Rheumatological 2 Flashcards
Infectious arthritis
- pathophys
- bacterial or fungal infection of joint
- Hematogenous seeding (MC), direct inoculation, contiguous spread from osteomyelitis, cellulitis, or septic bursitis
- bacteria trigger inflammatory response of neutrophilic infiltration into joint space
- can rapidly progress to joint damage
Infectious arthritis
- dx
- joint aspiration: gram stain, fungal stain, culture, acid fast stain (TB), crystal analysis
- If suspect GC, gram stain and culture anus, urethra, cervix, and pharynx also
Risk factors for infectious arthritis (10)
- age
- alcoholism
- cutaneous lesions
- dm
- ESRD
- hx steroid injections
- IVDU
- immunosuppresant
- sickle cell
- malignancy
Infectious arthritis
- common bugs
- staph aureus (MC)
- strep pneumo
- gonococcal (MC female)
- non-gonococcal gram negs (MC in immunosuppressed)
Why is gonococcal infection in joint more common in females
- males with GC have dysuria = treatment
- females dont’ have obvious sx, infection travels from vagina to cervix to uterus to fallopian tubes without obv sx, longer time to get in blood
What is presentation of GC infectious arthritis
- acute arthritis
- fever
- skin lesions (vesiculopustular, hemorrhagic macules)
5 MC fungus in infectious arthritis
- coccidiodes
- sporothrix
- cyptococcus
- blastomyces
- candida
Viral infections associated with infectious arthritis
- HBV
- HCV
- Parvovirus B19 (Fifth disease)
What can both HBV and HCV cause in addition to infectious arthritis
polyarthritis in the prodromal phase of the hep virus
Describe presentation of Fifth disease infectious arthritis
- aka erythema infectiosum
- acute arthritis
- usually young people
- resembles acute RA without the antibodies
- fever
- slapped cheek erythematous rash
- tx: supportive care
Chikungunya
- describe pathogen
- type of infectious arthritis
- virus endemic to asia/africa
- transmitted via mosquitos
- in US from travelers
Chikungunya
- sx
- fever
- myalgia
- thrombocytopenia
- leukopenia
Chikungunya
- dx
- travel hx
- IgM anti-chikungunya antibodies
Idiopathic inflammatory myopathies (IIMs)
- describe
- autoimmunity and inflammatory involvement of muscle fibers
- result in muscle weakness
What are the three Idiopathic inflammatory myopathies (IIMs)
- Polymyositis (PM)
- Dermatomyositis (DM)
- Inclusion body myositis (IBM)
Idiopathic inflammatory myopathies (IIMs)
- pathophysiology of each subtype
- PM: CD8-positive T cell mediated immune disease = direct myocyte injury
- DM: immune complex dz with vascular inflammation, cutaneous findings
- IBM: mho-degenerative dz with vacuoles in muscle cells and related t-cell response
Idiopathic inflammatory myopathies (IIMs)
- common sx
- antibodies
- fatigue, wt. loss, fever
- Anti-jo-1 antibodies (acute onset)
What is antisynthetase syndrome?
- Anti-jo-1 antibodies with m weakness, pain and 2+ of:
- inflammatory myositis
- interstitial lung dz
- Raynaud
- non-erosive inflammatory arthritis
- mechanics hands
Dermatomyositis cutaneous manifestations
- Gottron sign/papules: symmetric erythematous violaceous macules, patches on extensor surface of hand
- Heliotrope rash on sun exposed surfaces (upper eyelid)
- shawl sign on back (sun)
- v sign on chest (sun)
- mechanics hands
How to diagnose idiopathic inflammatory myopathies
- Hx and exam
- muscle enzymes: Ck, aldolase, AST
- electromyography
- auto-antibodies (lots)
- MRI: active muscle inflammation
- Muscle bx
4 meds that can cause idiopathic inflammatory myopathies
- cochicine
- hydroxychloriquine
- glucocorticoids
- statins
idiopathic inflammatory myopathies
- management
- high dose glucocoricoids initially, then taper to low dose
- immunosuppressants
- Rheumatology referral
Systemic Vasculitis
- describe
- inflammation of arteries, veins, capillaries
- small, large, intermediate size vessels
- r/o the many secondary causes
Two types of large vessel vasculitis
- giant cell arteritis
2. takayasu arteritis
Giant cell arteritis
- incidence
- M vs. F
- age
- MC primary vasculitis
- F > M
- 70 av age of onset
Giant cell arteritis
- commonly affected
- MC temporal
- carotids
- ciliary ophthalmic
Giant cell arteritis
- pathyophysiology
- dendritic cells in vessel adventitia are activated & recruit T cells and monocytes to vessel wall
- Macrophages coalesce and form giant cells
- cells secrete metalloproteinases = disrupt vessel wall
- Intimal proliferation = vessel closure, ischemia, dead tissue
Giant cell arteritis
- sx
- scalp pain
- HA
- tenderness over temporal artery
- jaw claudication
- temp blindness
- diplopia
Giant cell arterities
- dx tests
- VERY high sed rate (>70)
- temporal artery bx
Giant cell arteritis
- tx
- glucocorticoids
- high dose at first, then taper down
What co-morbidity is often found with giant cell arteritis
- polymyalgia rheumatica
Takayasu arteritis
- what vessels affected
- who does it MC affect
- aorta and its main branches
- young women
Takayasu arteritis
- presentation
- s/s inflammatoin
- PE findings of vascular disease: decreased pulse, asymmetrical pulse, BP, bruits
Takayasu arteritis
- dx
- angiography: aneurysms and narrowing of affected arteries
Takayasu arteritis
- tx
- high dose steroids
3 medium sized vasculitis
- polyarteritis nodosa
- primary angiitis of CNS
- Kawasaki dz
Polyarteritis nodosa
- incident
- rare
- m = f
- 30% assoc. with past HBV infection
polyarteritis nodosa
- presentation
- aneurysm formation
- ruptured aneurysms
- organs: kidney, peripheral nerve, tests, ovaries, GI, coronaries
How most likely to pick up polyarteritis nodosa?
ROS - will get unusual sx such as mononeuritis multiplex (numbness comes and goes)
Polyarteritis nodosa
- tx
- high dose glucocorticoids
- DMARDS sometimes
- GET HELP
Primary angiitis of CNS
- incidence
- describe
- VERY rare
- vasculitis limited to CNS
- necrotizing granulamatous vasculitis
Primary angiitis of CNS
- presentation
- CNS issues
- stroke
- visual field defects
- seizures
Primary angiitis of CNS
- Dx
brain biopsy (ahhh)
Primary angiitis of CNS
- management
- high dose steroids
- cyclophosphamide
Kawasaki disease
- incidence
- asian boys
Kawasaki disease
- presentation
- spiking fevers
- conjunctivitis
- mucusitis of lips and oral cavity
- palmar/plantar erythema, induration, desquamation (like hand, foot, mouth dz)
Kawasaki disease
- tx
- IV immune globin
- ASA
Small vessel vasculitis
- what affected
- two major categories
- post-capillary venues, arterioles, capillaries
- ANCA associated
- immune complex-mediated (C3 & C4)
What are the three ANCA associated vasculitis diseases
- granulomatosis with polyangiitis (Wegener Granulomatosis)
- Microscopic polyangitis
- Eosiniphilic Granulomatosis (Churg-Strauss syndrome)
what does ANCA stand for
anti-neutrophil cytoplasmic antibody
ANCA associated vasculitis diseases
- pathophys
- when ANCA attacks neutrophil, neutrophil degranulates
- enzymes attack endothelium of small blood vessels
- neutrophils disintegrate: can find remnants in vessel walls on bx
- dz d/t closure of small vessels
What do you order when suspect small cell vasculitis?
- c-ANCA antibodies
- p-ANCA antibodies
- helps determine which disease
Granulomatosis with polyangiitis
- presentation
- upper resp. tract, nose, upper airway
- lungs: SOB, hemoptysis
- Kidney: glomerulonephritis
Granulomatosis with polyangiitis
- Dx
- ANCA antibodies
- lung/kidney tissue bx
Granulomatosis with polyangiitis
- tx
- steroids
- cyclophosphamide
Microscopic polyangiitis
- presentation
- very similar to granulomatosis with polyangiitis
How differentiate microscopic polyangiitis from granulomatosis with polyangitis?
P-ANCA for microscopic
Eosinophilic Granulomatosis
- presentation
- allergic rhinitis
- nasal polyps
- asthma
- lung dz
- peripheral nerve dz (mono or multiple nerve numbness, permanent or come and go)
Eosinophilic Granulomatosis
- lab findings
- hypereosinophilia
- P-ANCA (but 40% negative…)
Eosinophilic Granulomatosis
- tx
- glucocorticoids
Immune complex vasculitis
- basic pathophys
- immune complex deposition in vessel walls
- fix complement and attract neutrophils
Immune complex vasculitis
- what organs affected?
- Any can be affected
- skin and joint MC
Three immune complex vasculitis diseases
- Cryoglobulin vasculitis
- Leukocytoclastic vasculitis
- Henock-Schonlein Purpura
Cryoglobulin vasculitis
- antibodies
- antibodies precipitate in vitro at temp < body temp
- can precipitate in cooler areas of body
Cyroglobulin vasculitis
- lab
- cryoglobulins
- C3 and C4 low
Leukocytoclastic vasculitis
- presentation
- immune complex manifestation in skin
- palpable purpura
Leukocytoclastic vasculitis
- dx
- neutrophils in tissue bx
Henock-Schonlein purpura
- describe
- MC childhood vasculitis
- rare in adults
- tends to appear after URI
- antigen unknown, antibody in IgA class
Henock-Schonlein purpura
- presentation
- abd pain
- palpable purpura
- vasculitis in abd and skin vessels
Henock-Schonlein purpura
- tx
- usually self limiting
- possibly steroids
Scleroderma
- aka
- describe
- aka systemic sclerosis
- autoimmune dz
- fibrosis = thickening and hardening of skin
- internal organ involvement common
- sig morbidity/mortality
Scleroderma
- Pathophysiology
- Excess deposition of structurally normal collagen
- small artery endothelial damage/dysfunction
- trigger unknown
Scleroderma
- dx
- mostly clinical dx
- symmetric skin induration of hands, arms, face, torso
- sclerodactyly (skin fibrosis of hands an fibers)
- digital infarction
- finger pitting
- DOE
- dysphagia
Scleroderma
- lab findings
- ANA in anti-centromere pattern
**KNOW THIS ONE
Scleroderma
- treatment
- immunosuppressant drugs IF active lung dz
Behcet Syndrome
- describe
- vasculitis that affects small to large arterial vessels
- can affect veins
Behcet syndrome
- presentation
- lab
- recurrent, painful oral ulcerations, vagina also
- HLA-B51
Behcet Syndrome
- dx
Recurrent painful oral ulcers and 2+ of:
- recurrent painful genital ulcerations
- eye involvement
- erythema nodosum
- pathergy test (needle into skin = immediate inflammatory response)
Sarcoidosis
- describe
- multisystem dz characterized by noncaseating granuloma ** in tissues
Sarcoidosis
- MC affects what organ
- what other sx?
Lungs
- bilateral hilar adenopathy
- erythema nodosum
Sarcoidosis
- dx
- bilateral hilar lymphadenitis
- erythema nodosum
if have both = dx - if don’t have both, bx showing noncaseating granulomas required for dx
Sarcoidosis
- tx
- symptomatic
- NSAIDs, colchicine, low dose steroids
- immunosuppressives
- TNF alpha inhibitors
