inflammatory conditions Flashcards
What is Systemic lupus erythematous ?
Chronic multi system Autoimmune condition - immune attacks healthy body tissue e.g o skin o joints o kidneys o brain o other organs
RISK FACTORS
0 Female sex (particulary during reproductive years)
0 Age - 15-45 years
0 Asian & African descent
0 Drugs - isoniazid,
terbinafine,
phenytoin,carbamazepine,
sulfasalazine (presumed due to its sulfonamide component)
Most common cause of clubbing in the fingers ?
Respiratory causes are the most common cause of this e.g
0 lung carcinoma
0 chronic suppurative lung disease
Suppurative - discharge or production of pus)
(used when referring to paedriatrics
– Cystic fibrosis
–Bronchiectasis (Irreversible dilation of bronchial tree - abnormal) – Primary cillary dyskinesa (congenital defect where cilia are unable to move)
0 cystic fibrosis
0 asbestos exposure
Investigation - chest radiograph
0 can have abdominal cause - less frequent.
Signs / symptoms of SLE ?
Malar / butterfly rash - raised rash across cheeks and bridge of nose .
- Photosensitive rash - appears after sun exposure .
- discoid rash
- fatigue ( found in 80 - 100 % - other SLE symptoms need to be present )
- weight loss
- fever ( present in less 50 % of pl )
0 PL with pre - existing SL with fever treated with immunosuppressants should be checked for opportunistic infections ) - oral ulcers ( 10 -45 % of people )
- alopecia - non scarring
(scarring alopecia - more common in chronic discoid lupus ) - arthralgia ( joint paint ) / arthritis ( normally multi joints )
- monoarthritis - more likely to have infective cause or avascular necosis.
- fibromyalgia - widespread musculoskeletal pain.
- Raynauds phenomenon - colour changes in the tip (Typically - from white to blue to red ) ( occurs in 50 %) - often less severe than Systemic sclerosis )
*- venous / arterial thrombosis
( presence of antiphospholipid increases risk of clots )
- Pleuritis - inflammation of lining btw lungs.
( sharp chest pain upon breathing )
Libman–Sacks endocarditis
( not caused by bacteria I.e. infective endocarditis)
pericarditis
Renal involvement is usually subclinical
- Hypertension - can be caused by underlying lupus nephritis ( inflammation of kidneys by SLE )
abdominal pain , vominting & nausea
- gastrointestinal manifestation - caused by lupus peritonitis or mesenteric artery occulsion.
Diagnosis of SLE ?
0 FBC
o leukopenia - reduced WBC ( immune system destroys them ) - In SLE - lymphopenia (more common ) & neutropenia o anaemia
o thrombocytopenia
(They don’t have to occur )
0 ESR & CRP
0 Activated PTT - (partial thromboplastin time )- Prolonged ( due to antiphospolipid antibodies.
PTT - measures how long it takes for blood clot to form - checks function of coagulation factors.
( also used to monitor pl taking heparin (coagulant medication )
0 Antinuclear anitbody (ANA) - positive - raised
0 Antibodies to dsDNA - raised - postive
0 U & E
0 Urinalysis - assess renal involvement
o Antiphospholipid test
o lupus anticoagulant (type of antiphospholipd antibody found in many people with lupus )
IF cardiopulmonary symptoms present - Chest Xray . ECG
What does a raised CRP usually indicate ?
Raised CRP - usually indicates that there is inflammation caused by infection.
e.g Raised CRP & ESR - infection
Raised ESR - inflammation - non infective.
Mostly ..
What is antiphospholipid syndrome ?
CAPS - meaning ?
Hughes syndrome / Antiphospholipid syndrome (APS )
disorder of immune system - causes increased risk of clots - Autoimmune condition
(production of antiphospholipid antibodies (abnormal ) - attach to fat phospholipid - cause blood to clot.
- greater risk of developing :
o DVT
(superfical thrombophlebitis - looks similar to DVT - but as severe , inflammation of veins under skin (usually in leg).
o arterial thrombosis - can cause High BP , Strokes, Myocardial infarction , Pulmonary emobolism.
o Blood clots in brain - problems with balance , mobility , vision , speech and memory.
(also can develop Livedo reticularis (small blood clots in the BV of skin - skin can become blotchy red or blue. - can develops sores or ulcers as a result. (often more severe in cold weather. )
Livedo - skin discoulouration
AUTOANTIBODIES CAN TARGET BLOOD COMPONENETS
Platelets - thrombocytopenia
RBC - Anaemia
CAPS - catastrophic antiphospholipid syndrome - blood clots form suddenly throughout body causing multiple organ failure
if block renal veins /arteries - lead to renal failure
- emergency , high dose anticoagulants needed.
Diagnosis
Meet Clinical and Laboratory threshold
0 History of thrombosis
0 History of Pregnancy complications e.g miscarriage
LAB
Either presence of ;
ANTI - PHOSPHOLIPID ANTIBODIES
0 Lupus anticoagulant
0 Anticardiolipin (also present in syphilis - leading to false positive test for syphilis)
0 Anti - beta 2 glycoprotein I antibodies
( these prevent excessive platelet aggregation - not the antibodies ex- beta 2 glycoprotein I )
if Anti - nuclear antibodies - suggest underlying SLE.
Treatment of SLE ?
Steriods
Immunosuppressants - Azathiopine , Hydroxychoroquine
joint pain and serositis (inflammation of serous tissue e.g pleura of lungs , heart , periotenium )
0 1st line - hydroxychloroquine
0 Corticosteroids
o Adjunct - methotrexate & folinic acid therapy (help reduce steriod use/ dose )
Mucocnatenous disease - disease of the mouth / skin ?
- sunscreen - SPF > 15 - prevent rashes / skin lesions.
- mouthwashes- Chlorohexidine , dental appointments , oral hygeine.
Lupus nephritis induction therapy - immunosuppression o - cyclophosphamide: - tacrolimus: - mycophenolate mofetil: - ciclosporin: o predisolone o hydroxychloroquine.
induction therapy - first treatments given.
What is lupus nephritis ?
inflammation of the kidney due to SLE .
- commonly presents as nephrotic syndrome.
(proteinuria , hypoalbuminemia , oedema , hyperlipidaemia , lipiduria ( less protein in blood / more fat )
can present as nephritic syndrome - damage to Glomerulus causes blood to be filtered in urine.
Type 3 Hypersensitivity reaction
SLE - half will develop this during course of condition.
can be focal or diffuse
- focal - deposition of antibody - antigen complex in one area
diffuse - in mulple areas / nephrons.
DIAGNOSIS
- Liver biopsy
TREATMENT
Immunosuppressants
Steriods.
What is nephritic syndrome ?
Nephritis - kidney disease involving inflammation , glomerulonephritis - nephritis in the glomerulus.
Glomerulonephritis - inflammation of the glomerulus ( capillaries and basement membrane )
- haematuria - microscopic most common ( only seen under microscope )
- proteinuria
- Hypertension -
reduced GFR + slat and water retention cause this. - oliguria - small amounts of urine - usually happens at later stage (unless renal failure develops early )
if there is a vascultic ( inflammation f the BV )cause.
- nausea
- malaise
- weight loss
- skin rash
- arthralgia
- haemoptysis
post-streptococcal Glomerulonephtotis ( inflammation caused by immune system fighting off Group A streptococcus throat infection / skin )
- abdominal pain
- sore throat - PSGN usually develops 10 days after strept throat / scarlet fever.
(Group A strept cause scarlet fever , strept throat , impetigo )
for a skin infection - PSGN usually develops 3 weeks after.
Fever - infectious cause
Diagnosis of nephritic syndrome ?
Urine analysis & microscopy
0 GFR
0 FBC
0 lipid profile
0 Comprehensive metabolic profile - test 14 substances in the blood - provide info on chemical balance & metabolism
o liver enzymes e.g ALT , ALP , AST - may be related to hepatits virus
o Glucose
o Creatine - elevated - indicates severe/ advanced disease
o Albumin - hypalbuminaemia - indicates nephrotic syndrome instead.
o Billirubin
o Electrolytes + C02
o Total protein etc.
0 Spot urine albumin : creatine ratio.
- > 220 mg / mmol (Elevated 0 - commonly used in diabetes to test renal function,
0 Ultrasound of kidenys.
Treatment of Nephritic syndrome ?
mild disease (e.g isolated haematuria , no or minimal proteinuria , normal GFR )
- treat underlying cause ( treat systemic cause e.g antimicrobials , withdrawal of causative drug. )
- salt restriction may be needed.
Moderate to severe - present with haematuria, proteinuria, and reduced GFR
1st line- ACE inhibitor / angiotensin II RB - to decrease proteinuria
Adjunct - Furosemide - loop diuretic - if still hypertensive
rapidly progressive - plasmapheresis
Steriod
Immunosuppressant
- when renal function is altered - plasmapheresis - urgent
(decision to stop this determined by anti - glomerular basement membrane titres )anti - GBM ) and clincal status.
What is Henoch - Scholein purpura ?
common form of vasculitis (inflammation of the blood vessels )
o effects small vessels
o occurs in childhood
Classic tetrad of symptoms
0 rash
0 abdominal pain
0 athritis / arthalgia , Glomerulonephritis .
or other signs of renal disease.
(progressive nephritis , nephrotic syndrome etc. )
Diagnosis
serum cretaine & electrolyte levels - elevated creatine (indicates renal impairment/ failure )
0 IgA - raised
0 Skin biposy - affected area will show leukocytoclastic vasculitis with IgA deposition.
(leukocytoclastic vasculitis - small BV inflammation )
renal biospy - shows IgA deposition to confirm diagnosis .
Ultrasound abdomen - indicated for severe abdominal pain
ultrasound testicles - if this region is involved.
Treatment
WITHOUT NEPHROTIC RANGE PROTEINURIA OR DECLINING GFR
with joint pain alone
1st line – analgesics
o Paracetamol
o ibuprofen
with severe oedema or scrotal involvement
1st line –
oral corticosteroids
with mild to moderate abdominal pain
1st line –
analgesics and supportive care
with severe abdominal pain
1st line –
corticosteroids
Adjunct –
surgical consultation
NEPHROTIC RANGE PROTEINURIA OR DECLINING GFR
without rapidly progressive nephritis
1st line –
corticosteroids
- IV methylprednisolone sodium succinate for 3 days
- oral pnednisolone after (3 months )
with rapidly progressive nephritis
1st line –
corticosteroids plus immunosuppressants
- azathoprine / cyclophosphaminde
Adjunct –
plasmapheresis
Adjunct –
consultation for renal dialysis or transplant
What is Sjogren syndrome ?
Chronic inflammatory and systemic auto-immune disorder characterised by diminished lacrimal and salivary gland secretion (sicca complex).
P resent with
0 dry eyes (keratoconjunctivitis sicca)
0 dry mouth (xerostomia).
Can occur on it own (Primary )
- Secondary to another auto immune disease - RA , SLE etc.
other signs
- Fatigue
- vasculitis (can present as rash )
- destruction of necks of teeth etc - tooth damage
- there are other less common signs .
Diagnosis of Sjogen’s syndrome ?
Anti RO antibodies e.g anti 60 kD Ro
0 Anti - La
plus other other signs pointing to sjogen’s syndrome .
Schrimer’s test - filter paper plced under eye - less than 5mm of the paper wetted after 5 mins - positive
Treatment of Sjogens sydrome ?
Dry eyes
1st line –
artificial tears alone
2nd line –
ophthalmic ciclosporin drops
ADJUNCTS
0 spectacle eye shields
0 humidifiers
0 punctal plugs or thermal punctal occlusion
3rd line –
cholinergic drug
salivary substitutes alone
2nd line – cholinergic drug
Primary options
- pilocarpine: 5 mg orally three times daily
- cevimeline: 30 mg orally three times daily
(Cholinergic drugs stimulate secretion by exocrine glands.)
ADJUNCTS
salivary substitutes
humidifiers and moisturisers
Musculosketetal problems -
1st line - paracetamol
2nd - NSAIDS
Adjunct - immunosuppression - but 1st & 2nd line preferred safer
corticosteriod - prednisolone
if peripheral neuropathy - IV immunoglobulin.
What is limited cutaeous systemic sclerosis / CREST syndrome / systemic scleroderma?
CREST
type of scleroderma
- Chronic Autommune condition - immune system attacks skin an d internal organs leading to excess production of collagen ————————————>thickened and hard skin & build up of scar tissue (Fibrosis )
also can causes obliteration of BV.
C- calcinosis - formation of calcium deposits in soft tissue
(ex -can be felt as painful bumps under the skin (not always ))
R - Raynauds sydrome
E - esophagael dysmolitily
S - Sclerodactyly - hardening of skin in hand - fingers curl inward like a claw.
(Shiny skin can also occur )
T - telangiectasia - enlarged BV under skin.
swelling of hands & tightness
( can cause carpal tunnel syndrome )
- prayers sign - hand cannot touch each other flat - make an O
Fibrosis of internal organs - can cause organ impairment . 0 oesophagus - dysmolitity & dysphagia 0 Heart - Hypertension 0 Lungs - SOB 0 Kidneys 0 impairment of muscle contractions moving food through GI. - GORD
What is systemic sclerosis ?
type of scleroderma
(MEANS - hardening (sclero) of skin (Derma)
- Chronic Autommune , inflammatory , fibrotic condition - immune system attacks skin and internal organs leading to excess production of collagen ————————————>thickened and hard skin & build up of scar tissue (Fibrosis )
also can causes damage to organs & obliteration of BV.
Types
- Limited cutaneous systemic sclerosis / scleroderma
(Effects only skin - (not really systemic just called that )
ORIGINALLY CALLED CREST SYNDROME
C - Calcinosis (calcium deposits under skin)- mostly at finger tips .
R - Raynauds Phenonomon (in response to cold - finger tips go white —> blue —–> black)
E - Esophageal dysmotility ( connective tissue issue - cases Dysphagia , Acid reflux , Oesophagitis)
S - Sclerodactyly ( shiny tiny skin - lacks folds - restricts range of movement of joints - limited use of hand —> eventually skin can break & ulcerate )
T- telanglectasia ( small dilated blood vessels - mainly found on face , cheeks - but other areas too)
- Diffuse cutaneous systemic sclerosis / scleroderma
(CREST syndrome + affects on internal organs)
AFFECT ON ORGANS
- Heart
0 Hypertension
0 Coronary - Lungs
0 Pulmonary HTN
0 Fibrosis
( Patient presents with gradual onset SOB & Dry cough - Pulmonary fibrosis makes lungs stiffer & harder ————–> more difficult to expand and let in air ( restrictive) - Kidney
0 Glomerulonephritis
0 Scleroderma renal crisis ( severe HTN + A cute renal failure)
(Kidney problems worsen HTN problems)
RISK FACTORS
0 Hx of Scleroderma
0 Immune dysregulation - Positive ANA
Diagnosis of Systemic sclerosis?
0 FBC
- anaemia of chronic disease
- iron deficiency anaemia
( reduced RBC , thrombocytosis - too much )
0 Serum creatine - normal or elevated
- check kidney function - increased may indicated issue - worsen diffuse systemic sclerosis - risk of scleroderma renal crisis.
0 urine microscopy
(if protein in urine - may indicate scleroderma with renal crisis)
0 ESR , CRP (inflammation)
serum antinuclear antibody - non specific
- positive but negative does not rule out.
0 other auto- antibodies
- anti - Scl 70 (anti DNA topoisomerase I) , Anti RNA polymerase I,II,III also occur in diffuse cutaneous type. more associated with diffuse type)
0 Anti - centromere - more asscoiated with limited.
0 ECG
0 Echocardiogram
0 PFTs
( if dyspnoea, dry cough , decreased exercise tolerance —–> prompt investigation of heart and lungs)
0 Chest Xray - if PFT abnormal.
Then CT scan to confirm pulmonary fibrosis
0 Barium swallow - if dysphagia , reflux etc. ( should be done - if reflux symptoms dont improve with treatment)
- those diagnosed with scleroderma often also diagnosed with sjogrens syndrome.
Treatment of Anti - phospholipid syndrome?
NOT PREGNANT
1st line- UFLWH
PLUS - Warfarin , management of risk factors
(encourage to stop smoking)
PREGNANT
1st line - UFLWH
PLUS - Foetal monitoring , warfarin post delivery
- if they have obstetric APS ( features
O recurrent early miscarriage,
O fetal death at or beyond 10 weeks of gestation
O early delivery for severe preeclampsia or placental insufficiency.)
give VTE (venous thrombosis embolism ) thromboprophylaxis post delivery
if incidental APL ( acute promyelotic leukemia ) - give aspirin - 1st line , plus -VTE thrombo…
Catosphophic APS - NOT OR PREGNANT
0 unfractionated or low weight heparin (UFLWH)
(end in parins e.g heparin , dalteparin , enoxaparin)
ADJUNCT - Immunosuppressive therapy e.g 1st -Prednisolone 2nd - plasma exchange or/and immunoglobulin 3rd - Rituximab - monoclonal antibody.
ADJUCNT - if pregnant foetal monitoring.
What is Immune Thrombocytopenia (ITP) ?
- TYPES
- APPEARENCE
- DIAGNOSTIC FACTORS
- DIAGNOSIS
also Immune Thromboctopenic purpura
Platelet count below 100 x 10^9/L - isolated thrombocytopenia ( ie. other figures in blood count normal) in the absence of identifiable cause—————> can cause bleeding (formation of Petechiae - rash formed by bleeding into the skin
0 Two types - Primary (isolated ) vs Secondary ( underlying cause)
APPEARANCE / CHARACTERISTICS
Signs of bleeding 1. Bruising ,
2. Petechiae
(indicate small capillary haemorrages - if thrombocytopenia is severe (normally) - found most commonly on lower limbs,
3. Haemorrhagic bullae
(IF HISTORY OFF BLEEDING POINTS TO ANOTHER DIAGNOSIS)
OTHER DIAGNOSITIC FACTORS - COMMON
0 absence of systemic symptoms e.g. weight loss , fever , ( symptoms of autoimmune conditions -skin rash , alopecia , DVT . (SUGGEST OTHER CONDITION)
0 Absence of medicines that cause thrombocytopenia
0 Absence of spleno/ hepatomegaly
or lymphadenopathy - if present could indicate infection , autoimmune or lymphoplofierative conditions.
DIAGNOSIS
0 FBC and peripheral blood smear.
Risk factors for ITP?
0 Women of child bearing age
0 Age - below 10 , above 65
Treatment of ITP?
ALL - life /organ threatening bleeding
1ST LINE
- Intravenous immunoglobulin (IVIG) + Corticosteriod (Prednisolone / methypred , dexamethasone) + platelet transfusion.
ADJUNCT
Aminocaproic acid or, Tranexamic acid.
(antifibrinolytic)
NEWLY DIAGNOSED CHILD
Asymptomatic /minor bleeding
1ST LINE
- observation
Major bleeding
1ST LINE
- Prednisolone
2ND LINE (if 1st line contraindicated or not working)
- IVIG or Anti - D immunoglobulin
(IF USED - EVALUATE FOR BLOOD BOURNE VIRAL INFECTIONS - HEP B)
NEWLY DIAGNOSED ADULT
0 platelet above 30 x 10^9 or equal & asymptomatic - no additional risk factors
1ST LINE
- Observation
0 platelet above 30 x 10^9 or equal & symptomatic - additional risk factors or platelet below 30 x 10^9
1ST LINE
Corticosteriod and / or IVIG
or anti D - in RH positive pateints or non splenectomised (spleen still present).
CHILD - chronic / persistent
1ST LINE
Mycophenolate mofetil (DMARD - disease modifying drug)
or Rituximab or thrombopoietin agonist receptor e.g. eltombrag , romiplostim
(if one does not work tty others)
2ND LINE
- Splenectomy + IVIG or pred
CHRONIC/ PERSISTENT ADULT- NON PREGNANT
1ST LINE - Low dose steriof (Pred) -repeat IVIG infusions 2ND LINE (1st line chronic childrens treatment) 3RD LINE - Splenectomy.
Pregnant - chronic
1ST LINE
-high dose methylprednisolone
ADJUNCT
IVIG or /and azathoprine
Splenectomy
Contraindications of Aminocaproic acid & tranexamic acid ?
Aminocaproic acid and tranexamic acid are contraindicated in patients with haematuria because clots in the collecting system of the kidneys can lead to outlet obstruction.
Aminocaproic acid and tranexamic acid inhibit fibrinolysis and help to stabilise clots that have already formed. These agents can be used as adjunctive treatment as they do not affect platelet count.
Common drugs that cause drug-induced thrombocytopenia?
0 Heparin - most common
0 Furosemide 0 NSAIDS 0 Penicillin 0 Quinine 0 Quinidine 0 Ranitidine 0 Sulfonamide 0 Statins 0 Linezoid & other antibiotics
0 Alcohol
Symptoms of Systemic Sclerosis ?
CREST
C- Calcinosis
R - Raynauds Phenomenon
E - Esophageal Dysmolitlity
(Dysphagia , Acid reflux , Oesophagitis)
S - Sclerodactyly
( tightening causes ulceration & gradual loss of hand function)
T - Telangiectasia
0 Dyspnoea - effects on Heart & Lungs
( Echocardiograms & Pulmonary function test ( Spirometry , lung volumes etc done to mointor condition)
0 Dry crackles at lung bases - indicate intersitual lungs (especially if PFT indicate restrictive pattern )
0 Abnormal nail fold capillaroscopy (test done to look at nail bed changes - can help diagnose scleroderma & Raynauds)
0 Swelling of hands and feet
( feet - may be problems with heart
Hands - can happen before Raynauds.)
0 Bloating
0 Faecal incontinence - loss of anal sphincter tone.
Interstitial lung disease - conditions that cause fibrosis of lungs.
Limited scleroderma version only refers to CREST.
Treatment of Systemic Scleroderma?
Caution with certain medicines?
Look om BMJ best practice
Treatment depends of symptoms diplayed e.g GORD (PPI etc)
High dose corticosteriods
(increased risk of renal crisis)
aim of treatment of renal crisis - is achieve normal BP to optimise renal recovery.
TREATMENT FOR CARDIAC TAMPONADE
Pericardial window ( surgery - small piece of the sac around heart is removed to drain excess fluid) + Prednisolone
RAYNAUDS (NO UCLERATION)
- Lifestyle - avoid cold , hand excercises , smoking cessation etc.
INTERSITITAL LUNG DISEASE
- Observation + Immunomodulator
(Immunomodulator - Mycophenolate , cyclophosphamide, azathropine , nintedanib)
What is Polymyalgia rheumatica?
Inflammatory rheumatologic syndrome Condition causes pain & stiffness in the muscles around the shoulders, neck & Hips..
(CAUSE - unknown - combination of genetic + environment)
CHARACTERISTICS
- Pain
- Morning stiffness
( in neck , shoulder , pelvic gridle in over 50s) - rapid improvement to NSAID therapeutic trial (24 - 72 hrs improvement seen)
- The following don’t all have to be present.
- Malaise
- Low grade fever
- Weight loss
- anorexia
- depression
- oligoarticular arthritis ( or transitory manifestations)
RISK FACTORS
0 Age - Above 50 yrs
0 Giant cell arteritis
- 15 - 20 % of pl with PMR have GCA
- 60 % of pl with GCA have PMR
Diagnosis of Polymyalgia Rheumatica (PMR)?
0 ESR - above 40 (some can have normal tho)
0 CRP
(both elevated)
0 FBC - to differentiate from myeloprofiferative diseases.
CONSIDER
0 TSH - high levels (indicate no negative feedback from T4 , T3)
(IF high - indiacted hypothyroidism)
- Ultrasound
- MRI
- RA & anti -CCP (rule out RA - if suspected)
Treatment of PMR?
ACUTE
1ST LINE
0 Corticosteriods (oral) + Calcium , VD , Biphosphonates
ADJUNCT - NSAIDS
2ND LINE
0 Methotrexate + Folic acid
3RD LINE
0 Tocilizumab (T) (IL-6)
(may increase risk of serious liver injury)
(AST , ALT levels monitored)
ONGOING - treatment resistant / relaspe / worsening
1ST LINE
Increased steriod dose + VD , Ca , Bispho
ADJUNCT - Methotrexate plus folic acid
( in this case used as a steriod sparing agent - should be continued until steriods can be tapered without recurrence of PMR symptoms. )
2ND LINE
T or Leflunomide (disease-modifying antirheumatic drugs) (DMARDS)
Side effects of Corticosteriods ?
C - Cushing syndrome
0 - Osteoporosis
0 Retardation of growth
0 T - Thinning of skin
0 I - Immunosuppression
0 C - Cataracts + Glaucoma
0 O - Oedema
0 S - Supression of HPA axis (hypothalamic - pituary )
0 T -
0 E - emotional disturbance
0 R - raised BP ( Hypertension)
0 Obesity
0 I - increased body hair
D - Diabetes
S -Striae (stretch marks)
Which conditions use Steroids as a treatment ?
acronym
- not exhaustive
MAGIC PAUL
M - Multiple sclerosis
A - Asthma/ Allergic rhinitis & Hay fever
G- Giant cell arteritis or Polymyaligia rheumatica
I - IBD ( Crohn’s & UC)
C - COPD
P - Painful & inflamed joints . muscles , tendons
A - Atopic eczema
U - Uriticaria (hives)
L - lupus (SLE)
What is Giant cell arteritis ?
OTHER NAMES
- Temporal arteritis (Temporal - sides of head
- Cranial arteritis
- Granulomatous arteritis
Form of Vasculitis (Inflammation of BV)
Affects Large , medium sized arteries.
(most common form of systemic vasculitis in adults ) (arteries at the side of head inflamed) particuraly
CHARACTERISTICS
- Headache
- PMR symptoms e.g. Stiffness/pain in shoulders, neck & pelvic gridle.
- extremity claudication (pain on use of extremities especially upper (rarely in lower ?)
- other Cranial arteries can become enlarged or tender.
- Systemic symptoms
(low-grade fever , malaise , fatigue , weight loss)
RISK FACTORS
- age - > 50 yrs
- Female sex
Diagnosis of GCA ?
0 ESR - > 50mm/hr (same may be below this)
0 CRP -elevated
0 FBC - normocytic anaemia with normal WBC count , elevated platelet count
0 LFT - transaminases and alkaline phosphatase are often mildly elevated
FOLLOWING 2 :
(Needed for all patients with suspected GCA)
0 Temporal artery ultrasound ( show thickening , stenosis , occulsion )
0 Temporal artery biopsy - to confirm diagnosiz
Treatment of GCA ?
Suspected GCA
NO VISUAL OR NEUROLOGICAL SYMPTOMS
1ST LINE
Prednisolone (oral)
Visual signs or neurological symptoms
1ST LINE
- Methylprednisolone pulse therapy
( Methylprednislone IV then followed by Prednisolone oral)
(suspected GCA - should not wait for confirmed diagnosis (biopsy results - should start steriods straight away)
CONFIRMED GCA
1ST LINE
PRED + Ca , VD (ergocaliferol) , ALendron or Risedronaus -
(OSTEOPOROSIS PREVENTION)
plus - Tocilzumab or methotrexate.
(if recurrent or replasping disease or severe gluccocorticoid adverse effects.
ADJUNCT -Aspirin (in selected groups without bleeding risk - aspirin thins blood to prevent clot - so risk of internal bleeding)
What is Axial Spondyloarthritis ?
OTHER NAMES
- axSpA
- Ankylosing spondylitis (AS)(radiographic stage of axSpA)
axSpA - non - radiographic stage.
AXIAL - SPINE
Spondylo - SPINE
ANKYLOSIN - STIFFENING
ITIS - INFLAMMATION OF JOINTS IN SPINE
Chronic , painful Inflammatory arthritis of the spine & sacroiliac joints mainly
(effects Axial skeleton)
- can affect :
enthesis (place where tendons/ligaments meet bone) - peripheral joints
- extra - articular sites e.g. eye , bowel
CHARACTERISTICS
- Severe pain in spine
- Spinal stiffness
(these lead to radiographic changes in spine & sacroiliac joints) - Inflammatory back pain (includes - early morning stiffness , stiffness improvement with exercise , insidious onset , age of onset below 40 , pain lasting longer than 3 months)
OTHERS
- Fatigue
- sleep disturbance
- tenderness at sacroiliac joint
- peripheral joint involvement (hips . costoveterbral joint, costochondral J (if CCJ affected - Dyspnoea can occur due to limited chest expansion- common in advanced stages)
- loss of lordosis (curvature of spine (lumbar lordosis - excessive curvature of the spine))
- kyphosis - advanced disease }
EXTRA - ARTICULAR MANIFESTATIONS
- Iritis / uveitis (more prevelant in AS -radiographic stage)
- Enthesitis ( heel . knee , ischial tuberosities - most commonly affected)
RISK FACTORS
- ## HLA -B27 gene
- Family Hx of AS
- Male sex
- Age - late teens , early 20s - most common (but can happen at any age )
- IL23R (Interleukin 23 receptor gene and ERAP1 (endoplasmic reticulum aminopeptidase 1)
Diagnosis of axSpA / AS ?
- Pelvic X ray
( positive diagnosis - if sacrolitis present)
NEGATIVE RESULT DOES NOT RULE OUT DIAGNOSIS AS SACROLITIS TAKES TIME TO SHOW ON RADIO GRAPH)
CONSIDER :
- HLA - B27
-MRI - may show abnormality if X ray comes back normal.
(in young patients early use is advocated to reduce exposure to radiation)
- Lumbar (may see bamboo spine in late disease) , cervical , thoracic lateral X rays
(look for progression in those with confirmed diagnosis) - Ultrasound - confirm enthesitis
JUST FOR KNOWLEGDE
Schober test can be done - checks if there is decrease in movement (flexion) at lumbar spine
- Wall to tragus test
( wall to ear tragus ) - looking at shape of spine e.g excessive kyphosis present etc. - test for chest expansion .
Treatment of axSpA/ AS?
ADULTS
PAIN + / OR STIFFNESS
1ST LINE
NSAID + Non - pharmological therapy
ADJUNCT - Analgesic e.g para , codeine
PLUS:
- intra-articular corticosteriods
(if there is intra-articular inflamm or enthesitis) - Sulfasalzine or methotrexate (if peripheral joint involvement)
WITHOUT PAIN +/OR STIFFNESS
1ST LINE
- Re- assessment and observation
ONGOING -REFRACTPRY TO 2 NSAIDS + NTP
TNF - alpha + physiotherapy
2rd line - alternative TNF - alpha inhibtor
2nd line - Secukinumab or ixekizumab
(all lines in ongoing - you continue a NSAID drug)
CHILDREN
OLIGOATHRITIS
1ST LINE
- NSAIDS + non - pharmalogical therapy (NPT)
PLUS - Intra- articular steriods
POLYATHRITIS
1ST LINE
- Sulfasalzine + NTP
with enthesitis and/or peripheral arthritis
1ST LINE
TNF - Alpha inhibitor + NPT
(methotrexate is not useful in axial disease)
What is seronegative spondyloathropathy
Group of conditions which are associated with HLA-B27 gene and share clinical manifestations - e.g inflammatory back pain , uveitis , rashes , GI symptoms etc
EX - Psoriatic athritis
- Reactie athritis
- Enteropathjic athritis ( Spondyloarthropathy whch develops in association with GI conditions :
IBD , Whipple disease etc)
- undifferiated SpA ( lacks clinical , radiological & serolgical features that would give it a diagnosis ) - often the early stage of a well known form of athritis.
Seronegative - means not positive for Rheumatoid factor, ANA.
What is a Psoas muscle abscess ?
Collection of pus in iliopsoas muscle.
TYPES
- Primary - caused by hematagous or lymphatic spread of infection from distant source or muscle injury.
- Secondary - contiguous spread of a pathogen e.g. GI or musculoskeletal , GU , Cardio origin etc.
CHARACTERISTICS
Classic Triad
- Flank pain
- Fever
- Antalgic pain - limp to avoid pain
If secondary type symptoms of underlying condition may be present e.g UTI symptoms
Treatment for Psoas Abcess ?
- Drainage (open or CT guided )
&
- antibiotic therapy
- treat underlying condition
What is Retroperitoneal fiborosis ?
Fibrosis (excessive growth of connective tissue)
Fibrosis in retro peritoneal area (behind stomach and intestine)
Fibrosis ————–> mass forms ————————–> compression of structures————> can alos wrap around BV, Ureter & nerves and encase them .
Can compress & block:
Ureters - Obstructive uropathy
(if this happens urine backs up —————> Material / substances build up in blood. )
- Arteries
SIGNS & SYMPTOMS
-depend on which part/organs affected
- Lower back pain
(Dull , vague , hard to localise) - can also be in flanks - anaemia
- weight loss
- fever
- loss of apeptite
- impaired movement of limbs
- jaudice
- swelling of leg (one)
- discolouration of leg due to reduced blood flow.
- kidney failure
- hydronephrosis (swelling of kidney duct with urine)
- Hypertension
JUST FOR UNDERSTANDING
(symptoms, such as low-grade fever, unexplained weight loss, anorexia and malaise. Because of its insidious nature, diagnosis often only occurs once late complications have developed. Pain is the most common presenting symptom, mainly in the back, fl anks and abdomen, and when the ureters are involved can mimic renal colic.3 Lower urinary tract symptoms, including haematuria, frequency, urgency and dysuria, occur less frequently.2 Other urological manifestations, such as testicular pain and swelling, hydrocele and varicocele can develop owing to compression of retroperitoneal gonadal vessels.2,3,6Encasement of the ureters often leads to obstructive uropathy and, if left untreated, can cause permanent kidney damage. New-onset hypertension (or worsening of pre-existing high blood pressure) may occur owing to impingement of renal vasculature or to hydronephrosis.2 Other vascular complications mainly affect the venous system (most commonly inferior vena cava and iliac veins) with involvement of major arteries being rare except in RPF related to aortic aneurysms. These may result in lower limb swelling, deep vein thrombosis, claudication and even hoarseness of the voice due to recurrent laryngeal nerve compression when the infl ammatory mass extends upwards around the thoracic aorta)
Treatment of Retroperioneal fibrosis ?
if blockage of ureters - ureterolysis (surgery)
Corticosterios
