Rheumatology Flashcards
RA
- define
- involves?
- intra or extra articular manifestations?
- Age of onset? MC in who
- etiology
- CM: MC?
- which joints MC involved and spares?
- list some characteristic hand deformities
- constitutional s/s
- PE?
- chronic systemic inflamm autoimmune dz involving synovium of joints
- inflammed synovium can cause damage to cartilage + bone
- EXTRA articular manifestations
onset= 20-40—W»M
Etiology unknown—-infection, genetic predispo
CM
- systemic/constitutional s/s: fever, fatigue, wt. loss, anorexia
- joint pain + stiffness: morning stiffness >1 hour and improves later in the day, decr ROM
- MC affects small joints: wrist, hands (MCP, PIP), MTP, ankle
- SPARES DIP
- characterisitc hand deformities
1. Boutonniere deformities of PIP
2. Swan-neck contractures of fingers
3. ulnar deviation of metacarpophalangeal joints
PE: SYMMETRIC***** inflamed joints
- warm, erythematous, boggy/soft,
- rheumatoid nodules over bony prominences
RA
- diagnosis (labs, radiographs)
- xray findings for general, severe, early and lte RA
- 5 diagnostic criteria
DIAGNOSIS:
- LABS
* elevated RF
* (+) Anticitrullinated peptide/protein antibodies
* elevated ESR
* elevated CRP - RADIOGRAPHS
*xray: symmetric joint narrowing, osteopenia, bone & joint erosions
SEVERE RA: joint subluxation
EARLY RA: xray normal
LATE RA: narrowing of joint space (bc of thinning of articlar cartilage)
FIVE DIAGNOSIS CRITERIA:
- inflammatory arthritis of 3 or more joints
- s/s > 6 weeks
- elevated ESR and CRP
- (+) serum RF and ACPA
- xray changes consistent with RA—erosions + periarticular decalcification
Is RF helpful to rend in RA pts?
NO
because it rarely changes with dz activity
best initial lab test for RA?
most specific lab test for RA?
Rheumatoid factor— initial
anti-citrullinated peptide antibodies–most specific
Tx for RA
goal is to minimize pain + swelling, prvent progression, help PT remain as functionl as possible
- exercise to maintain ROM and muscle strength
- DMARD (methotrexate or Leflunomide) + NSAID for immediate sympt control
* **DMARDs started early!! - Corticos second line for ss control— this does not slow the dz process tho
why are DMARDs rx for RA
- onset of action?
- ex
- ***Reduce morbidity and mortality by:
- *slow dz progression
- preserve joint function
- limit complications
***needs to be initiated early—at time of diagnosis
onset= 6+ weeks
EX:
- methotrexate, leflunomide, hydroxycholoquine, sulfasalazine= SYNTHETIC
- adalimumab, infliximab=TNF inhibitors aka BIOLOGIC dmards
- Abatacept, anakinra, rituximab–other biologics
best initial DMARD for RA?
- alternative to this drug?
- adjunct?
- alternative first line?
- which is used in less severe cases
Methotrexate=best initial
Leflunomide=alternative to methotrexate or adjunct
hydroxychloroquine and sulfasalazine= alternative for first line if cannot give methotrexate–BUT less effective + not usually used
Hydroxychloroquine used in less severe cases
which DMARD is safe in pregnancy
hydroxychloroquine
SE of methotrexate
- GI upset
- oral ulcers
- mild alopecia
- bone marrow suppresion
* **HAVE TO GIVE WITH FOLIC ACID - hepatocellualr injury
second line if all DMARDs dont work for RA?
anti-tumor necross factor (TNF) inhibiting drugs
- etanercept
- infliximab
Reactive Arthritis
- what is it
- also called?
- when does it show and what causes it
- MC occurs in?
- CM
- which joints MC affected
- inflammatory arthritis in resp to an infection or inflammation in another part of the body
- also called Reiter’s Syndrome
- MC seen 1-4 weeks after infections–>Chlamydia trachomatis or GI (salmonella, shigella, campy, yersinia)
MC occurs in HLA-B27 +
CM: *recent GI or genitourinary infection
- asymmetric arthritis–> new joints may be involved over days–painful with effusions and lack of mobility
* joint pain can persist or recur over long-term pd
* lower extrem joints MC affected–knees*** - systemic: fatigue, malaise, weight loss and fever
- TRIAD: arthritis + ocular s/s (conjunctivitis, uveitis) + genital (urethritis, cervicitis)
- Keratoderma blennorrhagicum—hyperkeratinized lesions on palms and soles
Reactive arthritis
- diagnosis–lab findings, etc
- tx–1st?
DIAGNOSIS
- arthrocentesis (synovial fluid analysis)— r/o crystals or septic arthritis
- incrs WBC (<50,000), negative cultures
- Incrs ESR
- incr IgG
TX:
- first line: NSAIDs
- if no response: sulfasalazine or Methotrexate second line +/- intraarticular glucocorticoid injections
* NO ABX*—- they do not fix the reactive arthritis—- only used to treat the underlying cause (GI or GU infection)
Polyarteritis Nodosa (PAN)
- what is it
- MC affects what part?
- spares what part?
- MC in who
- Assoc with?
- CM
- Diagnosis–labs, imaging, definitve?
- TX
- *systemic vasculitis MC medium sized vessels: renal, CNS, GI
- *SPARES pulmonary vessels
MC in men 40-60 YO
INCR assoc with chronic Hep B and C***** HIV, and drug rxns
CM
- Renal HTN: renal artery stenosis, renal ischemia–NOT assoc with glomerulonephritis
- GI: abd pain worse when eating (intestinal angina), N/V
- Constitutional: fever, arthalgia, myalgias
- CNS: neuropathy, stroke, Monoeuritis multiplex (type of peripheral neropathy), foot drop**, ulnar nerve neuropathy
- Dermatologic: ulcers, livdeo reticularis (skin appears mottled/purplish), rynaouds
DIAGNOSIS:
- LABS
* incr ESR
* proteinuria
* ANCA (-) - ANGIO:
* renal or mesenteric: microanurysms “beading”/strung together —> “rosary sign” - BIOPSY=definitive
* necrotizing medium vessel vasculities and NO GRANULOMAS
TX:
- Glucocorticoids +/- Cyclophosphamide if severe or refractory
- Hep B+: tx for BHV and possible tx with plasmaphoresis
what distinguishes PAN from other vasculities
PAN spares the pulmonary vessels
Polymyalgia Rheumatica
- define
- MC in who
- etiology
- duration of dz
- CM— worse when? most promient s/s?
- PE
- **idiopathic inflammation of: joints, bursae and tendons
- **closely assoc with giant cell arteritis
**MC in elderly (rare before 50) with avg onset=70YO
W»M
ETIOLOGY: unknown–auto immune
DURATION: self limiting–lasting 1-2 years
CM–usually begins ABRUPTLY
- pain + stiffness in proximal joints and muscles: shoulders, neck, hips and pelvic girdle >2 weeks
- BILAT pain
- stiffness in shoulder and hip regions after period of inactivity—-MC s/s AND profound morning stiffness and sometimes daylong stiffness
- Pain on movement
- can have diff combing hair or getting up from chair
- constitutional: fever, malaise, wt loss, depression
PE:
- normal muscle strength ****
- decr active and passive ROM
Polymyalgia Rheumatica
- diagnosis–labs
- tx
LABS
- incr ESR and CRP
- normal muscle enzymes (distinguishes PAN from polymyositis)
TX
- low-dose corticos=initial toc
- methotrexate is no resp to corticos OR if corticos are contraindicated
- ->Response usually occurs in 1-7 days
- ->After 4-6 weeks, begin to taper slowly
- ->Most patients can stop corticosteroids within 2 years; a few patients have symptoms up to 10 years
- ->Steroids are not curative, but are effective in suppressing inflammation until the disease resolves itself.
what distinguishes polymyalgia rheumatica from polymyositis
what is simialr about the two dz?
LABS–>muscle enzymes
- normal muscle enzymes= polymyalgia rheumatica
- incr muscle enzymes (CK and aldolase)= Polymyositis
PE:
- normal muscle strength in polymyalgia rheumatica
- decr muscle strength in polymyositis
- +weakness in polymyositis
- NO weakness in polymyalgia rheumatica
ONSET OF SYMPTOMS:
- polymyalgia rheumatica—abruptly
- polymyositis–more sub-acute–weeks-months
- 30-50YO for polymyositis
- > 50 YO polymyalgia rheumatica
TREATMENT
- polymyalgia rheumatica gets low dose corticos
- polymyositis gets high dose corticos
SIMILARITY:
- hip and shoulder pain
- diff rising from chair
- diff combing hair
Polymyositis
- what is it
- who is aff more
- MC affects what parts
- if skin is invovled–what is it called
- CM-MC affected ?
- PE
- diagnosis– best initial? most specific? definitive?
- *idopathic autoimune dz leading to MUSCLE INFLAMMATION
- *MC limbs, neck and pharynx–can aff heart, lungs, GI tract
W»M
30-50 YO
Skin involvement=Dermatomyositis
CM
- progressive symmetric proximal muscle weakness (hips and shoulders)–dev over weeks or months
- diff combing hair, going up stairs or geting up from chairs
- earliest and most severely affected muscle groups=neck flexors, shoulder girdle & pelvic girdle
- +/- myalgia, dysphagia
PE
- decr muscle strength—esp proximal muscles and often symmetrical
- muscle atrophy
DIAGNOSIS LABS: 1. sig incr muscle enzymes (CK and aldolase)--- best initial test 2. AUTOANTIBODIES: *anti-Jo-1 (myositis-specific antibody) *anti-signal recognition protein (most specific for this dz) *(+) ANA 3. Incr ESR and CRP 4. Incr RF
BIOPSY=definitive diagnosis–>endomysial inflammation
ELECTROMYOGRAPHY is abnm in 90% of patients
TX
- high dose corticosteroids first line
- Methotrexate, Azathioprine, IVIG, Mycophenolate—refrac to steroids or if contra
Dermatomyositis
- define
- PE
- diagnosis– most specific, best initial test, definitive
- idiopathic inflamm leading to dermatologic s/s + muscle inflammation
- polymyositis + derm s/s**
CM= same as polymyositis
PE=same as polymyositis AND:
- heliotrope rash–> edema & blue or purple discoloration of the upper eyelids
- Gottron’s papules–>raised violaceous scaly patches (dorsum of the PIP and DIP MC)
- shawl sign: erythema shoulders, upper chest and back
- V sign: erythema of neck and upper chest
DIAGNOSIS:
1. INITAL TEST= incr muslce enzymes (CK and aldolase)
2. autoantibodies: anti-Jo 1 and Anti-Mi-2**** MOST SPECIFIC
3. incr ESR, CRP, RF,
4 BIOPSY=definitive
5. EMG abnormal
TX
1. high dose glucocorticoids first line
2 Hydroxychloroquine useful for skin lesions
3. Immunosuppressive agents for patients who don’t respond to steroids or if corticosteroids are contraindicated (methotrexate, azathioprine, IVIG, mycophenolate)
Fibromyalgia
- MC in?
- what is it
- CM–aggrivatd by? better by?
- Diagnosis
MC in adult women—-80-90% of cases–>20-55YO
**waxing/waning in severity
*abnormal pain perception of unknown etiology
CM
- MSK pain–>constant and aching,
- —>pain aggrivated by weather changes, strs, sleep deprivation and cold temps, worse in am
- —>rest, warmth and exercise improve pain
- extreme fatigue
- stiffness
- sleep + cognitive disturbances (“fibro fog)
- HA
- neuro s/s: numbness
- anxiety
- depression
DIAGNOSIS
- primarily clinical
- CRITERIA: tenderness in at least 11/18 trigger points + chronic/widespread pain >3 MO
TX
- conservative tx: initial tx–>stay active + low intensity exercise
- 1st line medical tx–>amitriptyline (TCA)
* SNNRI: Duloxetine or Milnacipram OR cyclobenzaprine alternative - Local anesthetic at trigger points
- Pregabalin FDA approved esp for sleep s/s
Sjogren Syndrome
- define
- primary vs secondary
- assoc with?
- MC in who?
- cm
- tx
- complications of dz
- autoimmune dz affecting exocrine glands
- multi-organ dz
- Lymphocytes infiltrate and destroy exocrine glands (lacrimal and salivary glands)
- primary=occurs alone
- secondary: assoc with other autoimmune dz (Hashimoto, SLE, RA)
- MC W>M 40-60YO
- Assoc with HLDA-DR52 gene
CM
1. dry mucous membranes: xerostomia, dry eyes (keratoconjunctivitis sicca), vaginal dryness, bilateral parotid gland enlargement, dental caries (comp from xerostomia)
TX
1. Increase mucosal secretions–>artificial tears to prevent corneal ulcer, increase fluid intake, sugar free gum, artificial saliva and fluoride treatments
2. Cholinergic drugs: Pilocarpine or Cevimeline=incrs salivation + lacrimation
SE: diaphoresis, flushing, sweating, bradycardia, dirrhea, N/V
COMPS
- incr risk of non-hodgkin lymphoma, pneumonitis, interstitial nephritis
- incr risk of congenital HB if mom has high antibody titers while preg
how to diagnose Sjogren Syndrome
- best initial
- definitive?
SCREENING LABS:
Best initial test: (+)Anti-nuclear antibodies (ANA)—specifically AntiSS-A (Ro) & antiSS-B (La)
OTHER LABS: +RF, anemia, leukopenia
(+) Schirmer test: decry tear production—– wetting of <5cm filter paper placed in lower lid for 5 mins
DEFINITIVE= lip or parotid gland biopsy–>gland fibrosis & lymphocytic infiltration
Systemic sclerosis aka
- define
- Mc in who
- describe the two types
- diagnosis
- tx
scleroderma
- *systemic autoimmue CT dz where collagen deposition leads to fibrosis of the skin + internal organs (Lung, kidney, heart, GI)
- high quantity of collagen deposition that leads to problems*
MC women 35-40YO
TWO TYPES:
1. Limited (CREST) syndrome: 80% of cases
*tight, shiny, thick skin of the face, neck and skin distal to the elbows and knees–SPARES trunk
Calcinosis cutis–>Ca deposites in skin
Raynaud’s
Esophageal motility disorder
Sclerodactyly (claw hand–localized thickness +tightness of skin on fingers/toes
Telangiectasis
- Diffuse: 20% cases
* tight, shiny, thickened skin involving trunk & proximal extremities
* assoc with greater internal organ involvement–>resitrictive lung dz, myocardial fibrosis
DIAGNOSIS
- anti-centromere antibodies->specific for CREST
- anti-SCL-70 antibodies–assoc with diffuse dz and multi organ involvement—-poorer prognosis
- (+) ANA– not specific but positive in 90% of patients
TX
-
organ specific
1. GERD: PPIs
2. Hypertensive renal dz: ACEI
3. Raynauds: vasodilators–CCBs
4. severe: DMARDs: methotrexate, Mycophenolate, Cyclophosphamide for refrac or severe
5. Pulmonary fibrosis: Cyclophosphamide
6. Pulm HTN: Bosentan, Sildenafil
Systemic Lupus Erythematous (SLE)
- define
- RF + onset
- types
- idiopathic chronic systemic, multi-organ, auto immune dz of CT
- genetic, environmental and hormonal factors invovled
- primarily type III hypersensitivity
RF
- young females–> onset 20-40
- AA
- hispanic
- native american
- genetic
- environmental
- sun exposure
- infections
- estrogen—OCPs
FOUR TYPES
- spontaneous SLE
- discoid lupus (skin lesions without systemic dz)
- drug-induced lupus
- ANA negative lupus
SLE
-CM
TRIAD: joint pain + fever + malar rash
Constitutional s/s: fever, chills, fatigue, night sweats
Discoid lupus: annular, erythematous pathces on the face and scalp that heal with scarring
Cutaneous s/s: malar rash, photosensitivty, oral or nasopharyngeal ulcers, alopecia, raynauds
MSK: joint pain–>may be first symp of dz, arthritis (inflammatory and symmetric), arthalgias, myalgias
OTHER: HA, serositis, glomerulonephritis, retinitis, oral ulcers, alopecia
Diagnostic findings for SLE
LABS
(+) ANA–>screening TOC
*anti-double stranded DNA and anti-smith antibodies—>either one is diagnostic of SLE (specific and pathognomonic)
*antiphospholipid antibodies=incr risk of aterial or venous thrombosis
*Pancytopenia: anemia of chronic dz, leukopenia, lymphopenia and thrombocytopenia
*Decreased complement C3, C4
Labs for a SLE flare up
increase in dsDNA antibodies and complement C3 and C4 decrease
what is used for monitoring SLE
dsDNA antibodies
Tx for SLE
- mild and define mild
- mod and define
- severe and define
*tx depends on level of organ involvement
FOR ALL PT: sunscreen + avoidance of prolonged sun exposure
MILD (skin, joint, mucosal s/s)
- hydroxychloroquine w/ or w/o NSAIDs and/or short term low-dose glucocorticoids
- sometimes NSAIDs can be used alone for very mild dz
MODERATE: significant but non-organ threatening
- hydroxychloroquine or chloroquine+ short term glucocorticoid
- CAN ADD: Belimumab—monoclonal antibody that inhibs B-lymphs—reserved for active cutaneous or MSK dz unresponsive to glucos or other immunosuppressants
SEVERE: life or organ threatening
*high dose glucos or intermittent IV “pulses” of Methylprednisolone with other immunosuppressive agents (cyclophosphamide, Mycophenolate, Rituxmab)
Antiphospholipid Syndrome
- define
- triggers
- diagnosis
- tx–asympto and sympto
Idiopathic disorder characterized by venous or arterial thromboses due to antibodies against negatively charged phospholipids
*can occur as primary OR with other Dz— such as SLE
TRIGGERS: smoking, prolonged immob, estrogen, CA, hyperlipidemia, HTN
CM
*increased risk of arterial and venous thromboses (atherosclerosis, recurrent DVT or PE, recurrent miscarriages)
DIAGNOSIS
- anticardiolipin antibodies
- Lupus-anticoagulant=incr PTT
TX
- asympto: no tx
- recurrent thrombosis may require lifelong Warfarin or other type of anticoag
- low molecular weight Heparin used in pregnancy
Jeuvenille (idiopathic) Rheumatoid Arthritis
- define
- types and CM
*autoimmune mono or polyarthritis in <16 y/ for >6 weeks
THREE TYPES 1. Systemic (Still's Dz): 20% of all cases *daily high fever *daily arthritis pain *salmon-colored pink migratory rash *lymphadenopathy NO UVEITIS
- Pauci (oligo) articular= 50% of cases
* less than 5 joints involved, typically large joints (knees, ankles)
* YES uveitis - Polyarticular
* 5+ small joints
* usually symmetric
* Uveitis
* similar to adult RA— + morning stiffness, can be RF +
* worse prognosis
Jeuvenille (idiopathic) Rheumatoid Arthritis
-dx
-tx–1st, 2nd, severe
tx for (+) ANA?
DX=clinical *incr ESR and CRP \+ANA if oligoarticular 15%= RF(+) Still's DZ often assoc with negative RF and ANA
TX
- NSAIDs=1st line
- Steroids=2nd or NSAIDs not eff
* *PT
Severe or as second line: Anakinra (interleukin-1 rec inhib), methotrexate, lefluonmide
IF (+)ANA= routine eye exams every 3 MO bc of uveitis
