Case 1.
Pain mostly in her small joints, such as MCP and PIP joints Stiffness in the
morning which lasts about 45 minutes Pain increases at night at wakes her up
* housewife running once a week eating healthy smoking
* CBC mild anemia
* URINALYSIS normal
* CREATININE normal
* ALT,AST normal
* CRP elevate
* ESR elevate
* RF ANTI-CCP antibodies +
* Radiology – decreased bone density, soft tissue swelling, Narrowing of joint space,
Bony erosions
* Radiography of hand - PIP joint of the 3rd 4th finger swelling soft tissue and osteope-
nia
RHEUMATOID ARTHRITIS
Chronic inflammatory affect joints can involve other organ brain muscle liver skin
blood vessels
Pain in 5 or more small joint MCP,PIP,MTP symmetrical pain increase in rest. Or after
being inactive for along period of time morning >30 min
Ulnar deviation rheumatoid nodules swan neck
Felty syndrome is rare condition involve rheumatoid arthritis decrease white blood cell
count and swollen spleen
* Splenomegaly
* Anemia
* Neutropenia
* Thrombocytopenia
* Arthritis rheumatois
Treatment -DMARD-MTX,leflunomide sulfasalasine chloroquine glucocorticoids biologic
therapy
Case 2. Pain mostly in her DIP joints Stiffness in the morning which lasts about 15 min-
utes Pain increases in the evening and relieves at rest Also she underlined that her
pain becomes more severe when the weather worsens
* office worker wear hight heels eating fast food mostly BMI 28
CBC normal
URINALYSIS normal
CREATININE normal
ALT,AST normal
CRP mildly elevated
ESR normal
OSTEOARTHRITIS
Degenerative wear and tear process damage to bone and cartilage
Modified risk factor ( obesity occupation smoking bone density physical activity
Non modified risk factor (age gender genetic joint injury anatomy factors)
Aging changes reduce hydration and accumulation of proteins containing advanced
glycation end products AGE-which cause increased cross linking of collagen resulting
in brittleness of cartilage
——hand DIP PIP 1STGMC , hip knee L-spine -l4-5-s1 C-spine , ankle shoulder elbow ,
rest of wrist——
chomdrocalcinosis —result of accumulation of calcium pyrophosphate dehydrate CPP
crystal
Pro inflammatory factor produce proteolytic enzyme responsible for the degradation
of the extracellular matrix that result in joint tissue destruction and loss articular carti-
lage is a central component
1. Sharp pain 2. Constant pain 3. Constant dull aching pain episodes severe limitation
Tenderness limitation of motion swelling deformation instability
Asymmetrical hebendes nodes
Diagnosis
Persistent usage-related joint pain in one or few
joints
Age ≥45 years
Morning stiffness ≤30 minutes
Radiology , Ultrasound
Lab tests – mild elevation of acute phase
reactants
Deformity of hand in X-ray ( thumb squaring , heberdens nodes , Bouchard nodes)
Case 3 woman no complains plans pregnancy previous 3 spontaneous abortion in his-
tory
SYSTEMIC LUPUS ERYTHEMATOUS
Skin,blood,cns,lung,serum , spleen, joint,kidney,joint
environmental hormones- oestrogen prolactin genetic ultraviolet light infection EBV
CMV retroviruses parvovirus b 19 , heavy metals , chemicals , drugs , genetic factors
complex genes HLA -DR2 -DR3 genes-C1Q , C4 ,C2 ,cytotoxic T lymphocyte
antigen ,programmed cell death , cytokine genes INF-a , TNF-a
Type 3 hypersensitivity immune complexes
Type 2 hypersensitivity Pseudoneutropenia resulting from neutrophilic clumping in a
patient of SLE with autoimmune hemolytic anemia.
* antinuclear antibodies (best screening test
* Double stranded DNA
* Small nuclear ribonucleoproteins (Ro -SS-ALA-SS B, RNP, Smith antigen)
* Enzymes (topoisomerase Scl 70)
* Histone proteins Centromere proteins
* Homogenous pattern very specific
Symptoms
* fatigue fever malaise lymphadenopathy weight loss
* Mucocutaneous manifestation : skin rash photosensitivity ulcers painful or painless in
the mouth nose vagina
* Alopecia with hypo or hyperpigmentation
* Malar rush butterfly shaped erythema flat or raised
* Discoid rash erythematous circular raised patches with keratitis scaling and atrophic
scarring
* Musculoskeletal manifestation - arthritis 2 or more joint involvement generalised
arthralgia + early morning stiffness deformities are unusual but may occur due to
ligamentous laxity , myalgia (cause by corticosteroids , antimalarial ) , avascular
necrosis ( progress to collapse of the femoral head)
* Haematological manifestation - anemia leucopenia lymphopaenia neutropenia
thrombocytopenia
* Renal manifestation - nephritis asymptomatic ( do urinalysis for protein blood and
cast )Proteinuria >0.5 g/d or ≥3+, or cellular casts
* renal biopsy is helpful - minimal focal diffuse
* Jaccouds arthropathy
* Nervous system manifestation - cns headache seizure aseptic meningitis cerebrovas-
cular accidents peripheral - Gillian barre syndrome ,mononeuropathy single or mul-
tiplex myasthenia gravis
* Pulmonary manifestation - pleurisy is common ( lupus pneumonitis pulmonary em-
bolism pulmonary hypertension)
* Cardio manifestation - pericarditis common ( myocarditis , fibrinous endocarditis of
Libman sacks
* GI manifestation - abdominal pain nausea vomiting diarrhoea -lupus hepatitis ALT
&AST increased , diffuse abdominal pain acute abdomen caused by autoimmune
peritonitis and mesenteric vasculitis
* Ocular manifestation - sicca syndrome ( sjogrens syndrome ) nonspecific conjunctivi-
tis , retinal vasculitis , optic neuritis —- demyelination inflammation of the optic
nerve, cataract common and glaucoma
* Diagnostic - ANA , ANTI-DNA , antiphospholipids , antismith
* Drugs - hydroxychloroquine , azathioprine , methotrexate , cyclosporin A, leflu-
namide ,cyclophosphamide , mycophenolate mofetil
Case 4 Rash spontaneous hair loss loss of appetite and weight loose mild pain in her
small joint in morning
Case 5 55 years old woman fatigue and sudden onset of pain in her left hip
Diagnosis (SLE arterial hypertension hyperlipidemia ) MI and coronary angioplasty 3
years ago
drugs: immunosuppressive therapy ACE inhibitor beta blockers aspirin and lipid lower-
ing agents
Case 6 ANTIPHOSPHOLIPID SYNDROME
-antiphospholipid antibodies produced by B cells
- activation of inflammatory cells and endothelial cells
- Promotion of coagulation
- Result inflammation , vasculopathy , thrombosis , pregnancy complication
- Arterial is more common in man can cause heart attack , stroke or limb ichemia ,lib-
man sacks endocarditis caused by vegetation generally in mitral valve
- Vein thrombosis is more common in women - deep vein thrombosis sometimes em-
bolus may cause pulmonary embolism which is a life threatening issue , miscar-
riage , thrombosis placental infraction — morbidity in pregnancy. fetal death beyond
10 week gestation
- More than one premature birth due to presence of severe placental insufficiency
pre-eclampsia or eclampsia before 34 weeks gestation
- Livedoreticularis
Case 7 systemic scleroderma or systemic sclerosis = CREST+ INTERNAL OR-
GAN
Fatigue ,muscular pain time after time , hardening of the skin on her hand finger and
on left lower extremity the element is line shaped , hypopigmentated and dense by
palpitation , gastro reflux ,
* autoimmune inflammatory fibrotic connective tissue affect mid age women mean
hard skin but affect not only skin also internal organ
* There’s localised version which affect only skin = CREST SYNDROME
* caused by genetic or environmental causes - vascular abnormalities , immunologic
abnormalities and extracellular membrane abnormalities fibrosis
* Systemic 1. Limited 2. Diffuse
* Localised 1.morphea 2. Linear
* Patient with linear type have thick streaks of skin usually affecting the limbs and
torso
* Morphea -painless discoloured patches on your skin , typically skin changes appear
on the abdomen ,chest or back can also appear on face arm legs
* Pulmonary - hypertension and fibrosis
* Cardiovascular -hypertension and coronary artery disease
* Kidney - glomerulonephritis ,scleroderma renal crisis
* Skin is firm coarse and thickened and extremities and trunk may be darkly pig-
mented in dark skinned patient vitiligo like hypo pigmentation may occur ,, because
pigment loss spares the perifollicular areas the skin may have salt and paper appear-
ance mostly on scalp upper back and chest
* Skin thickening with fibrosis of tendon causes contractors of the wrist elbow and
knees the face (mauskopf) with taut and shiny skin loss of wrinkles and expression-
less facies due to reduces mobility of eyelids cheeks mouth
* Skin ulceration -painful and may get infected resulting in osteomyelitis - breakdown
of atrophic skin leads to chronic ulceration fingertips elbow
* Lungs- infiltration of the alveolar wall interstitial fibrosis , progressive thickening
alveolar sedate cause honeycombing as well as loss of pulmonary blood vessels ,
pulmonary hypertension ,,most common , dyspnea fatigue reduces exercise toler-
ance
* Synovitis - synovium become fibrotic
* Fibrosis of thyroid glans and minor salivary glands may be seen
* Diagnosis == no laboratory , carful history and physical examination
* Antibodies == ANA . ANTI-SCL-70 ANTITOPOISOMERASE I . ANTICENTROMERE . ANTI-
RNS-POLYMERASE
* Diagnosis = nailfolf capillaroscopy = examine nailfolds areas of the skin where the
base of the fingernail meet , evaluation of health of peripheral capillaries = avascular
microhemorrages indicated scleroderma ,,, skin biopsy dense macro aggregates of
microfibrils in dermis
* Manage -= should screened routinely , immunosuppression, glucocovrticosteroids ,
nifedipine , ace inhibitor for hypertension , organ specific therapy , eat small fre-
quent meals , not eat within 2-3 hours of bedtimes , keep head elevated , avoid to-
bacco alcohol caffeine
*
Case 8 CREST SYNDROME
Calcinosis -calcium deposit in the skin , the TH cells accumulated around the small
vessels mostly on hand and face releasing cytokines cause inflammation that lead to
necrosis after cell death calcium in cytosol binds to cell membrane and build up in the
skin which called calcinosis cutis they will be positive anticentromere antibodies
Rayanaud phenomena - 1. Episodic 2. Primary 3. Secondary spasm of blood vessels in
response to cold or stress
Phases - -pallor vasoconstriction - cyanosis ischemia - erythema reperfusion
Esophageal dysfunction - lower oesophagus mostly with atrophy of muscular layer acid
reflux and decrease in motility of oesophagus diminished peristaltic activity with gi re-
flux and small bowel obstruction can lead barrets metaplasia
Sclerodactyly -thickening and tightening of the skin on the fingers and hands
Telangiectasia - dilation of capillaries causing red marks on the skin surface
Case 9.
Sjogrens syndrome
* pathophysiology - environmental factors and activation of glandular cells - HLA-DR
independent immune system -alteration of glandular vascular endothelium - infiltra-
tion of the gland by lymphocytes of HLA dependent immune system - activation of
lymphocytes lead to cell destruction cytokines autoantibodies metalloproteinase -
impaired secretion due to glandular dysfunction
* Two types of ANA is formed against ribonucleoproteins - anti RO SS A LA SS B
* The number of interleukin 18 positive macrophages correlates with parotid gland en-
largement
* Low level of the C4 component of complement is a predictor for lymphoma develop-
ment
* Symptoms. Related to lacrimal and salivary gland function , dry mouth xerostomia
difficulty in swallowing dry food inability to speak continuously burning sensation in-
crease dental caries ,,,dry eyes burning decrease tearing ,redness itching eye fatigue
blurring vision increase photosensitivity - keratoconjuctivitis sicca , inflammation of
cornea and conjunctiva ,,, involvement of other exocrine glands occurs less fre-
quently and includes respiratory or gastrointestinal tract , decrease in mucos glands
secretion of the upper and lower respiratory tree leads to dry nose throat and tra-
chea , dry cough is the major manifestation that is attributed to small airway disease
, in gi tract may be oesophageal mucosal atrophy , atrophic gastritis ,, dyspareunia
and dry skin also may occur ,, swelling of glands can press nerve which cause pain
* Systemic manifestation —extraglandular manifestation — fatigue , low grade fever -
rayanaud phenomena ,myalgia, arthralgia , at least one episode of non erosive
arthritis , renal involvement interstitial nephritis hyposthenuria and renal tubular
dysfunction with or without acidosis
* Untreated acidosis may lead to nephrocalcinosis
* Vasculitis affect small and medium vessels - purpura recurrent urticaria , skin ulcera-
tion glomerulonephritis and mononeuritis multiplex
* Lymphoma present later = persistent parotid gland enlargement , purpura leukope-
nia cryoglobulinemia low c4 complement level
* Diagnosis ==== sialography and scintigraphy ultrasound MRI of salivary gland used
laboratory tests normocytic anemia , elevate ESR ,CRP normal
* Diagnostic Schirmmers test measurement of tear flow to keep it moist
* Management === keep eye and mouth moist = hydroxichloroquine glucocorticos-
teroid , pilocarpine to stimulate secretion
Case 10
GOUT
Is metabolic disease that most affect men and postmenopausal women , characterised
by episodic acute arthritis caused by deposition of MSU crystals in a low perfusion ar-
eas such joints and connective tissue , MSU deposition in kidney interstitium may
nephrolithiasis
* purine breakdown product = uric acid
* Elevate purine source = catabolism or tumor lysisnsyndrome , diet
* Gout risk factor = male , age , obese , genetic polymorphism , kidney disease
* Hyperuricemia = joint inflammation kidney or bladder stones nephropathy metabolic
syndrome
* Decreased renal clearance = drugs like aspirin , fructose , kidney disease
* Symptoms ==== intermittent acute monoarthritis especially first metatarsopha-
langeal joint
* Hallux valgus misdiagnosed as a gout attack difference—- deformity MSU crystals
men -women
* Stages = 1. Asymptomatic hyperuricemia 2. Acute and intermittent gout 3. Chronic
tophaceous gout
* tophi == is a deposit of monosodium rate crystals
* Diagnosis = hyperuricemia , uric acid in blood low ,, uric acid crystal identified in
joint fluid
* During acute attack crystal seen intracellularly and extracellularly -needle shaped
MSU crystals
* manage== pain relief , prevent attack , prevent formation of tophi , diet purine
should be removed , water 2 later per day , NSAID , colchicine , diuretics should be
stopped , uric acid should use allopurinol
Case 11
PSEUDOGOUT
Calcium pyrophosphate dehydrate crystal deposition , acute gout like attack , monoar-
ticular inflammatory arthritis lasting for several days to 2 weeks
* between episodes patient asymptomatic half of episodes involve knees
* Without synovial fluid analysis impossible to know from septic arthritis
* Rhomboid shaped and positively birefringent
* Manage == NSAID , intra articular injection of glucocorticosteroid
Case 12
RHEUMATIC FEVER
Systemic , immune trigger by pharyngeal in section with group A streptococci
* fever , migratory poly arthritis , carditis
* Common in 5-15 year old
* Rare in children under 3 year
* Group a strep cross react with human tissue through molecular mimicry
* Diagnosis = jones criteria = major = carditis, polyarthritis , chorea , erythema
marginatum , subcutaneous nodules ,,,,minor = fever,arthralgia, elevate acute phase
reactant , prolong PR interval on electrocardiogram
* One major and two minor = diagnosis plus strep infect
* ARTHRITIS = migratory , contrast to post streptococcal reactive arthritis and pol-
yarticular , affect large joint of the hands and feet , axial skeleton rarely , red hot
swollen tender joint and so much pain ,, respond to salicylates
* Carditis == endocardium myocardium pericardium
* Endocarditis = mitral or aortic valvulitis , tricuspid and pulmonary valve are rarely
affected
* Required auscultation murmur echocardiography finding valvular regurgitation with-
out murmur do not fulfil criteria
* Subcutaneous nodules = firm nontender less than 2 cm , they typically over bony
prominences or tendon sheaths
* Minor == fever resolves
* Lab test= antistreptolysin O titer to establishes a recent strep. Infection above 500 ,
antistreptokinase test
* Differential diagnosis=== juvenile rheumatoid arthritis , SLE , gonococcal arthritis,
reactive arthritis , acute polyarticular arthritis in children
POSTSTREPTOCOCCAL REACTIVE ARTHRITIS
* acute in onset , usually nonmigratory , lack of respond NSAID , culture
* symptoms = large joint and lower limbs , arthritis monoarticular or polyarticular and
symmetric or asymmetric , tenosyvitis , fever scarlatiniform rash , resolve slowly ,
* Extra - articular manifestation - glomerulonephritis and uveitis
* Treatment == aspirin , anti streptococcal prophylaxis for 1 year monitor for 1 year ,
penicillin first line , erythromycin for penicillin allergic ,
Case 13
Dermatomyositis
- presence of a vacuoles formed by clumps of proteins that collect within the muscle fi-
bre
May overlap with scleroderma and mixed connective tissue disease
Caused by statin lung disease radiation genetic smoking
* humoral immunity , involve B cells and CD4+ immune complexes leading to perifas-
cicular vascular abnormality
* Injury to both capillaries ( by attack on endothelial antigen ) and myofibers , perifas-
cicular atrophy occurs as a consequence of a lack of blood supply to these particular
myofibers
* symptoms= progressive symmetrical muscle weakness , walking up stairs sitting up
from a chair , reaching things from shelf ( myalgia, dysphagia , disathtria == associ-
ated condition interstitial lung disease , raynauds phenomenon , SLE
* Gottron’s papule - erythematosusand scaly lesion mostly on PIP DIP MCP extensor
surfaces can be on knees elbows
* Heliotrope rash
* Mechanics hands
* Shawl sign - hyperpigmentated upper back mostly , affect area
* V-sign
* Poikiloderma on the lateral aspects of the thighs , referred to as the “ holster sign”
* Can mimic mallard rush and weakness
* Associated with malignancy = colon cancer lung breast ovarian
* Can be a paraneoplastic syndrome
* Perivascular and perymisial inflammation
* Lab test = ck increased , ast alt increased ANA positive
* Shrinkage weakness and atrophy the proximal and distal muscles
* Biopsy = inflammatory cells invading necrotic myofibers and displacing other my-
ofibers in inclusion body myositis
* Optimize mescle function , physical and occupational therapy , immunosuppressive
therapy ,
Case 14
Polymyositis
With systemic autoimmune or connective tissue disease or viral and bacterial infection
Caused by zidovudine statin
* cell mediated immunity , CD8+ cytotoxic t cells
* Diagnosis of exclusion should not have this - rash , family history of neuromuscular
disease , endocrinopathy , muscular atrophy biochemical muscle disorder IBM as ex-
cluded by muscle biopsy analysis myotonic or toxic drug
* Criteria - symmetric proximal muscle weakness , elevate enzymes muscle biopsy cu-
taneous finding
* Lab test = ck increased , ast alt increased ANA positive
* Endomysial inflammation
* Inflammatory
* Biopsy = intense interstitial mononuclear infiltrate with some myocyte degeneration
* Treatment = corticosteroid , DMARD ,MTX , AZATHIOPRINE , CYCLOPHOSPHAMIDE ,
HYDROXICHLOROQUINE , physical and occupational therapy , screening for malig-
nancy
*
Case 15
Fibromyalgia
* chronic widespread musculoskeletal pain and tenderness , fatigue , unfreshing
sleep , cognitive dysfunction , anxiety , depression
* Common in women
* Widespread musculoskeletal pain - pain all over
* Pain above and below the waist on both sides of the body and involves the axial
skeleton
* Fatigue and sleep disturbances
* Cognitive problem - attention tasks rapidly
* Psychiatric - depression anxiety headache migraine tension , parenthesias , numb-
ness tingling burning sensation
* Diagnosis based on history physical exam ,,, laboratory normal
* manage= exercise program education , drugs = SSRI , SNRI
Case 16
37 old man , low back pain at night , can’t tie the shoe laces , office worker but can’t
sit for a long time painful
Axial spondyloarthritis = equals for men and women
* axial joint -chest , spine , hip bone
* No definitive structural damage on X-ray
* Often MRI evidence of inflammation on sacral and. Spine
* Spinal inflammation = gene HLA 28 , cytokine TNF -A play role in immunopathogene-
sis
* Pain and stiffness in buttocks , low back and maybe chest wall , worsen with rest and
get better with exercise , inflammatory rhythm of pain
* Reduced spinal mobility
* Oligoarticular monoarticular large joint involvement ( hips and shoulder )
* History of eye pain redness blurry vision that may have been diagnosed as anterior
uveitis
* Physical examination = sacroiliac joint tenderness pain with compression /stress
* Hip shoulder -pain on motion decreased range of motion
* Lumbar spine , tenderness, paravertebral muscle spasm , loss of lordosis ,( Adams
forward bending test ) decrease flexion - schober test <5cm , decreased lateral mo-
tion and extension
* Thoracic spine - increased kyphosis , tenderness , pain with rib cage compression ,
decreased chest expansion <2,5 cm
* Cervical spine - tenderness , pain on motion , muscle spasm , decreased motion
kyphosis , decreased lordosis
* Diagnoses = no specific lab test , HLA B27 positive , radiology more important -
sacroiliitis , MRI =shiny corners or Romans lesion, may develop bamboo spine on the
later stage
* Limitation of motion of the lumbar spine in the sagittal and frontal planes , limitation
of chest expansion to 2.5 cm or less , measured at the level of the fourth intercostal
space
* Criteria = radiography -sacroiliitis - unilateral grade 3 (sclerosis and erosions of the
joint ,margin,joint space widening ) or grade 4 ( fusion across the joint) ,,,, bilateral
grade 2 ( sclerosis of joint margins to 4
* Manage = NSAID
Case 17
26 old , pain swelling left knee , medical history -chron disease
Enteropathic arthritis
* With ulcerative and Crohn , peripheral arthritis correlate with bowel activity ,usually
self limited
* Erythema nodosum and pyoderma gangrenous may be cutaneous manifestation with
the peripheral arthritis
* Role out septic arthritis avascular necrosis
* manage= treat bowel disease , if this persist , sulfasalazine can be effective
* NSAID avoided as first line it can worsen bowel disease
* If sulfalazine fails to fully control symptoms , then anti TNF agent should be used
Case 18
Young man , pain stiffness and swollen middle finger on the hand , low back pain at
rest , family history father has psoriasis
Peripheral spondyloarthritis = more man
* peripheral joint - fingers , knees , toes
* Definitive structural damage
* Bony growth leading to vertebrae fusion
* Most in men
* Manage = DMARD , glucocovrticosteroids , TNF alpha inhibitor
Case 19
Reactive arthritis
Inflammatory arthritis that develops after certain infections gi or genitourinary tracts
After 1-4 weeks of gi by shigella , salmonella , campylobacter , yersinia , STD chlamy-
dia , trachomatis HIV ,
* symptoms = oligoarthritis ( knee and ankle ) , enthesitis , , dactilitis “sausage nails” ,
conjunctivitis iritis , keratitis
* Specific rash-blenoragic ceratoderma
* Manage = NSAID antibiotic glucocorticoids , DMARD
Case 20
Psoriatic arthritis
* associated with skin and nail psoriasis
* Axial skeleton , asymmetric oligoarthritic , symmetric polyarthritis of peripheral joints
, enthesitis and the absence of rheumatoid factor
* Unlike rheumatoid arthritis , psoriatic arthritis has a predilection for the DIP joints
* Test = CRP ,ESR + HLA B-27 + hyperuricemia radiology - sacroiliitis , enthesitis
* Pencil in a cap
* Manage = NSAID , DMARD , glucocorticoids , biologic therapy
Case 21
Giant cell arteritis - temporal arteritis common in adult >50 years old
* headache ( new onset ) polymyalgia rheumatic , jaw claudication, visual symptoms ,
PMR
* Temporal artery biopsy is the gold standard for diagnosis
* Temporal artery tenderness to palpitation or decreased pulsation unrelated to arte-
riosclerosis of cervical arteries
* Elevate ESR >50 by western method
* Biopsy = mononuclear cell infiltration or granulomatous inflammation with multinu-
cleate giant cell and endothelial proliferation
* Respiratory - dry cough , throat pain , tongue pain
* Large artery involvement - claudication in arms or legs , unequal arm blood pres-
sure , thoracic aortic aneurysm
* Giant cell arteritis stenosis , proximal left subclavian artery , with diffuse , marked
narrowing of the axillary artery
Case 22
Polymyalgia reumatica - aching and stiffness of the shoulder neck and hip-girdle area
that can occur with GCA
* stiffness and aching of the shoulder , neck , hip region , diagnosis is clinical e
* Elevate ESR
* Criteria for change —-50 years or older , bilateral ageing for one month and more in-
volving 2 of the following neck torso shoulder proximal arm hips proximal thigh ESR
>40
* Criteria of Healey — pain persist >1hour , rapid response to prednisone 20mg , ab-
sence of other disease capable of causing , musculoskeletal symptoms
* Funduscopic appearance in a patient with giant cell arteritis in whom blindness has
developed
Case 23
Takayasu arteritis
* large vasculitis affect women during their reproductive years
* Aorta and its major branches , young women ,
* Absent pulse , bruit , claudication , hypertension , fever of unkown origin
* ESR elevated
* Most patient respond to prednisone
* Symptoms === chest wall pain , joint pain , myalgia, constitutional , malaise ,
fever , weight loss, cardiac , aortic regurgitation ,angina , congestive heart failure ,
vascular , bruit , claudication, hypertension , unequal arm blood pressure , aortic re-
gurgitation , visual abnormalities , stroke
* Age <40 ,limb claudication, decrease brachial artery pulse , unequal arm blood pres-
sure >10 , subclavian or aortic bruit
Case 24
Weger granulomatosis // granulomatosis with polyangiitis
* most common form of systemic vasculitis
* Small to medium vessels
* Affect both arterial and venous circulation
* Granulomatous inflammation , vasculitis ,necrosis
* Fatigue ,myalgias weight loss, fever , persistent upper respiratory tract and ear in-
fections that don’t respond to antibiotic therapy
* Orbital pseudotumor ,
* ANCA helpful for diagnosis
* Rhinorrhea , bloody brown nasal crusts ,nasal septal perforation saddle nose defor-
mity , hearing loss
* Strawberry gums, tongue or oral ulcers , uveitis
* Laboratory == cbc =normocytic anemia , leukocytosis ,,, electrolytes = hiperkalemia
in the setting of advanced renal dysfunction
* Liver function test can be elevated , urinalysis = hematuria ,proteinuria
* ANA negative ,,, , ESR dramatic elevation ,,,,
Case 25
Microscopic polyangiitis
* ANCA , ESR
* Most common cause of pulmonary renal syndrome of alveolar hemmorhage and
glomerulonephritis
* Fatigue myalgia weight loss fever , migratory arthralgia , arthritis , palpable
purpura , skin ulceration , alveolar haemorrhage , glomerulonephritis
* Manage = cyclophosphamide daily and glucocorticoids , azathioprine , methotrexate
Case 26
Eosinophilic granulomatosis with polyangiitis /// churn Strauss syndrome
* asthma eosinophilia and systemic vasculitis ,
* Allergic rhinitis and nasal polyposis ,reactive airway disease
* ANCA
* Asthma wheezing or diffuse high -pitched rales on expiration , eosinophilia >10 on
white blood cell
* Livedo reticularis and a cutaneous ulcer just superior to the the medial malleolus ,
Case 27
Polyarteritis nodosa
* necrotising inflammation of muscular arterioles and medium sized arteries that
spares the small blood vessels
* Not associate with glomerulonephritis ,can cause renovascular hypertension and re-
nal infraction through its involvement of the medium -sized intrarenal vasculature
* Fever weight loss malaise arthralgia lower extremity nodules and ulceration , intesti-
nal angina ,
* Nodular lesion
* An ulcer with scalloped border
* Mesenteric angiogram showing multiple micro aneurysms
* A wedge-shaped renal infraction
* ESR CRP
Case 28
Behcet disease
Triad of recurrent oral aphthous ulcers genital ulcer ( at least three time over 12
months) , ocular inflammation
* skin lesion erythema nods,
* can cause inflammation on any organ
* Age 25-35 years
* Blindness , central nervous system
* Topical and systemic
* Glucocorticoids , immunosuppressive drugs
Case 29
HSP hence schonlein purpura
* blood vessels of superficial dermis
* Ig A
*
* Bulls lesion with a purpuric component
* Palpable purpura with some superficial ulceration , right ankle swelling due to arthri-
tis
* Lab test - leukocytosis cbc normal ,, hyperkalemia ,,
Case 30
Juvenile arthritis
* under 16 years
* Swelling pain stiffness growth problem
* Eye problem
* Esr increase
* Ccp
* NSAID
* DMARD
* CORTECOSTEROID
