Crystal arthritis

CRYSTAL ARTHRITIS Dr. M Kasi Viswanadham PG in Orthopaedics

GOUTword Gout is derived from the latin word gutta means drop.

based on the ancient belief that the arthritis is due to deposition of malevolent humor by evil spirits into the joint, drop by drop

Gout is a disorder of purine metabolism characterized by hyperuricaemia, deposition of monosodium urate monohydrate crystals in joints and peri-articular tissues and recurrent attacks of acute synovitis.

Late changes include cartilage degeneration, renal dysfunction and uric acid urolithiasis.

history 2640 BC: podagra first identified by the Egyptians

5th century BC: Hippocrates referred to gout as unwalkable disease and noted links between gout & lifestyle, demographics & other variables

Galen described the tophi.

Crystals in gouty tophi was first demonstrated by Antony Van Leeuwenhoek in (1679 )

Sir Alfred Garrod demonstrated hyperuricemia as the basic cause of gout(1848)

McCarty and Hollander established the association between gouty arthritis and articular crystal deposition(1961)

Gout is much more common in men than in women; rare before menopause and more common in old ageMale ,female ratio 2.7:1

pathogenesisThe pathogenesis of gout depends on three vital steps: (1) the development of hyperuricaemia; (2) the formation of monosodium urate monohydrate (urate) crystals; (3) the interaction between the urate crystals and the inflammatory system.

This disorder can be progressive through four stages if undertreated1. Asymptomatic hyperuricemia2. Acute gout3. Intercritical gout4. Chronic tophaceous gout

Asymptomatic hyperurecemiaAsymptomaticSerum uric acid levels-more than 6mg/dlrisk of gout increases with the degree and duration of hyperuricaemia. Acute gout most common early clinical manifestation Usually, only one joint is affected initially, but polyarticular acute gout can occur in subsequent episodesFactors provking manifestations-. alcohol, obesity abrupt change in serum uric acid concentration Diuretic use, hypertension, renal insufficiency, and osteoarthritisMost acute attacks of gout involve a single joint in the lower limb, most commonly the first metatarso phalangeal joint also referred to as podagra.

Others-ankle, heel, knee, wrist, fingers, and elbow jointsSymptoms-severe pain,Swelling ErythemaWarmthTendernessLow grade fever Starts during night peak 1-2 days.last for 7-10 daysDifferential diagnosis-septic arthritis,cellulitis

Acute gout

Intercritical gout intercritical period is the time between the acute attacks;the term prophylactic period has been preferred because its emphasizes the value of medication which inhibits or prevents recurring acute episodes. Chronic tophius goutMassive deposition of monosodium urate crystal(tophi) in the articular cartilage, subchondral bone, synovial membrane, capsular, periarticular tissue and tendon sheaths.Occurs late (an avg 12 yrs)after intial attack

Chronic gout

Tophaceous nodule consist of multicentric deposition of urate crystal ,inter cellular matrix and foreign body grannulomatous reaction.Tophi firm yellow in colour occsionally discharge cheesy or chalky white material. +

o

Complications of tophi gout-pain,soft tissue damage and deformity,joint destruction, nerve compression syndromesRenal manifestation of goutNephrolithiasisActe gouty nephropathyChronic gouty nephropathydiagnosis

Base line lab tests-urine analysis renal function testsSerum urate- normal value is 6-7 mg/dl (6.8mg/dl)

an elevated serum uric acid level does not indicate or predict gouthyperuricemia in the absence of symptoms is not diagnostic of gout. diagnosed based on the discovery of urate crystals in the synovial fluid or soft tissues.

radiographyEarly in the disease, radiographs are often normal or show only soft-tissue swelling

Radiographic findings characteristic of gout, which generally do not appear within the first year of disease onsetAsymmetrical Punched out erosionLytic lesionsOverhanging edgesScleorotic bordersDiffer from rhematoid arthrits Maintenance of the joint space Absence of periarticular osteopenia Location outside the joint capsule

identification of urate crystals the gold standard for conformation of the diagnosis

identification of urate crystals in synovial fuid using polarized light microscopy is highly specific for gout.examined with a polarizing filter, they are yellow when aligned parallel to the slow axis of the red compensator, but they turn blue when aligned across the direction of polarization (ie, they exhibit negative birefringence).

Histology Sample send in 100% alcohol

ultrasoundA "double contour" sign, consisting of a hyperechoic, irregular line of monosodium urate crystals on the surface articular cartilage overlying an adjacent hyperechoic bony contour"Wet clumps of sugar," representing tophaceous material, described as hyperechoic and hypoechoic heterogeneous material with an anechoic rimBony erosions adjacent to tophaceous deposits

Differential diagnosis acute gout= infective (septic) arthritis; traumatic synovitis; palindromic rheumatism seronegativspond-arthritidesRhematic fever

d Differential diagnosis of chronic goutnodular rheumatoid arthritis; osteoarthritis with Heberdens / Bouchardsnodes;

Management

Treatment The acute attackResting the jointApplying ice packs if pain is severe,NSAIDsColchicine (may cause diarrhoea, nausea and vomiting)A tense joint effusion may require aspiration and intra-articular injection of corticosteroids. Oral corticosteroids. The sooner treatment is started the sooner is the attack likely to end. Interval therapy Between attacks, attention should be given to simple measures such as losing weightcutting out alcohol and Eliminating diuretics. Chronic goutUricosuric drugs (probenecid or sulnpyrazone) can be used if renal function is normal. Allopurinol a xanthine oxidase inhibitor, is usually preferred for patients with renal complications or chronic tophaceous gout allopurinol is denitely the drug of choiceUrate-lowering drugs should never be started before the acute attack has completely subsided, and they should always be covered by an anti-inammatory preparation or colchicine, otherwise they may actually prolong or precipitate an acute attack.SurgeryUlcerating tophi that fail to heal with conservative treatment can be evacuated by curettagethe wound is left open and dressings are applied until it heals. Calcium pyrophosphate dihydrate (CPPD) crystal associated arthropathies idiopathic presents as sporadic episodes in the majority of patient rare familial formsThe autosomal dominant form of the disease has been shown to be related to a mutation in the ANKH gene, which encodes a transmembrane inorganic pyrophosphate transport channel

association with some metabolic disorders- hyperparathyroidism, haemochromatosis, hypophosphatasia, Wilsons disease, ochronosis, hypo-calciuric hypercalcaemia diabetes mellitus hypomagnasaemia intra-articular injections of hyaluronic acid preparations (such as Hylan GF-20) may trigger acute attack of pseudogout

o Age is most important risk factor

Osteoarthritis (OA) - threefold increased risk if CPPD present

Previous joint trauma/injury

Joint surgery/lavage promotes crystal sheddingIncidence and prevalence uncertain

Strong association with age prevalence 3.7% in age 55-59 17% in age 80-84%

CPPD associated arthritis is third most common inflammatory arthritis

most common acute mono-articular arthritis in elderly; typically involves the kneepathogenesisNot knownDisorder of articular cartilage with altered chondrocyte cartilage matrix abnormalities,altered activity of enzymes that produces pyrophoshates which combines with calcium ions to form CPPD crystals

The deposits are often visible as a fine layer of calcification overlying the menisci and articular cartilage of the knee- termed chondrocalcinosis

The crystals may provoke an acute attack of synovitis- referred to as pseudo-gout.

Pseudo gout Clinical syndrome of acute synovitis with intraarticular (IA) Calcium pyrophosphate dehydrate (CPP) crystal deposition

o Most common joints knee and wrist

Chronic form referred to as calcium pyrophosphate deposition (CPPD) Arthropathy

Increases development of osteoarthritis

Chondrocalcinosis Asymptomatic Incidental findingPseudogout is the most common form

presents as acute episodes of synovitis at a single joint, most often at the knee.

More common in men, the attack typically lasts from a few days to 3-4 weeks

characterised by acute pain, swelling and warmth at the affected joint, often indistinguishable from gout.

Chronic CPPD: predominately affects women; it is a progressive, often symmetric, polyarthritis.Usually affects the knees, wrists, 2nd and 3rd MCPs, hips, spine, shoulders, elbows and ankles.Chronic CPPD differs from pseudogout in its chronicity, involvement of the spine and MCPs.

Radiograph of involved joint Chondrocalcinosis (CC) = linear densities in joints; best seen on films of pelvis, hand and knee Only detects 40% articular CPPD disease

Subchondral cysts Aggressive joint degeneration; osteophyte formation

Arthrocentesis

Inflammatory Synovial Aspirate: mean leukocyte count of 24,000 cells/L with neutrophil predominance

Gram stain and culture necessary to rule out infectious arthritisScreening for metabolic disordersGold standard: synovial fluid analysis under polarized light microscopy with rhomboid crystals, absent/weak positive birefringencepolarizing filter

CPP crystals change color depending upon their alignment relative to the direction of the red compensator.

They are positively birefringent, appearing blue when aligned parallel with the slow axis of the compensator and yellow when perpendicular.

The sensitivity of a synovial fluid analysis for crystals is 84%, with a specificity of 100%.

Ultrasound CPPD in peripheral joints appears as punctuate pattern with thin hyperechoic deposits Specificity 86.7%, Sensitivity 96.4%; Sensitivity varies with joint Most accurate in mild cartilage degeneration

MRI - insensitive for articular CPPD CT sensitive for CPPD; however not specific

Differential diagnosiso Gout - negative birefringent monosodium urate crystals; elevated uric acid; first attack usually in foot

o OA: may be associated with CPPD; chronic symptomso RA : positive serology (Rheumatoid factor, Anti-citrullinated peptide antibody) and elevated acute phase reactants; morning stiffness; polyarticular; symmetric arthritis of wrist, hand or finger joints; rheumatoid nodules; Symptoms >6 weeks

Septic Arthritis: SF analysis with turbidity, >10,000 PMNs low glucose and elevated lactic acid; positive Gram stain and cultureo Trauma treatment-o Iceo Resto Joint aspirationo Intraarticular glucocorticosteroids o Oral Nsaidso Cyclooxygenase-2 selective agentso Colchicineo Parenteral Adrenocorticotropic HormonePrognosis1. Acute attacks self-limited, resolve 7-10 days

2. Progressive joint damage; destructive arthropathy resembling neuropathic(Charcots) joints

3. Large joint arthropathy may require joint replacement

II. Basic calcium phosphate hydroxy-apatite (BCP) crystal associated arthropathies:

The BCP crystals includes- hydroxy-apatite, octacalciumPhosphate and tricalcium phosphateManifestations-Periarthritis / tendonitisMilwaukee shoulder syndrome (MSS)OsteoarthritisErosive arthritis

Milwaki shoulder syndromemore common in women

affects a single joint- most often the shoulder, or the knee.

there may be a history of preceding trauma or overuse of the joint.

There are marked degenerative changes on radiology, with the presence of loose bodies and calcification

BCP crystals is difficult and the diagnosis mayeventually depend upon excluding other causes such as gout or septic arthritis.

Thank you

The serum urate concentration may reduce during an acute attack; a normal urate concentration at this point does not rule out a diagnosis of gout Eunuchs do not take gout, nor become bald. A women does not take gout unless her menses be stopped,An young man does not take gout unless he indulges in coitus. In gouty affection, inflammation subsides in 40 days hippocrates