Exam 7 Flashcards
HLA common type
B27, usually class II MHC
Central tolerance
Cells expressing auto antibodies are marked and destroyed through apoptosis
Clonal anergy
Inactive T cells that have not encountered antigen
Immunological ignorance
Lack of antigen antibody encounter
Active regulation
Surveillance for self reacting antibodie
Rheumatoid arthritis definition
Chronic and systemic inflammatory dz affecting synovial tissues
Synovitis of multiple joints
Genetic risk factor for RA
HLA with shared epitope, 4x great risk of RA, but only present in 30% of population
How long are autoantibodies present in RA before onset of sx?
5-10 years
RA pathophysiology
Synovial fluid increases as synovial tissue proliferates, pannus develops
Pannus infiltrates adjacent structures and destroys them
RA joint symptoms
Early pain and stiffness
Symmetric swelling and pain of multiple joints
Worse with activity, worse in morning
Joint deformities in RA in the hands
Swan neck- hyperextension of PIP
Boutonnière- hyperflexion of PIP
Ulnar deviation of MCP
Other RA symptoms
*constitutional symptoms
Rheumatoid nodules (over bony prominences, bursae, tendons)
Dry eye, scleral nodules
Felty syndrome
Found in RA
RA, splenomegaly, neutropenia
RA diagnostics
Anti-CCP (most correlated and more specific)
RF (not as specific)
RA imaging
Early- soft tissue swelling of wrists and feet, juxta-articular demineralization
Late- joint space narrowing, bony erosions
What does RA have that OA doesn’t?
MCP involvement Systemic symptoms Extra articular symptoms Persistent morning stiffness Symmetrical joint involvement Erosions on x rays Increased ESR, CRP, RF, and anti-CCP
What is more specific to OA vs. RA?
Asymmetrical joint involvement
Osteophytes on x ray
3 types of juvenile idiopathic arthritis
Oligoarticular
Polyarticular
Systemic onset
What is an ocular risk of all JIA patients?
Uveitis
JIA diagnosis
Effusion noticed acutely by parents
Pain and stiffness that restricts motion
Arthritis onset slow
JIA exam
Inflammation, erythema, tenderness, effusion
Still disease general
Related to chronic juvenile arthritis
Young adult onset
Still disease symptoms
High fevers
Sore throat
Evanescent rash
Destructive arthritis
Dramatic increased WBC, ferritin
What are the lupus criteria?
- Malar rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis
- Serositis
- Kidney disease
- Neurological disease
- Hematologic disorders
- Immunologic abnormalities
- Positive ANA
How many criteria does a patient need to be diagnosed with lupus?
Any 4 or more of the 11
Systemic sx of lupus
Fever, anorexia, malaise, weight loss
Malar rash
SLE
Butterfly rash, red/purple, mildly scaly, spares nasolabial folds
Discoid rash
SLE
Erythema, inflammation, scaling/crust. May scar
Photosensitivity of SLE
Rash in sun exposed areas
What may be the earliest sign of SLE?
Arthritis
Serositis in SLE
Inflammation of serous tissue
Pericarditis, pleural effusions common
Screening test of choice for SLE?
ANA!
If it is negative, need to consider other dx’s
Polymyositis
Insidious proximal muscle weakness and maybe pain
Dysphagia fairly common
Polymyositis diagnostic
Muscle biopsy
White blood cells attacking muscle cells
Dermatomyositis
Similar weakness to polymyositis
Heliotrope rash
Gottron papules
Shawl sign
Rhabdmyolysis
Acute necrosis of skeletal muscle
Very increased CK levels
Renal failure
Common causes of rhabdo
Crush injury
Cocaine, alcohol
Prolonged inactivity
Statins
Scleroderma types
Diffuse and limited
Scleroderma
Fibrosis of skin and internal organs
CREST syndrome
Aka limited scleroderma
Calcinosis cutis Raynauds Esophageal motility disorder Sclerodactyly Telangiectasia
Diffuse scleroderma manifestations
Tendon friction rubs over forearm, shins
Renal, cardiac dz
Interstitial lung dz
Tightening of skin
Tightening of skin with CREST
Just face and hands, greater risk of digital ischemia
What autoantibody marker does CREST have more commonly than diffuse scleroderma?
Anti-centromere
Sjogren syndrome
Dry eye, dry mouth
Usually females
Schirmer test to quantify tears
Polymyalgia rheumatica
Pain/stiffness in axial muscles (shoulders, hips/pelvis)
Associated with giant cell arteritis
Difficulty putting on coat, standing from chair
Behcet disease
Oral aphthae is the hallmark
Genital lesions, CNS involvement, vasculitis, arthritis
Uveitis- severe!
Behcet disease diagnosis
Recurrent oral ulcerations plus 2 more things, like…
Genital ulcerations
Eye lesions
Skin lesions
Positive pathergy test
Pathergy
Behcet diagnosis
Formation of a sterile pustule at the site of needle insertion
Polyarteritis nodosa
Medium vessel disease
Skin, nerves, mesenteric vessels, brain, etc.
Polyarteritis nodosa manifestations
Livedo reticularis
Digital gangrene
Primary angiitis of CNS
Rare vasculitis limited to brain and spinal cord
Brain bx is only way to make dx
Leukocytoclastic vasculitis
Small vessel vasculitis
Most commonly of skin, aka hypersensitivity vasculitis
Leukocytoclasatic vasculitis exam
Palpable purpura
Urticarial lesions- last greater than 24 hours, burning more than itching
Henoch schonlein purpura
Most common systemic vasculitis in kids
Palpable purpura, arthritis, hematuria and maybe abdominal pain
Essential cryoglobulinemia
Hep C is the most common cause
Cold precipitable immune complexes
Essential cryoglobulinemia findings
Palpable purpura
Peripheral neuropathy
Glomerulonephritis
Positive serum test for cryoglobulins
Osteoarthritis general
Most common joint disease
Degenerative dz due to cartilage breakdown in joints, bony and synovial changes
What joints does OA commonly affect?
Knee
Hip
Spine
Feet
Weight bearing joints!
Asymmetrical
OA pathophysiology
Edema of cartilage early
Progression- cartilage softens and loses elasticity
Loss of joint space
Bony degeneration and osteophyte/cyst formation
OA presentation
NO systemic sx
Slowly progressive joint pain
Deep and achy, tenderness
Worse with heavy use, better with rest
Gelling
Stiffness during rest with OA
Heberdens nodes
Palpable osteophytes and or cysts at DIP joint
OA
Bouchard’s nodes
Palpable osteophytes and or cysts at PIP joints
OA
Imaging findings of OA
Narrowed joint space
Osteophytes
Increased density of subchonral bone
Bony cysts
OA prevention
Weight loss
Correct any vitamin D deficiency
Joint fluid analysis general rule
RBC/WBC ratio in blood and normal fluid is 1000/1
More WBCs suggests infection or inflammation
Gout general
Associated with consumption of fish foods and alcohol
High purine foods are main trigger
Gout pathophysiology
Monosodium urate crystal formation
Often excess uric acid
Synovial fluid cooler than body temp, supersaturation and crystal aggregation
Inflammation via macrophage response
Podagra
Gout of the MTP joint (most common site)
Gout presentation
Sudden onset arthritis
Night onset common
Erythema, swelling, fever, warmth, TENDERNESS
Monoarticular usually
Tophi
Granulomataous inflammation around a deposit of MSU crystals
Chronic gouty arthritis
Persistent elevation in articular MSU
Chronic changes to bone, cartilage
“Rat bites” on X-RAYS
Gout diagnosis
Joint aspirate is key, especially for 1st diagnosis
Negatively birefringent needle like crystals
Chondrocalcinosis
Calcium salts in articular cartilage
Usually XR diagnosis
Pseudogout
Aka calcium pyrophosphate crystals
Gouty sx of large joints, knees and wrists common
Chondrocalcinosis of joint almost always seen
Diagnostic finding of pseudogout
Rhomboid, positively birefringent crystals under polarized light
Spondyloarthropathy general
Group of systemic inflammatory diseases
Musculoskeletal findings, extra articular manifestations, immunogenicity issues
Ankylosing spondylitis
Young adults
Chronic, inflammatory dz of axial skeleton
Low back pain that improves with activity
Ascending disease, lumbar curve flattens and cervical curvature exaggerated
Ankylosing spondylitis other findings
Peripheral arthritis
Uveitis
Entesthopathy (disordered tendon/ligament attachment)
Think of AS with…
Less than 30 years old, inflammatory pain that improves with activity
Reactive arthritis
Preceded by infection, but joints are not infected
Usually GI or STD pathogens
Reactive arthritis classic triad
Asymmetric arthritis, uveitis, and urethritis
“Can’t see, can’t pee can’t climb a tree”
Fever, weight loss, arthritis of large weight bearing joints
Septic arthritis definition, causes and pathogen
Bacterial joint infection
Hematogenous spread, direct inoculation, immunocompromise are the causes
Staph aureus most common!
Septic arthritis presentation
Acute pain, effusion, and warmth of joint
Fever, chills common
Knee most common site
Septic arthritis diagnostics
Synovial fluid analysis- increased WBCs
Often blood culture is positive
Prosthetic joint worries
Periprosthetic lucency!!
Gonoccoccal arthritis common patient populations
Menses, pregnancy, MSM
Gonococcal arthritis presentation
Migratory polyarthralgia, then EITHER
- tenosynovitis of wrist, hands or feet
- purulent monoarthritis
Osteomyelitis
Bony infection due to direct or hematogenous spread (always get blood cx)
Manifestations of osteomyelitis
Fever, chills, pain and elevated ESR/CRP
Wound that probes the bone is a clinical dx
Best imaging for osteomyelitis
MRI
Best way to obtain cultures for osteomyelitis?
Bone bx
Diskitis
Vertebral osteomyelitis
Insidious onset
Fever, back pain
Spinal/paraspinal tenderness
Elevated ESR, CRP, WBC
MRI best imaging
What do you see on an MRI for diskitis?
Disk collapse, irregular vertebral bodies
Epidural abscess
Can complicate diskitis
Increased neuro signs
-cord compression, so weakness, numbness, and radicular pain
Lemierre syndrome
Suppurrative thrombophlebitis of the jugular vein
Clot in setting of bacteremia
Typical bacteria for lemierre syndrome
Oral flora, usually fusobacterium
Osteomyelitis from Tb
Prolonged monoarticular arthritis
Send synovial fluid for AFB cx
Pott disease- spinal TB, months of back pain
Sickle cell disease with osteomyelitis
Salmonella is most common pathogen, microinfarcts in gut cause leakage, bony infarcts are setting for infection
Long bones
Osteosarcoma
Persistent bone pain, often epiphyseal
Rare, usually under 20 or over 65
Ewing sarcoma
1/3 of bony cancers effecting kids
Femur and pelvis most commonly
Fever and weight loss common here
Ewing sarcoma imaging
Moth eaten, finely destructive lesions
Onion skinning also seen
Soft tissue mass