arif k s presentation final 2

8/3/2019 Arif k s Presentation Final 2

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DR . ARIF K SAssociate Professor

Yenepoya Medical College


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p y g

CLASSIFICATION :

MONOSODIUM URATE CRYSTALARTHROPATHY GOUT

CALCIUM PYROPHOSPHATEDIHYDRATE CRYSTALSARTHROPATHY PSEUDOGOUT


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CALCIUM HYDROXYAPATITE CRYSTALSAND OTHER BASIC CALCIUM PHOSPHATECRYSTALS ARTHROPATHY

ASSOCIATED WITHCALCIFIC TENDINITIS,

ACUTE CLACIFIC PERIARTHRITIS,MILWAUKEE SHOULDER SYNDROME

MISCELLANEOUS .


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GOUT

Gout -latin word Gutta means a drop Gout is a term representing a group of diseases

found exclusively in human species thatinclude

An elevated serum uric acid concentration(Hyperuricemia)

Recurrent attacks of acute arthritis in whichmonosodium urate monohydrate crystals aredemonstrable in synovial fluid leukocytes.


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Aggregates of Sodium urate monohydratecrystals (tophi) deposited chiefly in andaround joints which sometimes lead todeformity.

Renal disease including glomerular, tubularand interstitial tissues .

Uric acid urolithiasis.


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Normal values

Uric acid (serum)

Males 3-7 mg/dl

Females

3-6 mg/dl

Children 3-4

mg/dl.


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Etiology of gout

Primary

Secondary


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Primary

Unidentified molecular defects

Partial HGPRT defeciency(Hypoxanthine guanine phosphoribosyl transferase)

PRPP Synthetase increased activity(Phosphoribosyl pyrophosphate)


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Secondary

Underexcretion of uric acid(10%)

Overproduction of uric acid(90%)


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Underexcretion of uric acid

Renal diseases polycystic kidneydisease, Lead nephropathy.

Diuretic therapy Accumulation of organic acids alcoholic

ketosis, starvation , diabetic ketoacidosis.


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Overproduction of uric acid

Glucose -6- phosphatase deficiency

Lesch-Nyhan syndrome.(completehypoxanthine guanine phosphoribosyl

transferase defeciency)

Fructose-1-phosphate aldolase deficiency


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Overproduction of uric acid

Increased nucleic acid turnover myeloproliferative and lymphoproliferativedisorders.

Excess ATP degradation Acutely illpatients eg. status epilepticus , excessalcohol consumption


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Drug induced

Diuretic therapy

Cyclosporine Lead intoxication IV Heparin


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Pathogenesis


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Tophi

They are the pathognomonichallmark of gout.

They are formed by largeaggregation of uric acidcrystals surrounded by aninflammatory reaction of macrophages, lymphocytes and

large foreign body giant cells,which may have completely orpartially engulfed the masses of crystals


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Acute arthritis

Characterised by denseneutrophilic infiltrate.

Monosodium uratecrystals in the synovium.

Synovium edematous andcongested and containsscattered lymphocytes,plasma cells,macrophages.


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Chronic arthritis

Due to repetitive precipitation of uratecrystals during acute attacks.

The urates form visible deposits in thesynovium.

The synovium becomes hyperplastic and

thickened by inflammatory cells and formspannus that destroys the underlyingcartilage leading to bone erosions.


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Clinical features


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Acute

Sudden onset.

Affected joint hot, red, swollen,extremely tender.


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Single joint involved (85-90%)

1. First metatarsophalangeal joint.

2. Ankle

3. Knees

4. Wrist


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Mild attack may subside in several hours.

Severe attack may last for weeks.


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Intercritical gout

Interval between gouty attacks

Complete recovery

Interval between first two attacks may varyfrom 6 months to several years(5-10years).


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CHRONIC

Polyarticular. Marked limitation of joint movements.

The crystals may assume large globular

shapes, distending the overlying skin and

rupturing to form a chronically discharging

sinus.


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Spine involvement

Facetal joints will be involved Cause of chronic back pain Usually no neurological involvement


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Associated conditions

Hypertension Atherosclerosis Pregnancy Acute illness Hypothyroidism


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Renal parenchymal disease

Precipitation of uric acid crystals in

Collecting ducts Ureters Urolithiasis.


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Diagnosis

Family history of goutyarthritis

Repeated attacks with intervalof freedom from pain Renal disturbance such as

renal calculi Hyperuricemia Satisfactory response to

adequate doses of colchine.


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Investigations


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Serum uric acid levels will be elevated

Systemic signs leukocytosis, elevated

ESR.


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Synovial fluid

CloudyIncreased cell count (2000-60000) Microscopic examination

Crystals which show negative birefringencewhen viewed under polarizing microscope.


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Murexide test

Suspected substance containing uric acid +nitric acid mixture evoporated to dryness .

Ammonia added to the mixture purplecolour indicates presence of uric acid.


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Radiological appearance

Joint effusion. Periarticular soft tissue swelling. Preservation of joint space. Absence of periarticular demineralization. Overhanging bony edges. Sclerosis of bone margins.


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TREATMENT OF ACUTE GOUT

REST Immobilization of the affected limb and

icepack to reduce pain.


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Diet restriction- Food rich in purine should be avoided

eg mussels, yeast, sardines, sweetbread,liver, turkey, goose.


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Drugs Colchicine

Orally 0.5 to 0.6 mg tablet is givenevery hour until

1. Pain is reduced and toxic featuressubsided.

2. omiting and diarrhea develops or amaximum of 10 doses is taken.Intravenously 1-2 mg is given as aninitial dose followed by an additional 1mgafter 6 hours.


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Mechanism of action Inhibits migration of

inflammatory cells into the joint.

Toxic effects nausea, vomiting, diarrhea,abdominal pain, bone marrow suppresion,renal complication(proteinuria,hematuria)


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Drugs used in chronic gout

Allopurinol.

Uricosuric agents.


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ALLOPURINOL

Dose Initial daily dose of 100 mg. Most patients are maintained at 300 mg/day. Those with more severe gout may require

400-600 mg/day.


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Mechanism of action -Xanthine oxidase inhibitor.

Adverse effects 1. Hypersensitivity reactions2. Fever, myalgia3. Hepatomegaly


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Uricosuric agents

Probenecid 250 mg twice daily increased over 1-2weeks to 500 to 1000 mg twice daily.

Mechanism of action Increases urinary excretionof uric acid by inhibiting reabsorption.

Adverse effects

1. Nausea2. Vomiting


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Sulfinpyrazone 100 200 mg twice daily.

Mechanism of action Inhibits renaltubular reabsorption of uric acid.

Adverse effects Nausea , vomiting.


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Newer drugs :

FEBUXOSTAT, orally administered, non purine,selective inhibitor of xanthine oxidase, indicated inchronic hyperuricemia

MOA : inhibition of xanthine oxidase

Dose : 80 or 120mg orally once daily for atleast 6 month

Adverse effects : liver function test abnormalities,diarrhea, headache, rashs


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Managament of nephrolithiasis

Increased fluid intake to maintain large

urinary volume .

The treatment of choice in nephrolithiasis is

allopurinol because it lowers both urinaryand serum uric acid values.


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Surgical management

Indications 1. Pain2. Interference with movement of joint or tendon3. Discharging sinus4. Cosmetic

Surgical intervention mainly involves excision of the lesion.


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INTRODUCTION

Calcium pyrophosphate dihydrate

disease (CPPD) is a disorder due toprecipitation of calcium pyrophosphate

dihydrate crystals in the connective

tissues.


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PSEUDOGOUTAdams-1850- First case of CPPD

ACUTE ARTHRITIS caused by release of

CPPD crystals into joint space Monoarticular

Takes a more slower clinical progressionwhen compared to gout


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OTHER NAMES

CHONDROCALCINOSIS ARTICULARIS

PYROPHOSPHATE ARTHROPATHY CPPD

CALCIUM PYROPHOSPHATE GOUT


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PATHOGENESIS


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PATHOGENESISThe exact pathology of CPPD is unknown,

Increased Adenosine Triphosphate BREAKDOWN

A CHROMOSOME T(2;10) has been described in one

case involving the temporomandibular joint.


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CPPD crystals are

PHAGOCYTOSED by

neutrophils

This results in release of

LYSOSOMAL

ENZYMES andchemotactic factors


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CPPD: ASSOCIATEDDISEASES

DEFINITELY ASSOCIATED

HYPERPARATHYROIDISM HYPOPHOSPHATEMIA

HYPOMAGNESEMIA

HEMOCHROMATOSIS

WILSONS DISEASE


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POSSIBLY ASSOCIATED

HYPOTHYROIDISM

GOUT

OCHRONOSIS

CPPD: ASSOCIATED DISEASES


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PATHOLOGY IN ACUTE FORM

Pyrophosphate deposit usually occur in the

MIDZONE OF HYALINE CARTILAGE

Rarely seen in tendons and bursae

Synovial CHALKY WHITE DEPOSITS

Chondrometaplasia


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PATHOLOGY IN CHRONIC FORM

Synovium with increased FIBROSIS

Mononuclear cell INFILTRATES

GIANT CELL surrounding crystalline deposits

Foreign body GRANULOMA

Deposits occur OUTSIDE the chondrocytes


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CLINICAL PRESENTATION


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COMMON SITES

KNEE> WRIST> MCPS>


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CLINICAL

CPPD has the capacity to mimic ANY TYPE OF

ARTHIRITIS

ASYMPTOMATIC DISEASE

Most joints with radiographic evidence of CPPD

deposition have mild to no symptoms of disease


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CLINICAL

Acute pseudogout

PSEUDO - rheumatoid pattern

PSEUDO - osteoarthritis

Chondrocalcinosis

Neuropathic joint


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PSEUDOGOUT

MONOARTHRITIS Commonly

Signs of severe acute INFLAMMATION

Occurrence of CLUSTER ATTACKS

Resembles gout

DIFFERENTIATE from gout

SLOWER IN PROGRESSION compared to gout


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PSEUDO-RHEUMATOID ARTHRITIS

5%

10%- positive RA factor

Polyarthritis

Symptoms lasting for months

Symptoms include significant morning stiffness, fatigue,

synovial thickening, localized edema, and restricted

joint motion


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PSEUDO-OSTEOARTHRITIS

50 %

The joints most commonly affected include the knees,

followed by the wrists, MCPs, hips, shoulders, elbows,and spine

DIFFERENTIATE from primary osteoarthritis

More likely to affect the lateral compartment knee

Patellofemoral arthritis

Uncommon site


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DIAGNOSIS


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SYNOVIAL FLUID ANALYSIS

CLOUDY HIGH CELL COUNT INTRACELLULAR AND

EXTRACELLULAR CRYSTALS


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DIAGNOSIS

Synovial fluid

POSITIVELY BIREFRINGENT rhomboid crystals

During acute pseudogout attacks phagocytosed crystals

within PMNs ARE FOUND


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Negative Birefringence


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CPPD-Arthroscopic View


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CHONDROCALCINOSIS

Punctate and linear radiodensities in fibrocartilage and

articular cartilage

RADIOGRAPHIC FEATURE


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RADIOGRAPHIC FEATURES General similarities to osteoarthritis

Chondrocalcinosis--calcification of cartilage

MENISCI of knees

CARTILAGE of wrists

SYMPHYSIS PUBIS


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Other sites demonstrating calcification

Bursae Joint capsule

Synovium

Tendon

RADIOGRAPHIC FEATURES


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Diagnosis

Chondrocalcinosis of knee joint


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WRAP AROUND appearance of patella


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DIAGNOSTIC CRITERIA

Definitive diagnosis of CPPD deposition requires

Demonstration of CPPD crystals in synovial fluid by x-ray

diffraction ,

or

POSITIVELY BIREFRINGENT CRYSTALS seen by

compensated polarized light microscopy


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DIAGNOSTIC CRITERIA BY MCCARTY


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1. Demonstration of CPPD crystals obtained by

joint aspiration or biopsy by infrared

spectroscopy

2. (a) Identification of positive birefringence bycompensated polarized light microscopy

(b) Presence of typical calcifications of

fibrocartilage and hyaline cartilage on radiographs


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3.(a) Acute arthritis, especially of knees or other large

joints

(b) Chronic arthropathy , especially of knees, hips, wrists,

carpus, elbows, shoulders, and metacarpophalangeal

joints


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Definite diagnosis : Criterion I or IIa

Probable diagnosis : Criterion IIa or IIb

Possible diagnosis : Criterion IIIa or IIIb


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TREATMENT OPTIONS

Joint aspiration

NSAIDs

Colchicine (not as effective as for gout)

Steroids (not as effective as for gout)

oral

intra-articular

Analgesics

Surgery if necessary to preserve function

TREATMENT


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TREATMENT

Acute Pseudogout

Removal of crystals by joint aspiration

Administration of NSAIDs or colchicine Intraarticular injection of steroids

Joint immobilization


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TREATMENT

Colchicine

Blocks release of chemotactic factors forneutrophils and mononuclear cells and inhibits

neutrophil-endothelial cell binding


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PROGNOSIS

ANKYLOSIS

CARPAL TUNNEL SYNDROME

DEFORMITIES

DEMINERALIZATION

STRESS FRACTURES

CALCIUM PHOSPHATE


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APATITE CRYSTAL

DEPOSITION DISEASE

Carbonate substituted apatite

Octacalcium phosphate

Tricalcium phosphate dihydrate

Dicalcium phosphate dihydrate


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PRESENTATION

Clinically similar to cppd and gout

Calcific tendinitis bursitis

Acute arthritis

Joint Destruction


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PRESENTATION

They are below the limits of resolution of

ordinary optic microscope Aggregate as irregular glossy clumps

Do not show any chracteristic crystallineshape


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DIFFERENTIAL DIAGNOSIS

MONOARTHRITIS


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InfectionRheumatoid arthritis

Gout

PseudogoutApatite-related arthropathy

Reactive arthritis

SLESickle cell disease

Transient synovitis of the hipMetastatic carcinoma

Pigmented villonodular

synovitisHemarthrosis

Neuropathic arthropathy

OsteoarthritisIntra-articular injury

.

DIFFERENTIAL DIAGNOSIS FOR


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DIFFERENTIAL DIAGNOSIS FORCHONDROCALCINOSIS

Systemic sclerosis

Dermatomyositis

Polymyositis

Systemic lupus erythromatosis

Hemodialysis

Heterotrophic calcification


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Thank you

arif k s presentation final 2

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