREST Diffuse morphoea face / below elbows and knees but spares trunk - typically get pulm fibrosis and gastroenteropathy Pulm fibrosis types - usually SCL70 +VE - CREST - term no longer used (true systemic sclerosis) Calcinosis - isolated hypercalcaemic deposits not unlike sarcoid granulomas (due to ectopic a1 hydroxylase production) Raynauds Eosphagial dysmotility / dilatation / achalasia Scleroderma / sclerodactyly Telangiectasia Dysmotiity extends to all bowel - bacterial overgrowth / malabsorption / weight loss and diarrhoea ``` pulmonary fibrosis -->p.HTN and CCF UIP - usual int pneumonia = crap - 2.5-5 years NSIP = non specific int pneumonia GOOD STEROID RESPONSE MORT 12-14 years ``` -Diffuse cutaenous systemic sclerosis Whole body morphoea SCL70 +VE - risk of severe ILD Anti-RNA pol 3 RISK OF SCLERODERMA RENAL CRISIS - in event of renal crisis treat BP with RAMIPRIL - Better outcome mortality - 76% survival at 1 year Vs 15% - amlodipine is good for BP control regarding raynauds but does not alter mortality in renal crisis

Answer 13

Localised thickening of fingers and toes DISTAL TO MCPJ INVOLVEMENT POST MCP IS BEYOND SCLERODACTYLLY AND IMPLIES MORE DIFFUSE INVOLVEMENT

Answer 14

``` SLE (RASH GANA) rash in the sun? Do you have any rashes? Joint pains - jaccouds SOB / chest pain Haematuria mononeuritis multiplex / gbs /psychosis / confusion apthous ulcers ``` Antiphos stroke / mi / PE / VTE DM rash - heliotropic muscle weakness / pain? Features of SLE and Slceroderma Sjorgens dry mouth dry eyes joint pains - jaccouds CREST Skin changes when cold ? painful? ?ulcers? Do you have difficulty swallowing / heartburn? DIARRHOEA /DYSPHAGIA / DYSPEPSIA

Answer 15

ONSET IN DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS - associated Anti RNA pol 3 - associated with SCL70 = diffuse ILD - Acute onset malignant HTN leading to acute renal failure / headache // blurred vision , seizures, CCF AMD microangiopathic haemolysis

Answer 16

Autoimmune process and associated with , CREST, SLE, malignancy (breast lung gynae / colorectal) Causes degernation of collagen resulting in - heliotropic (surrounding eyes) rash - proximal muscle weakness - GOTTRON's PAPULES over DIPJ / MCPJ - violaceous papules Abs: Anti Jo onset 40-60 more common in women

Answer 17

1. topical steroids 2. anti-histamines 3. daily emollients 4. topical calciuneurin inhibitors for refractive disease

Answer 18

DMARDS MTX - terato Leflunomide - terato azathioprine - shown to be safe but not recommended SULFASALAZINE and HYDROXYCHLOROQUIN are the current recommendation TNF-a inhibitors - inflix / adalimumba and golimumab have been shown to be safe but are not yet recommended due to lack of longterm evidence

Answer 19

- glossitis - candidiasis - macrocytic anaemia - thrombocytopenia - severe mouth ulcers --> FOLINIC ACID More efficacious than folic acid 75mg over 12 hours 6-12mg every 4 hours thereafter If severe Bicarbonate fluids Dialysis does not remove mtx

Answer 20

Widespread pain for >3 months Pain elicited by pressure of 4kg/cm2 in 11 areas Psychological stress = > pressure points Overlaps substantially with depressive disorders / anxiety and chronic fatigue overlaps with regional pain syndromes - atypical CP / IBS / chronic pelvic pain / TMJ pain

Answer 21

1. psychotherapy 2. pharmacotherapy for disordered sleep = tricyclics / melatonin / zopiclone 3. graded exercise programs

Answer 22

Polyarticular or monoarticular gout - single or multiple hot effused joints - restricted ROM due to pain - attacks last 3-10 days - first presentation monoarticular - big toe / ankle / foot arch - 50% onset hallux-->90% involvement - Diurnal variation - onset in night and worst in morning - Olecranon bursitis - scars from ulceration and sinus formation over joints -Aspiration - needle shaped intracellular and extracellular crysals of monodosium urate yellow crystals-->red aligned parrallel-->blue when aligned perperndicular (NEGATIVE BIREFRINGENCE) ``` PSEUDOGOUT: Calcium pyrophosphate (CPP) - short / rhomboidal - no colour change underpolarising filter ``` ``` TOPHI: ACHILLES BEHIND PINNA ELBOWS FINGERS / TOES EXTENSOR FOREARM ALONG TENDONS ``` PODAGRA - foot torturer in greek mythology HALLUX GOUT Caused by precipitation of urate crystals within the joint space causing localised inflammation and pain with restricted movement Gouty arthritis is the most common for of arthritis in men over 40 4x more common in men Prevelance rises with age and serum urate concentrations ``` 4 PHASES 1 Asymptomatic Hyperuricaemia 2 Acute Gout 3 Intercritical Gout 4 Chronic tophaceous gout ``` ``` triggers: alcohol purine ingestion (shellfish)- concurrent azathioprine use will require AZA reduced dose will need to be considered to maintain IBD control and avoid excessive BM suppresion UGIB and ingestion of digested blood sepsis trauma dehydration ``` ``` drugs: TZDs - LOW POTASSIUM aspirin theophyllin ciclosporin levodopa ethambutol ``` ``` Risk factors CKD hypothyroid myeloprolif / lymphoprolife: high turnover DM DI hereditary hyperuriacaemia Sarcoid Psoriasis obesity ``` Allopurinol is a xanthine oxidase inhibitor which would normally facilitate breakdown of 6'mercaptopurine (active form of AZA and mercaptopurine) (also inhibits TPMT) - Therefore in IBD can use a low dose of thiopurine analogue and supplement wit allopurinol to have clinical effect Mx: Colchicine / pred / NSAIDs (naproxen NOT IBUPROFEN) in the acute setting Allopurinol or febuxostat (XOI) after symptoms resolve for secondary prevention RASBURICASE - recombinatn urate oxidase Avoid precipitants - alcohol / caffeine / cheese

Answer 23

Mx: Colchicine / pred / NSAIDs (naproxen NOT IBUPROFEN) in the acute setting Allopurinol or febuxostat (XOI) after symptoms resolve for secondary prevention RASBURICASE - recombinatn urate oxidase Avoid precipitants - alcohol / caffeine / cheese

Answer 24

Indicative of dermatomyositis

Answer 25

Spondyloarthritis presenting with triad: urethritis / dysuria uveitis arthritis Causes E.Coli / shigella / salmonella Gastroenteritis Chlamydia trachomatis Chlamydia pneumoniae Psoriatic like rash = chlamydia Treat = cause

Answer 26

CTS results from median nerve compression in the wrist affecting the median nerve distribution - Thumb movements except flexor pollicis brevis - thenar wasting - 1st and 2nd fingers - Tinnels and phalens sign positive ``` Associations: RA Hyperthyroid Hypothyroid Idopathic ``` Mx: local consticosteroid hand splint median nerve decompression EMG studies to confirm

Answer 27

Needle shaped negative birefringence crystals yellow at base aligned to light = red perpendicular to light = blue WCC +++ no growth

Answer 28

calcium pyrophosphate crystals | no birefringence

Answer 29

Clubbing | periosteal inflammation i.e. effusion similar to dactylytis but involves whole fingers

Answer 30

Hypertrophic pulmonary osteoarthropathy

Answer 31

global joint pain | proximal weakness

Answer 32

Results in Charcot's joint - TYPICALLY PAINLESS DUE TO SENSORY PARAESTHESIA AND IMPAIRED PROPRIOCEPTIVE FEEDBACK - GROSS DEFORMITY XRAY - loss of joint margin - bony destruction - significant and active remodelling Rx: immobilisation for 3-4 months bisphosphonates IRREVERSIBLE ONCE ESTABLISHED ``` Causes: DM Charcot Marie tooth - hereditary sensory and motor neuropathy - demyelinating / schwann cell damage - loss of sensation - pes cavus or pes planus - CMT is divided into the primary demyelinating neuropathies (CMT1, CMT3, and CMT4) and the primary axonal neuropathies (CMT2), with frequent overlap. ```

Answer 33

Allopurinol is a xanthane oxidase inhibitor XO converts 6 mercaptopurine to inactive metabolites Inhibition of XO results in build up of active component 6 mercaptopurine i.e. dose seen by tissue is much greater than dose prescribed.

Answer 34

Immunoglobulins that precipitate out of solution when exposed to cold temperatures causing microvascular complications - purpuric rash - polyarthralgia - polyarthritis - Raynauds Abs: ANA RF - 90% present in Sjorgens and cryoglobulinaemia low C3 - immune complex deposition

Answer 35

Remember pulmonary sarcoid will cause bilateral hilar lymphadenopathy and basal pulmonary fibrosis ``` P.HTN is a significant cause of morbidity and MORTALITY -core pulmonale severe = PA pressure >40mmHg -PR -TR -peripheral oedema -OSA -T2RF -impaired ET ``` Treatment for sarcoid = improve QOL and slow progression 1. Pulmonary rehab 2. stop smoking 3. Sildenafil to reduce Preload 4. iloprost infusion Longterm: Pred MTX

Answer 36

``` Presentation: 40M Skin pigmentation Diabetes Heart problems CLD Arthropathy ``` ``` Arthropathy is due to CHONDROCALCINOSIS CALCIUM PYROPHOSPHATE DEPOSITION i.e. pseudo gout / chondrocalcinosis / chronic arthropathy Rhomboid crystals may be weakly positive birefringence ``` -High transferrin saturation >15% raised serum ferritin >500

Answer 37

scalp i.e hairline gluteal cleft naval flexor tendon synovitis

Answer 38

Usually female Usually monoarthritis Usually glenohumeral joint aspirate: blood hydroxyapatite crystals VERY DESTRUCTIVE ARTHRITIS

Answer 39

Treatment should continue through pregnancy especially given the immuno changes that come i.e. tolerance MTX / Leflunomide = teratogenic Sulfasalazine = FIRST CHOICE Azathioprine = Safe but not first choice Infliximab = data shows no increased morb / mort but not yet approved by NICE for pregnancy

Answer 40

RA + splenomegally +neutropenia 50s female longstanding RA >10 years Think as a differential in a patient with RA on MTX i.e. not simply MTX tox = folinic acid resuce ``` Rx: pulse methylpred cyclophos switch DMARD - leflunomide / sulfasalazine / azathioprine SPLENECTOMY IF FAILS TO RESPOND ```

Answer 41

Manifests as EEA - Driver is allergen to aspergillus - Raised eosinophil count - Th2 response - Raised aspergillus IgG ``` Aspergillus can seed in old TB cavities Can become invasive aspergillus Pulmonary haemorrhage Form a gravity dependent aspergilloma pulmonary fibrosis - apical Can also cause bronchiectasis due to chronic poor drainage ``` (CAUSES Bronchiectasis = TONNES - CONGENITAL = CF / YOUNGS (middle Age + bronchiectasis, rhinosinusitis and reduced fertility. / A1 ANTI-TRYPSIN / Kartageners - Primary ciliary dyskinesia / TOXIN - Recurrent aspiration / COPD / Traction bronchiectasis (pulmonary fibrosis splints open airay) / IMMUNO - HIV +LEUKAEMIA +SCID /MECHANICAL - obstruction / carcinoma / granuloma - sarcoid wegners tb ) ``` Treatment Fostair - formeterol / fluticasone OR Seretide - salmeterol / fluticasone Pred for wheeze if active ITRACONAZOLE pulmonary rehab ``` SLOW PROGRESSIVE IN MOST CASES

Answer 42

BUPRIOPION AND VARENICLINE single agent = 20-30% cessation double agent up to 44% cessation Willpower alone = 2-4% cessation nicotine replacement lozenges / gum = 10%

Answer 43

Bilateral crackles pyrexia and T1RF leukopenia CXR: interstitial shadowing basal cystic changes CYSTS AS ANY BULLAE TYPE DISEASE AN RUPTURE CAUSING PTX - 10%

Answer 44

AIDS defining illness CD4<200 also: SCID / any immunosuppressed ``` Diagnosis: History CXR - interstitial shadowing + cystic lower zone changes BAL - fungus immunofluorescence and BAL ```

Answer 45

long term prophylaxis: Co-trimoxazole HIV ART/HAART compliance and CD4>200 Acute: Prednisolone Co-trimoxazole

Answer 46

Humeral head --> almost all humeral neck fractures Femoral head --> intracapital fractures Scaphoid SLE presentation Chronic and intractable hip pain 90% progress to bilateral <12 months Antiphospholipid Gauchers storage disease MRI best modality Mx: non weight bearing to prevent the colpase of the the femoral head and secondary OA Surgical deompression Hemi Arthroplasty

Answer 47

dermatitis / polyarthritis / tenosynovitis / urethritis Pustulous eruptions Scaly pustular plaques nail pitting single joint disease above others - hot swollen (septic joint) Mx: Ceftriaxone Azithromycin NSAIDs

Answer 48

Benzylpenicillin and flucloxacillin

Answer 49

``` commonest manifestation Cutaeneous rash which is photosensitive +VE ANTI-HISTONE ABS +VE ANA +VE RF -VE ds DNA Normal C3 and C4 ``` i.e appears like inactive lupus but cutaenous photsensitivity causes: HYDRALAZINE SULFASALAZINE CAPTOPRIL RX: low dose steroid stop drug

Answer 50

Anti-RNP antibodies

Answer 51

Granulomatous and necrotising systemic vasculitis of medium and small calibre vessles not associated with raised eosinophils. ``` Presentation: Necrosis of nasal septum and bridge = SADDLE BRIDGE Epistaxis Pulmonary Haemorrhage GN Cavitating chest lesions ``` C-ANCA positive PR-3 (CHURG STRAUSS + microscopic polyangitis =P-ANCA MPO) Immediate Mx: Pulsed IV methyl pred + cyclophosphamide / ritux Biologics: Rituxumab - non inferior to cyclophosphamide Remission rate is only 75% Chronic: Oral pred + steroid sparing DMARD Steroid bridges to get steroid sparing agent to a therapeutic level e.g. MTX Azathioprine or Mercaptopurine (need TPMT level - low TPMT = avoid) Infliximab Prophylactic co-trimoxazole

Answer 52

80% UC is positive for atypicaly ANCA - ANCA-X Vs 20% of CD

Answer 53

Antineutrophil cytoplasmic antibodies Abs directed against neutrophils C and P denote where Ab found C = cytoplasm P = perinuclear MPO or PR3 are the targets C-ANCA is always targeted at PR3 P-ANCA is always targeted at MPO

Answer 54

1 LAT AVR V1 septal V4 ant circumflex LAD RCA High Lat LV ant/septal Ant LV 2 INF AVL: LAT V2 septal V5 Lat RCA circumflex LAD circumflex INF LV High Lat LV ant/septal Lat LV 3 INF AVF: INF V3 ant V6 Lat RCA RCA RCA circumflex INF LV INF LV Ant LV Lat LV

Answer 55

``` Carotid upstroke LVH and heave Reversed splitting on second heart sound - delayed A2 syncope = severe SOBOE poor pulse volume syncope on exertion SVT / Vt secondary to LVD ```

Answer 56

LVF may cause an underestimate of the valve pressure gradient and valve surface due to impaired contraction and impaired force of contraction(FALSE LOW) For this reason it is better to go on : DOBUTAMINE STRESS ECHO Severe AS = rise in trans-aortic pressure on dobutamine stimulation with same valve area i.e. a true estimate If the pressure falls and valve area increases then estimate was likely a false low estimate An increase in EJF of over 20% is associated with a much better prognosis post TAVI

Answer 57

Morphoea is a localised area of skin thickening which can be isolated or form part of the manifestation of limited or diffuse systemic sclersis or CREST (calcinosis / raynauds (ANA / RO / LA) / GI / sclerodact / telangiectasia) ``` Typically linear thick plaques that become atrophic hypo or hyperpigmented over new damaged tissue tight and itchy skin on head - morphoea en coup de sabre white striae ``` Mx: topical or oral steroid phototherapy MTX

Answer 58

Anti-jo ``` onset 40-60 2:1 F associated cancer - lung breast uterine stomach rectum -precedes cancer 40% -concurrent 20% -30% post neoplasm ``` - Gottrons papules - heliotropic rash

Answer 59

Anti-RN pol III increased risk of scleroderma renal crisis REDUCED RISK PULM FIBROSIS Anti-SCL70 increased risk of PULM FIBROSIS Anti-topoisomerase -1 ILD + renal involvement

Answer 60

systemic sclerosis | ILD and renal involvement

Answer 61

limited cutaenous sclerosis RF for P.HTN NOTE ILD AND RENAL INVOVLEMENT VERY RARE IN LIMITED DISEASE

Answer 62

Ramipirl | 76% survival at 1 year Vs 15% without

Answer 63

Amlodipine

Answer 64

Prednisolone + cyclophosphamide or mycophenolate Rtiux no use MTX onset too slow

Answer 65

Regular analgesia and pain relief physio and mobiisation Atni-TNFa = ETANERCEPT / ADALIMUMAB / GOLIMUMAB STEROID INJECTIONS for sacroilitis and enthetesis Bisphosphonates for osteoporosis