8. Crystal arthropathies Year 4 Internal Medicine Notes

8. Crystal arthropathies Year 4 · Internal Medicine Collection: 4TH YEAR ALL NOTES / IMED / IMED 4.3 / NGUGI -- Slide 3 CPPD deposition arthropathy is an inflammatory condition in which insoluble calcium salt (calcium crystals) deposit in joints Is most common in the elderly, occurring in 10–15% of persons aged 65–75 years and 30–50% of those 85 years.\nIn most cases this process is asymptomatic, and the cause of CPPD deposition is uncertain Crystals deposition - articular cartilage, synovium, entheses, tendons and ligaments – both axial and peripheral skeletal sites. Introduction Slide 4 The commonly affected joints\nthe knee and the wrist.\nother joints such as the shoulder joint, the ankle and the MCP joints can be involved\nIt can occur as monoarticular, oligoarticular, or polyarticular asymmetrically Slide 5 Forms of CPPD CPPD arthropathy may be:\nasymptomatic (Chondrocalcinosis) acute\n• subacute,\n•chronic\nIt can also cause acute synovitis superimposed on chronically involved joints.\n• Acute CPPD arthritis was originally termed pseudogout because of its striking similarity to gout. Slide 6 Risk Factors Mostly in the elderly\nAge Affected People over 85 years → 30%–60%\n 65 - 75 years → 10%-15% of <55 years→ uncommon unless there is a hereditary link Slide 7 Pathophysiology Overproduction od PPi enhanced activity of (ATP) pyrophosphohydrolase and 5’-nucleotidase, which catalyze the reaction of ATP to adenosine and pyrophosphate. Hypophosphatasia OA –\nThere are decreased levels of cartilage glycosaminoglycans that normally inhibit crystal formation ANKH mutation (GoF mutation) This stimulates PPi movement in chondrocytes is from the intracellular to the extracellular space → ↑deposition Slide 8 Increased chondrocyte metabolism\nIncreased levels of extracellular pyrophosphate\nDecreased Levels of proteoglycan Ectopic mineralization\nDeposition of crystals in joints\nPhagocytosis of the crystals Slide 9 Cont..... Age( 50) and osteoarthritis\nJoint injury Hemochromatosis Primary hyperparathyroidism Hypophosphatemia Hypomagnesemia Familial predisposition Slide 11 Clinical Features Acute CCP Arthritis Swollen tender joint\nJoint is warm and erythematous with a large effusion Joint pain that is worse in the morning\nFever is common\nIll looking and confused and confused patient The knee is most commonly affected, → wrist → shoulder → ankle → elbow Trigger factors: trauma, intercurrent illness, dehydration and surgery DDx: Septic arthritis + Gout Slide 12 Chronic CCP can mimic RA, though joint involvement patterns are generally different i.e wrists, MCP joints 2 and 3, ankles, shoulders with affected joints showing OA features Joint destruction and limited joint mobility Effusion and synovial thickening most apparent in knees and wrist Carpal Tunnel Syndrome Slide 13 Cont… In neck - crowned dens syndrome →Neck Pain →Affected neck rotation. Slide 14 Diagnosis Full haemogram and inflammatory markers\nArthrocentesis – Synovial Fluid Analysis\noften turbid and may be uniformly blood-stained - inflammation Polarised Microscopy CPPD crystals weakly positively birefringent Perpendicular light – Yellow Parallel light – Blue\nGram staining to R/O sepsis Slide 16 X ray- chondrocalcinosis - may show chondrocalcification or periarticular entheses calcification.\nScreen for secondary causes Slide 18 Management Acute CCP arthritis Attack duration (Few dys – 1 month) Ice packs Joint Elevation Joint aspiration NSAIDS Colchicine (0.5-2mg daily) Chronic CCP Crystal Arthritis Glucocorticoids – after ruling out infection Colchicine (0.5-1mg daily) – may prevent acute flare ups Treat underlying disease Slide 21 Introduction Basic calcium phosphate (BCP) deposition disease is caused by the deposition of hydroxyapatite or apatite crystals and other basic calcium phosphate salts (octacalcium phosphate, tricalcium phosphate) in soft tissues. The main affected sites are tendons, ligaments and hyaline cartilage in patients with degenerative disease, and skeletal muscle and subcutaneous tissues in connective tissue diseases. Hydroxyapatite( apatite ) is the primary mineral in bone and teeth Common with increasing age Usually asymptomatic\nM/C around shoulder and knee Slide 22 Rheumatic diseases associated with basic calcium phosphate deposition: Slide 23 Pathophysiology Under normal circumstances, inhibitors of mineralisation, such as pyrophosphate and proteoglycans, inhibit calcification of soft tissues.\nWhen these protective mechanisms break down, abnormal calcification occurs.\nIn most situations, calcification has no consequence, but when the crystals are released, an inflammatory reaction may be initiated, causing local pain and inflammation. Slide 24 Clinical manifestations Can be asymptomatic\nCan present with features of: Acute calcific periarthritis\nOccurs particularly in the supraspinatus tendon, other sites may also be affected i.e the tendons around the hip, feet and hands.\nThe presentation is with acute pain, swelling