Bone, Cartilage and Muscle Disorders Flashcards
Crystal Arthritis
•gout
•over production of or under secretion of uric acid
-under secretion: 90% of cases, kidney disease, hypertension, obesity, use of certain drugs/medications
-over production: high cell turnover states (myeloproliferative and lymphoproliferative neoplasms, psoriasis) and consumption of certain foods high in purines; genetic metabolic diseases -Lesch-Nyhan syndrome/Kelley Seegmiller syndrome (HGPRT deficiency), PPRP overactivity, glycogen storage diseases, mutations in specific kidney transporters (GLUT -9, ABCG2, URAT1)
•tophi
•podagra
•inflammasome
•diagnosis:
-clinical history and physical exam
-labs - CMP, BCB, ESR, CRP looking for signs of kidney disease and inflammation
serum uric acid can drop during attack - check in the absence of attack
-arthrocentesis and synovial fluid analysis - gram stain, culture, cell count and crystal analysis (needle shaped crystals, yellow) MUST RULE OUT SEPTIC ARTHRITIS AND INFECTION
-X-ray - punch out lesions, US - double contour sign, dual energy CT
•treatment:
-dietary changes
-NSAIDs —-> glucocorticoids ——> low dose colchicine (cont. for 6 months to prevent recurrence)
-IL 1 antagonists
-2 weeks after attack, prophylactic therapy to lower uric acid - hypourecemic agents like xanthine oxidase inhibitors that prevent uric acid formation Allopurinol, febuxostat
-drugs that prevent uric acid secretion - probenecid, sulfinpyrazone (limited use for patients with renal failure, risk of uric acid stones
-uricases - enzymes that occur in non primates that break down uric acid into the more soluble allantoin - pegloticase (IV at 2 week intervals, can cause antibody formation)
Pseudogout
•calcium pyrophosphate deposition disease (CPPD)
•can mimic gout, RA or OA
•may be an incidental finding on a routine radiograph in an asymptomatic patient
•sometimes associated with metabolic disease - functionality of alkaline phosphatase (Mg, Ca, Fe, Cu)
•associated with hemochromatosis (Fe), HPT (Ca), Wilson disease (Cu), hypomagnesemia (Mg)
•diagnosis:
-crystals deposited in tissue, including synovium and cartilage - rhomboid shaped, blue-green
-X-rays show fine line of calcification (chondrocalcinosis)
-fibrocartilage usually affected, examine wrists, knees, and symphysis pubis
•treatment:
-NSAIDs—->glucocorticoids—->colchicine for acute attacks and prophylactically
-hydrooxychloroquine, methotrexate, IL 1 antagonists
Systemic Lupus Erythematous
•autoimmune
•gene, environment, cell damage, immune function
•auto antibodies, immune complexes, elements from innate immune system
•related to Sjogren Syndrome, scleroderma, polymyositis/dermamyositis and mixed CT disease (MCTD) through a collection of ANAs
•dsDNA, Smith ANAs may be present
•women > men, African Americans, Hispanics
•ages 15-44
•diagnosis:
-clinically made - careful history and physical exam
-common symptoms- malar rash, photosensitivity, painless oral ulcers, inflammatory arthritis, serositis, haematologic, Raynaud’s, fatigue, myalgia, fever
-lab tests- +ANA (dsDNA or Smith), complement levels of C3 and C4 may be lower than normal
-malar rash usually spares nasolabial folds and triggered by sun exposure
-Jacobs’s arthropathy pain and deformity that resembles RA but lacks erosions
-lupus nephritis kidney damage related to circulating immune complexes (associated with hypertension and protein uric, WHO Class IV)
-Libman-Sack Endocarditis non bacterial vegetation’s on mitral or aortic valves
-neonatal lips - SSA, SSB causing fatal heart block in pregnant lupus patients
-drug induced lupus - procainamide, hydralazine, quinine, isoniazid, minocycline
•treatment:
-antimalarial - hydroxychloroquine
-NSAIDs, DMARDs, glucocorticoids for inflammation and fatigue
-Belimumab
-for lupus nephritis or other life threatening conditions, pulse steroids + cytotoxic agent (cyclophosphamide or rituximab)
•cutaneous lupus erythematous - acute, subacute and chronic
Sjogren’s Syndrome
•glandular lymphocytic inflammation ——. Decreased saliva (xerostomia) and tears (keratoconjunctuvitis sicca) —->tooth decay and cornea damage
•females in mid 50s
•primary or secondary (RA, lupus, scleroderma)
•can affect joints (inflammatory arthritis), kidneys (interstitial nephritis), gall bladder (cholangitis), lungs (interstitial fibrosis)
•SSA/SSB antibodies
•increased risk of lymphoma, esp MALT
•diagnosis:
-biopsy of inflamed glands
•treatment:
-directed at symptoms, for dryness, supportive treatment
-for inflammatory arthritis - similar to RA—->DMARDs
Scleroderma
•widespread fibrosis
•can involve almost every organ system, esp skin
•>females, associated with high morbidity and mortality
•Raynaud’s syndrome, thickened lightened skin (usually on hands, sclerodactyly)
•lungs (interstitial fibrosis, pulmonary hypertension), GI tract (pseudo obstruction, reflux), kidneys (scleroderma renal crisis) and heart (constrictive pericarditis, heart block)
•impaired micro vascular blood supply —->tissue hypoxia and fibrosis
•diagnosis:
-clinical symptoms and physical exam
-antibodies to centromere and SCL-70
-patients with Raynaud’s Syndrome —-> dilated nailfold capillaries (mailfold capillarascopy)
-2 forms - limited (+ centromere antibody, organ fibrosis limited to several systems, CREST, slower disease course, pulmonary hypertension, better prognosis) and diffuse (+SCL-70, widespread fibrosis —>organ failure and death, rapid disease course, pulmonary fibrosis)
•symmetric cutaneous sclerosis, finger swelling, sclerodactyly, digital pits and ulcers, telangiectasia, calcinosis cutis, hyperpigmentation and ischemic ulcers in finger and toes
•skin involvement differentiates the subtypes: diffuse, limited (CREST) and localised (morphea)
Polymyositis/Dermatomyositis
•immune system attacks skeletal muscle
•part of a group called “inflammatory myopathies” —->muscle destruction and weakness, usually proximal muscle groups (shoulders, anterior thighs), other muscle groups (upper esophagus, diaphragm, and intercostal muscles)
•muscle weakness, dysphasia, shortness of breath, Raynaud’s Syndrome, calcinosis (esp. in children)
•diagnosis:
-biopsy
-lab work - CK, Aldo last, LDH, AST, ALT, ESR, CRP (all elevated)
-electromyelogram (EMG), nerve conduction velocity (NCV), and MRI
-anti-Jo -1 antisynthetase syndrome (arthritis, fevers, Raynaud’s, mechanics hands)
-anti-SRP signal recognition peptide (acute and sever PM)
-anti-Mi-2 classic DM
-PM weakness and muscle biopsy will show endomysial inflammation
-DM weakness associated with a rash on sun exposed areas
-Gottron papules, Gottron sign, heliotrope rash, shawl sign, poikiloderma atrophicans vasculare, periungual telangiectasia, dystrophic cuticles, calcinosis cutis
-malignancy and adenocarcinomas (breaststroke, lungs, GI, ovary)
•treatment:
-glucocorticoids—-> +azathioprine, methotrexate, rituximab, IVIG, mycophenolate, or tacrolimus
-MALIGNANCY should be treated first, symptoms will resolve following cure of cancer
Mixed CT Disease
•similar to PM/DM, scleroderma, Sjogren’s •diagnosis: -U1 RNP antibody •treatment: -directed at symptoms
Polymyalgia Rheumatica (PMR)
•systemic inflammatory condition of unknown etiology
•severe fatigue, fever, weight loss, proximal aches and pains
•>50 years of age
•associated with Giant Cell Arthritis —>blindness if untreated
•can mimic PM/DM and rhabdomyolysis
•diagnosis:
-ESR, CRP elevated, CK normal
•treatment:
-glucocorticoids —-> DMARDs (methotrexate and tocilizumab)
Inflammatory Arthritis
- joint pain the result of inflammation and inflammatory processes that originate in both innate and adaptive immune systems
- DIFFERENT from non-inflammatory arthritis which are driven by cartilage damage, degradation, and aberrant repair of cartilage
- RA, gout, spondyloarthritis (ankylosing spondylitis, psoriatic arthritis, enteropathic arthritis which are also known as seronegative spondyloarthropathies), lupus and many more
Rheumatoid Arthritis
•most common inflammatory arthritis
•Pima Native Americans 5%
•genetics and environment
•females>males null parity, recent birth, family history, smoking
•acute or chronic polyarthritis of small joints
•RF antibodies, anti citrus instead peptide antibodies —-> PTPN22, PADI-4, and HLA-DRB subgroups are some of the genes involved
•major cell types involved - macrophages, T cells, B cells
•major cytokines involved - IL-1. TNF, Il 6
•immune response in joint synovium —-> joint synovium proliferates —-> inflammation causes increase in bone turnover —> bony erosions at margin of epiphysis (related to increased osteoclast activity)
•morning stiffness >1 hr
•low grade fever, malaise, fatigue
•pain improves with activity
•joint swelling, warmth, tenderness in wrists, MCP, PIP —->ulnar deviation, swan neck deformity, boutonnière deformity, z-deformity of thumb
-elbows, shoulders, knees, ankles, MTPs
-C1-C2
-usually symmetric
-anemia, rheumatoid nodules, Felly’s syndrome (neutropenia with splenalomegaly), AA amyloidosis, scleritis, interstitial lung disease, mononeuritis multiplex, rheumatoid neutrophilic dermatosis, pyoderma gangrenous, interstitial granulomatous dermatitis, erythema multiforme, livedo reticularis and vasculitis
•diagnosis:
-clinical findings in history and physical exam + small joint synovium
-lab work - CBC, CMP, CRP, ESR, RF, CCP (CCP more specific than RF)
-radiographs - joint space narrowing (usually symmetric), marginal erosions, periarticular osteopenia
-musculoskeletal US is used to detect early erosions and synoviti (increased vascularity) within joints
•INFLAMMATORY ARHRITIS ≠ RA
•treatment:
-NSAIDs —-> glucocorticoids ——>DMARDs (traditional or biologic)
Seronegative Spondyloarthropathies
•inflammatory arthritides
•ankylosing spondylitis, psoriatic arthritis, reactive arthritis, enteropathic arthritis
•different from RA-
1)-RF
2)inflammation involves large joints (knees, hips, shoulders) and the spine
3)HLA B27
•inflammatory BACK PAIN and extra articulate manifestations in the eye and skin
Inflammatory Back Pain
- inflammatory arthritis in the spine
- > 3 months and associated stiffness >1 hr
- pain worsens at night and may wake patient
- worsens with immobility and improves with activity
- <40 years of age, reduced spinal mobility with time
- sacroiliac joint may become inflamed —-> buttock pain
- ask about LOCATION and quality of pain and morning stiffness
RED FLAGS for Back Pain
- 1- Trauma
- 2- Cancer especially Prostate, Breast, Lung Cancer (metastasize to spine)
- 3- Fever, chills (epidural abscess, osteomyelitis)
- 4- Immunocompromised (TB spine)
- 5- Osteoporosis (primary or secondary )
- 6- IV drug use/sepsis (epidural abscess)
- 7- Over 6 weeks no improvement
- 9- Sickle Cell Disease (osteomyelitis, osteonecrosis)
- 10 Pain not relieved by medications
Ankylosing Spondylitis
•most common inflammatory disorder of the axial skeleton
•begins at adolescence or young adulthood
•large joint oligoarthritis (shoulder, hip, knee), inflammatory back pain and sacroilitis
•can lead to loss of mobility in cervical spine, lumbar spine and chest, fusion of spine
•>males
•HLA B27
•unilateral anterior uveitis, aortic insufficiency, and pulmonary fibrosis
•diagnosis:
-history and physical (reduced spinal mobility)
-Schöber’s test, chest expansion, occipital to wall measurement
-labs-ESR, CRP (elevated)
-radiographs and MRI
•treatment:
-NSAIDs—->biologic DMARDs (not traditional)
Psoriatic Arthritis
•develops in 5-7% of patients with psoriasis
•30-55 years of age
•men = women
•most cases psoriasis precedes
•may coexist with HIV to cause severe joint destruction
•5 different patterns of joint involvement-oligoarthritis, DIP, RA-like, spondylitis, arthritis mutilans
•nail changes including pitting, oncholysis and onchomycosis
•diagnosis:
-clinical
-labs - CRP (high), +HLA B27
-radiographs - erosions, periostitis, asymmetric sacroilitis, pencil in cup
•treatment:
-NSAIDs—->traditional DMARDs —-> biologic DMARDs
-topicals don’t help with joint pain
•papulosquamous plaques on the scalp and extensor surfaces of the body and nail dystrophy
•common subtypes: plaque, guttate, nail and pustular
•uncommon subtypes; generalised pustular, erythrodema, pustulsosis
