MSK and Connective Tissue Disorders II Flashcards
who as the most susceptible to lupus?
Americans of African descent
lupus is a ______ tissue disease
connective
Congenital deficiencies of what are associated w/ increased incidence of SLE?
C1, C2, C4
when is onset of lupus most common?
2nd and 3rd decades
people with lupus have a higher risk of what?
premature death
lupus is a ____ system problem
profound immune
In lupus there is an uncontrolled production of what?
autoantibodies and immune complexes
2 ways tissue injuries occur in lupus?
formation of immune complexes (ex- glomerulonephritis)
damage/ destruction of specific cells (ex- generation of C3 and C5) causing inflammation,
what hormone plays a factor in SLE?
estrogen
when are exacerbation of SLE common?
pregnancy and OCP meds
male with what condition have an increased incidence of SLE.
Klinefelter’s
what drugs can cause drug induced lupus?
procainamide hydralazine isoniazid methyldopa quinidine chlorpromazine phenothiazines OCPs anticonvulsants
if a patient has a drug induced lupus will that ANA be positive?
yes, it can be
if you suspect drug induced lupus what must you do?
Stop drug, monitor symptoms and then repeat ANA
what are the three main symptoms of lupus
arthritis (joint inflammation)
arthralgia
fever
what syndrome if common in SLE?
Raynaud’s syndrome
a hx of what is common in women with SLE.
spontaneous abortions
what are skin signs common with SLE
vasculitis
purpura
periungal erythema
what often leads to deformities and contractures in SLE?
chronic arthritis
tensosynovitis
the involvement of what organ with SLE has the worst prognosis
renal
what are renal symptoms of SLE
nephrotic symptoms
renal failure
what are some CNS symptoms seen with SLE
epilepsy depression, dementia, psychosis cranial nerve lesions hemiplegia, transverse myelitis chorea, ataxia
what are ocular manifestations of SLE?
keratoconjunctivitis
episcleritis
retinal vasculitis
retinal exudates
what are abdominal manifestations of SLE?
peritonitis
splenitis
pancreatitis
GI ulcers
what do you need for a lupus diagnosis?
Positive ANA and four crieteria
what are SLE criteria for diagnosis
malar rash photosensitivity arthritis serositis (hearts/ lungs) renal dz (proteinuria, cellular casts) hematologic disorders (hemolytic anemia, leucopenia, leukocytosis, thrombocytopenia) immune disorders (include false + syphilis) neuro dz (seizures, psychosis) discoid rash oral ulcers
people with SLE can present with spontaneous what?
DVTs
what labs should you get for SLE?
ANA CBC BUn creatinine UA ESR Serum complement (C3 and/or C4)
what can be used to monitor the progression of SLE?
antibodies to Smith antigen
dsDNA
depressed serum complement
Tx for lupus
NSAIDs
regular exercise and sun protection
antimalarial
corticosteroids
what antimalarials are used for SLE?
Hydroxychloroquine or quinacrine
what is the best combo for corticosteroids for SLE to prevent renal failure and tx symptoms?
Prednisone and cyclophosphamide
drug for SLE that works well for arthritis, rashes, serositis, and constitutional symptoms
methotrexate
long term use of ________ carries a risk of infertility in females >25 and increase malignancy risk
cyclophosphamide
what s late mortality w/ SLE usually due to?
secondary CAD or CVA
what are the most common infections in SLE?
pneumocystis carinii
candida albicans
most SLE infection respond to what?
steroid therapy
Overlapping clinical features suggestive of SLE, progressive sclerosis & polymyositis
mixed connective tissue disorders
will mixed connective tissue diseases have a high ANA titer (>1:80)
Yes
what diseases are included in mixed connective tissue diseases
Sjogrens
scleroderma
vasculitis
Autoimmune disorder causing destruction of salivary and lacrimal glands (exocrine glands)
Sjogren’s syndrome
can sjogren’s be a complication of a prexxsisting disease?
Yes, in RA, SLE, polymyositis, scleroderma
who is sjogren’s most common in?
middle-aged females
what are clinical features of sjogren’s syndrome?
dry mouth (xerostomia) dry eyes (xerophthalmia or keratoconjunctivitis sicca) enlargement of parotid glands
what will labs look like w/ connective tissue disorders
Don’t quite make sense
some positive, some negative
What test evaluates tear secretions for Sjogren’s
Schrimer’s test
how do you do schirmer’s test
– wetting of less than 5 mm of filter paper placed in lower eyelid for 5 min is considered +
(for sjogren’s)
tx for sjogren’s syndrome
artificial tears and saliva
protect the eyes
increase PO intake
ocular and vaginal lubricants
are steroid indicated in Sjogren’s?
rarely
What drugs are helpful for Sjogren’s
NSAIDS (inflammation)
corticosteroids for huge flare up
ACEI (if HTN)
Tx for Raynaud’s
Nifedipine (CCB)
prostacyclin
protect limbs from cold
when is sjogren’s prognosis worse
late onset
widespread skin, renal, pulmonary, cardiac inolvement
what do people of sjogren’s usually die of
aneurysms or renal issues
what are the two types of scleroderma?
diffuse (systemic)
limited (skin of face, neck, elbows, knees)
where does scleroderma most often begin?
fingers and hands
What do most people with scleroderma typically have?
Raynaud’s phenomenon
what is a common presentation of scleroderma
skin changes
polyarthralgias
esophageal dysunfction
what does CREST syndrome stand for?
Calcinosis Raynaud's esophageal dysfunction sclerodactyly telangiectasia's
is ANA typically positive in scleroderma?
Yes
what must you monitor w/ scleroderma
development of HTN
look for kidney problems
Multi-system, autoimmune disease of unknown cause
Vasculopathy & excess collagen deposits are characteristic
systemic sclerosis
what does systemic sclerosis lead to
fibrosis
degenerative changes of skin and internal organs
what are early manifestations of systemic sclerosis
raynaud's phenomenon skin calcinosis (scleroderma)
who is systemic sclerosis common in?
females ages 30-60
what are early manifestations of systemic sclerosis
wrist pain wheezing skin- abnormally light/dark pigment joint pain hair loss, alopecia eye burning, itching, discharge arthralgia
what is a unique component of systemic sclerosis
non-pitting edema
sausage swelling of fingers
what are some GI problems w/ systemic sclerosis
reflux/ hiatal hernia loss of normal peristalsis dilation of large and small bowel pain, constipation, diarrhea primary biliary cirrhosis is associated
what does patients w/ systemic sclerosis die of?
constrictive pericarditis
if someone has pulmonary fibrosis/ dz out of no where what should you look at?
connective tissue disorders
Tx for symptoms of systemic sclerosis
PPI for GERD
ACEI for renal
CCB for Raynaud’s
immunosupressive drugs for pulmonary HTN
what are complications from glucocorticoids?
cushing's syndrome osteoporosis DM Infection IMmunosuppression
What are ADRs from cyclophosphamide?
Pancytopenia mucositis alopecia hemorrhagic cystitis TC carcinoma sterility
what are ADRs of azathioprine?
leukopenia
anemia
risk of infection
possible hematologic malignancy
what are ADRS of methotrexate
Teratogenic
liver fibrosis
infection
ADRs of vasodilators
HPOTN
constipation
ADRs of antimalarials
retinal toxicity
what muscles does inflammatory myopathy usually affect?
proximal muscles
What test is abnormal in inflammatory myopathy?
EMG
what are the three inflammatory myopathies
dermatomyositis
polymyositis
inclusion body myositis
what makes dermatomyositis unique?
rash
good response to corticosteroids
increased serum creatinine cleranace
perifascular atrophy (on muscle biopsy)
Inflammation of large, medium, or small blood vessels leading to end-organ or necrosis/tissue damage
Systemic vasculitis
Acute systemic disorder of childhood
Resembles viral exanthem
Boys affected 3x more than girls
Kawasaki’s Disease
what complications are associated w/ Kawasaki’s Disease?
coronary aneurysms coronary artery dilatation MI pericarditis effusion cardiac failure
Tx for Kawasaki’s Disease
ASA 5mg/kg/day
high dos IVIG
should people with kawasaki’s be given steroids?
No, can increase coronary dilatation
tx for vasculitis
corticosteroids or cytotoxic drugs
plasmapheresis
complications of vasculitis
coronary aneursysms coronary artery dilatation MI pericarditis w/ effusion cardiac failure
what three things is temporal arteritis associated with?
steroids
blindness
biopsy
Pain and stiffness in neck, shoulder, upper arms, pelvic girdles and thighs + constitutional symptoms (fever, wt loss, fatigue, depression). Usually >50
polymyalgia rheumatica
tx for polymyalgia rheumatica
steroids
what is also often seen w/ polymyalgia rheumatica
temporal arteritis
Presents with scalp tenderness, jaw claudication, HA, temporal artery tenderness
May lead to vision loss
temporal arteritis
when is stiffness more severe w/ polymyalgia rheumatica
after rest and in AM
what lab test do you do for polymyalgia rheumatica?
ESR markedly elevated (>50)
Tx for polymyalgia rheumatica
low-dose corticosteroid therapy (PO)
may be required for 2 years, must slowly taper off
higher doses needed when temporal arteritis present
fibromyalgia is a diagnosis of _______
exclusion
clinical features of fibromyalgia
Non-articular musculoskeletal aches, pains, fatigue, sleep disturbance, and multiple tender points on exam (is a chart available for practice)
there is a strong _________ component in fibromyalgia
psych
what do you need to exclude to make a dx of fibromyalgia?
thyroid disease
hep C
vit D deficiency
abnormal T-cell subsets (not diagnostic)
do you give narcotics for fibromyalgia?
No
what drugs may be helpful for fibromyalgia?
SSRIs
SNRIs
TCAs
Lyrica (only FDA approved)
what labs are helpful to r/o other pathology before dx fibromyalgia
x-rays around joints CBC ESR RA factor ANA CPK helpful (elevated in muscle destruction)
what will be abnormal in a sleep study in a person w/ fibromyalgia
disturbance of stage IV non-REM on EEG
