connective tissue ds Flashcards
a pt comes with pain in both wrists , distal interphalygeal, metacarpophalyngeal jts wht r diffrential diagnosis
rheumatoid arthritis
SLE
viral arthritis
hepatitis B
EBV
Parvovirus B19
parvovirus b19 adult dont hve slapped cheek as in children usually adult who comes alot in contact with children like kindergarden teacher
how to differentiate rheumatoid arthritis and SLE
systemic symptoms present in SLE
a pt comes with involvement of knee from yrs
monoarticular chronic 》 osteoarthritis
no joint swelling and redness
noninflammtory thus
⬇️
osteoarthritispt comes with pain and swelling of knee from 2-3 days
monoarticular acute inflammatory
↙️ ↘️
septic arthritis gout
pt comes with migratory arthritis wht r diffrential
lyme ds
gonococcal arthritis
rheumatic fever
oligoarticular asymmetrical <5-6 joints involved wht r the diffrential
spondyloarthopathies
osteoarthritis
ANA VERY SPECIFIC FOR LUPUS ARE
ant DDNA
ANTI SMITH
anti histone ab is seen in
drug induced lupus
anti SSA (anti rho )
neonatal lupus
sjogren
anti ssb( anti LA )
sjogren
anti centromere is seen in
CREST
anticentromere antibodies are specific for
limited form of scleroderma
antibody specific for diffuse scleroderma
antitopoisomerase I ie antiscleroderma 70
clinical features of scleroderma
1.Raynauds syndrome
caused by vasospasm and thickening of vessel wall in digits
cold temperature and stress brings first blanching 》cyanosis》reactive hyperemia
2.cutaneous fibrosis
tightening of skin and face leads to sclerodactyly refers to claw hand
3.esophageal dismotility
4.lung, heart,renal involvement
difference bw diffuse and limited scleroderma
diffuse limited
skin involvement limited to distal extremities
widespread sparing of trunk
2.rapid onset delayed onset
3.significant organ involved organs involved late
4.no anticentromere anticentromere +
antitopoisomerase
diffrential diagnosis of raynauds phenomenon
PRIMARY no cause SECONDARY scleroderma SLE vasculitis certain medication bblocker, nictotine,bleomycin thromboangitis obliteratans
drugs causing lupus
quinidine
hydralazine
procainamide
isoniazid
pattern of joint involvement in reactive arthiritis
migratory arthritis
aymmertric inflammatory oligoarthritis
Reiter syndrome triad
arthritis
uveitis
urtheritis
organisms implicated in reiter syn
salmonella shigella campylobacter chlamydia yersenia
types of seronegative spondyloarthopathies
ankylosing spondylitis reiter syn psoriatic arthritis arthopathy with IBD undifferentiated spondyloarthopathies
sausage shaped digits and nail pitting characteristic of
psoriatic arthritis
pattern of joint involvement
asymmetric and polyarticular
difference between joint involvement of psoriatic and reiter
psoriatic polyarticular
reiter oligoarticular
felty syndrome
anemia
neuropenia
splenomegaly
Rheumatoid arthritis
juvenile RA
begins before 18 yrs of age
extraarticular manifestations may predominate
caplan syn
CWP + rheumatoid nodules
deformity in rheumatoid arthritis
•hallux valgus deformity, commonly called a bunion, is when there is medial deviation of the first metatarsal and lateral deviation of the great toe (hallux)
•ulnar deviation of hand
•swan neck
•boutonniere deformity
•hammer toe
Ahammer toeorcontracted toeis adeformityof theproximalinterphalangeal jointof the second, third, or fourthtoecausing it to be permanently bent, resembling ahammer.Mallet toeis a similar condition affecting thedistalinterphalangeal joint.[1][2]
wht is DISH
Diffuse idiopathic skeletal hyperostosis(DISH) is a condition
characterized by abnormal calcification/bone formation (“hyperostosis”) of the soft tissues surrounding the joints of the spine, and also the peripheral or appendicular skeleton.[1]In the spine, there is bone formation along theanterior longitudinal ligamentand sometimes theposterior longitudinal ligament, which may lead to partial or completefusionof adjacent spinal levels. The facet and sacroiliac joints tend to be uninvolved. The thoracic spine is the most common level involved.[2]In the peripheral skeleton, DISH manifests as a calcificenthesopathy, with pathologic bone formation atsites where ligaments and tendons attach to bone.
other names if DISH
Forestier’s disease,
senile ankylosing spondylosis,
ankylosing hyperostosis
diagnostic criteria of DISH
Diagnostic criteria
•flowing ossification along the anterolateral aspect ofat least 4 contiguous vertebraepreservation ofdisk heightin the involved vertebral segment;
• relative absence of significant degenerative changes (e.g. marginal sclerosis in vertebral bodies or vacuum phenomenon)
absenceof facet-joint ankylosis
•; absence of SI joint erosion, sclerosis or intraarticular osseous fusion
wegener granulomatosis
upper and lower RESPIRATORY tract
renal involvement
vasculitis small vsel
cANCA +ve
c ANCA is positive in
wegener granulomatosis
temporal/ giant cell arteritis
temporal arteries most commonly effected
#symptoms
headache
intermittent jaw claudication
visual impairment can lead to blindness
tender temporal arteries
also associated with polymyalgia rheumatica ie proximal stiffness ( shoulder, hip)
mnemonic 60 age of patient
60 mg prednisone dose
60 ESR needed to diagnose
treatment of temporal arteritis
clinical features of temporal arteritis
⬇️
do ESR and temporal artery biopsy
if ESR high immediately start 60 mg prednisone without waiting for temporal artery biopsy results
large vsel vasculitis
temporal arteritis
takayasu arteritis
medium vessel vasculitis
chiurg strauss PAN wegner granulomatosis Kawasaki ds microscopic polyangitis
small vessel vasculitis
henoch scholein purpura
hypersensitivity vasculitis
behcet syndrome
takayasu vasculitis
granulomatos vasculitis of arch of aorta
leading to its stenosis and narrowing
decreased or absent peripheral pulses
discrepencies of BP
ARTERIAL BRUITS
churg strauss syndrome
prominent respiratory tract finding AASTHMA
EOSINOPHILIA
skin lesions palpable purpura , subcutaneous nodules
kidney
PANCA + VE
PAN
NO LUNG INVOLVEMENT effects nervous sys and GIT associated with hepaitis B nerves involvement leads to mononeuritis mutiplex git abdominal angina
PANCA + ve
behcet syndrome
vasculitis causing
painful oral and genital ulcer
eye involvement
CNS involvement
arthritis (knee and ankle most common )
buerger ds symptoms
thromboangitis obliteratans
occurs in young men smokers
segmental inflammation of arteries and veins
may lead to gangrene and autoamputation
symptoms
claudication
painful distal extremities
ulceration
raynauds phenomenon
causes of proximal myopathy
eaton labert
myasthenia
endocrinopathies
hyper /hypo thyroisidsm
adrenal insufficiency
drug induced
cyclosporine
statin
inflammatory myopathy
polymyositis
dermatomyositistypes of idiopathic inflammatory myopathies
polymyositis
dermatomyositis
symptoms of polymyositis
Symmetrical proximal muscle weakness that develops subacutely over weeks or several months
•The earliest andmost.severely.affected muscle groups are the
neckflexors,
shoulder girdle, and
pelvic girdle muscles
symptoms and signs of dermatomyositis
symptoms of polymyositis + skin involvement
Heliotrope rash (butterfly)—around eyes, bridge of nose, cheeks
b. Gottron papules—papular, erythematous, scaly lesions over the knuckles (MCP,PIP,DIP)
c. V sign—rash on the face, neck, and anterior chest
d. Shawl sign—rash on shoulders and upper back, elbows, and knee
s e.Periungual erythema with telangiectases
f. Subcutaneous calcifications in children—can be extremely painful
g. Associated with vasculitis of the GI tract, kidneys, lungs, and eyes (more common in children)
h. There is an increased incidence of malignancy in older adults (lung, breast, ovary, GI tract, and myeloproliferative disorders). Once dermatomyositis is diagnosed, make an effort to uncover an occult malignancy. Dermatomyositis associated with malignancy often remits once the tumor is removed
diagnostic criteria of polymoyositis
If two of first four→possible polymyositis
If three of first four→probable polymyositis
If all four→definite polymyositis
- Symmetric proximal muscle weakness
- Elevation in serum creatine phosphokinase
- EMG findings of amyopathy
- Biopsy evidence of myositis
- Characteristic rash of dermatomyositis
odd ball of inflammatory myopathies
inclusion body myositis is the“odd ball of Inflammatory myopathies”for the following reasons
:Affects male patients more than female patients,
absence of autoantibodies,
distal muscle involvement, and relatively
low creatine kinase(CK);
prognosis is poor.
lab finding of polymyositis
CK level is significantly elevated.
CK levels correspond to the degree of muscle necrosis, so one can monitor the disease severity
b. LDH, aldolase, AST, ALT elevated
c. ANA in over 50%
d. Antisynthetase antibodies (anti-Jo-1 antibodies)—abrupt onset of fever, cracked hands,Raynaud phenomenon,interstitial lung disease and fibrosis,arthritis; does not respond well to therapy
e. Antisignal recognition particle •Cardiac manifestations(common) •Worst prognosis of all subsets f. Anti-Mi-2 antibodies—better prognosis
2. EMG—abnormal in 90% of patients
3. Muscle biopsy
a. Shows inflammation and muscle fiber fibrosis in all three b. Dermatomyositis—perivascular and perimysial c.Polymyositis and inclusionbodymyositis—endomysial
biopsy finding of polymyositis
Polymyositis and inclusion body myositis—endomysial
muscle finding found in dermatomyositis
Dermatomyositis—perivascular and perimysial inflammation
inclusion body myositis
Insidious onset of slowly progressive proximal and distal weakness,
Patients can also have loss of deep tendon reflexes(nerves are not involved in polymyositis and dermatomyositis)
Not associated with autoantibodies
