ICM - Rheum 5 Flashcards
this inflammatory myopathy is due to problems with the humoral immune system
dermatomyositis
these inflammatory myopathies are due to cell-mediated response
polymyositis and inclusion-body
rash seen over knuckles/elbows in dermatomyositis
Gottron’s papules
this inflammatory myopathy is at increased risk for malignancies
dermatomyositis
inflammatory infiltrate seen in dermatomyositis biopsy
perivascular and perimysial
inflammatory infiltrate seen in polymyositis and inclusion-body myositis biopsy
endomysial
treatment for inflammatory myopathies
corticosteroids, immunosuppresion (methotrexate, cyclophosphamide)
muscle most affected in inflammatory myopathies (symmetrical proximal muscle weakness)
neck flexors, shoulder girdle, pelvic girdle
enzymes that are elevated in inflammatory myopathies
CPK, LDH, aldolase, AST, ALT
inflammation seen in inflammatory myopathies
lymphocytes
pathology: perivascular and perifascicular lymphocytic infiltrate, immune complex deposition in vessels, perifascicular atrophy and fibrosis
dermatomyositis
pathology: cellular infiltrate within fascicle, inflammatory cells invade individual muscle fibers
polymyositis
pathology: perivascular exudates rare, characteristic intracellular lined vacuoles; intracytoplasmic or intranuclear tubular or filamentous inclusions
inclusion body myositis
MCTD is overlap of these 3 conditions –> high ANA and anti-RNP
polymyositis, scleroderma, SLE
myositis specific Ab –> association with interstitial lung disease, Raynaud’s, arthritis, mechanics hands
anti-histidyl tRNA synthetase (anti-Jo1)
myositis specific Ab –> rapid onset of classical dermatomyositis
anti Mi2
pathognomic for dermatomyositis or scleroderma
nail fold capillary changes
5 diagnostic criteria for inflammatory myopathies
proximal muscle weakness, elevation serum muscle enzymes, EMG, biopsy, rash
possible pulmonary problems associated with inflammation myopathies
interstitial lung disease, infection
peak incidence (time) for inflammatory myopathy related malignancy
within 2 years
top 3 malignancies related to inflammatory myopathies
breast, lung, ovary
mainstay of therapy for inflammatory myopathies
corticosteroids
patient population affected mostly by scleroderma
female, 30-40 yo
DDX for Raynaud’s phenomenon
scleroderma, SLE, MCTD, vasculitis, medications
most common cause of death related to scleroderma
interstitial fibrosis/pulmonary HTN
CREST –> variant of limited scleroderma (symptoms)
calcinosis of digits, raynaud’s, esophageal dysmotility, sclerodatyly, telangiectases (digits and under nails)
Ab found in most limited scleroderma patients
anticentromere
markers for renal problems associated with scleroderma
proteinuria, HTN, azotemia, microangiopathic hemolytic anemia
DDX for scleroderma
sclerederma, scleromyxedema, eosinophilic fasciitis, toxic syndroms
specific test for the diffuse scleroderma form
antitopoisomerase I
most common cause of death in Sjogrens
malignancy
possible test used to diagnose Sjogrens…measures lacrimal gland output –> highly sensitive and specific
Schirmer test
Rx treatments for Sjogrens –> to enhance oral and ocular secretions
pilocarpine or Cevimeline
these NT are in lower levels in serum and CSF in fibromyalgia
serotonin and NE
this NT is elevated in CSF in fibromyalgia patients
substance P
type of oligarticular JRA –> female, 50% ANA+, uveitis, long term risk of developing polyarticular
I
type of oligoarticular JRA –> male, 90% B27+, long term risk of ankylosing spondylitis
II
hallmark for systemic JRA onset
spiking fever
post-infectious arthritis that are on DDX for JRA
yersinia and rheumatic fever
anemia common in JRA
normocytic hypochromic
characteristic change of cervical spine in Xray of JRa –> complication in polyarticular JRA
atlantoaxial subluxation
main treatments for JRA
NSAIDs, local corticosteroids, methotrexate (if don’t respond to NSAIDs)
triad of Reiter’s syndrome
urethritis, conjunctivitis, arthritis
primary site of inflammation in seronegative spondyloarthropathies
enthesis
possible extra-articular manifesations of ankylosing spondylitis
prostitis, iritis and conjunctivitis, upper lobe fibrosis (lung), CV, cauda equina inflammation
will see dacytlitis (sausage fingers) and “lovers heel” in this arthritis
reiter’s
distribution of Reiter’s syndrome arthritis
pauciarticular, large joints, lower extremity
characteristic skin lesions seen on soles, glans penis and toes in Reiter’s
keratoderma blenorrhagica
these forms of arthritis can have accompanying “sausage fingers” (dactylitis)
Reiter’s and psoriatic
deficiency in these enzymes can cause overproduction of uric acid
HGPRT, G6PD, F1P aldolase
overactivity of this enzyme can cause overproduction of uric acid
PRPP
primary reasonse for under excretion of urate
deficiency urate exporter, medullary cystic kidney disease (kids)
drugs that promote hyperuricemia
diuretics, organic acids (salicylates, pyrazinamide), cyclosporine, ehtambutol
treatment of choice in acute gouty arthritis
NSAIDs (indomethacin)
alternative to NSAID treatment of acute gouty arthritis…but does have side effect of N/V, abdominal cramps, severe diarrhea
colchicine
drugs for prophylaxis of gouty attack
uricosuric drugs or allopurinol
what does allopurinol inhibit? (*used when uric acid is >800 mg/day*)
xanthine oxidase
prophylaxis for gout if uric acid is undersecretion of urate (*drug works to increase excretion*)
uricosuric drugs
will see this on Xray of pseudogout –> cartilage calcification
chondrocalcinosis
only potent uricosuric agent in US (increases uric acid secretion)
probenecid
these crystal deposition in articular cartilage is closely tied to OA –> can also cause chondrocalcinosis
basic calcium phosphate
most common BCP crystal –> amorphous not visible with polarized light, chronic low grade inflammation and cartilage/bone damage
hydroxyapatite
most commonly affected by hydroxyapatite (BCP) crystals –> can cause Milwaukee shoulder in elderly women
knee, shoulders, hips, fingers
deposition disease associated with patients in end-stage renal disease who need hemodialysis and received ascorbic acid supplements
calcium oxalate deposition
