Rheumatology Step Up Flashcards
associated findings in ANA-negative lupus
- arthritis, Raynaud’s phenomenon, subacute cutaneous lupus
- risk of neonatal lupus in offspring
serology in ANA-negative lupus
Ro (antiSS-A) positive
ANA negative
CF in neonatal lupus
skin lesions
cardiac lesions - AV block, transposition
valvular and septal defects
criteria for dx. SLE (11)
malar rash discoid lesions photosensitivity oral/nasal ulcers arthritis pericarditis/pleuritis hematologic disease - cytopenias renal disease - proteinuria CNS - seizures/psychosis ANAs other ab - dsDNA, Smith, FP on VDRL
presence of what ab’s is diagnostic of SLE?
anti-dsDNA
anti-Smith
how do you diagnose a flare of SLE?
rise in anti-dsDNA
fall in C3/C4 levels (CH50 is more sensitive)
which ab’s are very SENSITIVE for SLE but not specific?
ANA
when Ro (SS-A) and Lo (SS-B) ab’s are found in SLE, they are associated with…
neonatal lupus subacute cutaneous lupus sjogren's syndrome complement deficiency ANA-negative lupus
major use of ESR
diagnose/rule out inflammatory processes
monitor the course of inflammatory conditions
major use of CRP
mainly infection - much more sensitive and specific for ESR
- if > 15, bacterial infection is present
best tx. for SLE pts with acute flare
steroids
best long term tx. for constitutional, cutaneous and articular manifestations of SLE
hydroxychloroquine
NSAIDs for less severe symptoms
what should you recommend for pts on hydroxychloroquine tx for SLE
yearly eye examination bc of retinal toxicity
Tx. of active lupus nephritis
cyclophosphamide
azathioprine
MCC of death in SLE
renal failure
infections
drugs that may cause lupus-like syndrome
hydralazine
procainamide
isoniazid
quinidine
which two organ systems does drug-induced lupus NOT affect?
CNS and renal
- if these sx are present, it is NOT drug-induced
pathophysiology of scleroderma
cytokines stimulate fibroblasts to produce collagen; high quantity of collagen is responsible for symptoms
what symptom(s) is/are present in almost all pts with scleroderma?
Raynaud’s
cutaneous fibrosis - thickening of skin of face and extremities
how do you dx. Raynauds?
nail-fold capillaroscopy - look for evidence of vessel damage
GI findings in scleroderma
dysphagia/reflux from esophageal immobility
delayed gastric emptying
abdominal distention
pseudo-obstruction
MCC of death in scleroderma
pulmonary involvement - pulmonary fibrosis or HTN (diffuse form only)
renal involvement in scleroderma
in diffuse form only
- renal crisis w/ rapid malignant HTN
ab specific for limited form of scleroderma
anti-centromere ab
ab specific for diffuse form of scleroderma
anti-topoisomerase I (anti-Scl70)
findings in Barium swallow in scleroderma
absence of peristaltic waves in lower 1/3 of esophagus
decreased LES tone
what predicts prognosis in scleroderma?
degree of skin involvement - difffuse form has worse prognosis than limited form
CREST syndrome
Calcinosis of digits Raynaud's phenomenon Esophageal dysmotility Sclerodactyly Telengiectasias
Tx. of scleroderma
symptomatic
- MSK = NSAIDs
- Raynauds = CCB
- skin findings = D-penicillamine
- renal = ACEi
- GI = PPIs/H2 blockers
20% of pts with scleroderma have…
Sjogrens syndrome
typical findings in anti-phospholipid syndrome
recurrent venous/arterial thrombosis
recurrent pregnancy loss
thrombocytopenia
livedo reticularis
lab findings in antiphospholipid syndrome
presence of lupus anticoagulant and/or anticardiolipin antibody
prolonged PTT or PT that is not corrected by adding plasma
Tx of antiphospholipid syndrome
anticoagulation - INR 2.5-3.5
primary Sjogren’s syndrome
dry eyes and dry mouth, along w/ lymphocytic infiltration of minor salivary glands (histology)
secondary Sjogren’s syndrome
dry eyes and dry mouth along with a connective tissue disease
MCC of death in Sjogren’s
malignancy
- increased risk of non-Hodgkin’s lymphoma
what test has high sensitivity and specificity for Sjogren’s syndrome?
Schirmer test - filter paper inserted into eye to measure lacrimal gland output
most accurate test for dx Sjogren’s
salivary gland biopsy (lip or parotid)
- not needed for diagnosis
Tx. for Sjogrens
- pilocarpine or Cevimeline
- artificial tears
- NSAIDs/ steroids - arthritis
ab found in mixed CT disease
anti-U1-RNP ab
predom. cells implicated in RA
T helper cells - therefore, RA is not seen in pts with HIV/AIDs
which joints are characteristically NOT involved in RA?
DIP joints
what symptom is present in all patients?
morning stiffness
cutaneous finding that is pathognomic for RA
subcutaneous rheumatoid nodules (sacrum, elbows, occiput, achilles tendon)
pulmonary findings in RA
pleural effusions (low glucose)
pulmonary fibrosis
rheumatoid nodules in lung
cardiac findings in RA
rheumatoid nodules - conduction block
pericarditis
eye findings in RA
scleritis
scleromalacia - softening of sclera
dry eyes and mucous mbs
nervous system findings in RA
mononeuritic multiplex - damage to one or more peripheral nerves
Felty syndrome
triad of RA, splenomegaly and neutropenia
associated clinical findings in Felty’s syndrome
anemia, thrombocytopenia
LAD
recurrent infections
high titres of RF
type of anemia seen in RA
normocytic, normochromic anemia
what must you screen for in every pt with RA prior to undergoing surgery or intubation?
cervical radiograph of spine to assess for evidence of C1-C2 subluxation/instability
poor prognostic indicators in RA
high RF titres
subcutaneous nodules
autoantibodies to RF
erosive arthritis
lab findings in RF
high titres of RF - more severe disease
anticitrullinated peptide (ACPA)
elevated ESR/CRP
normocytic, normochromic anemia
what are pts with positive RF and ACPA at risk for? how should you intervene?
erosive joint damage
- early tx. with DMARDs indicated
criteria for dx. of RA
- inflammatory arthritis > 3 joints
- sx. lasting 6 weeks
- high ESR/CRP
- serum RF or ACPA positive
- radiographic changes
general tx. approach to RA
when pt presents with sx, start them on NSAIDs (steroids if NSAIDS not adequate relief); at the same time, start pt on DMARD therapy, taper NSAIDs/steroids after approx 6 weeks of tx and then use those PRN
first-line DMARD to start in RA
Methotrexate/Folate
side effects of Methotrexate
GI upset, oral ulcers mild alopecia bone marrow suppression hepatocellular injury pulmonary fibrosis
what should the patient have monitored while on Methotrexate therapy?
CBC every 2-3 months
LFTs
renal function