Syndromes Flashcards
What are polymyositis and dermatomyositis?
group idiopathic inflammatory myopathies
Dermatomyositis can be associated with?
malignancy
Does polymyositis and dermatomyositis impact men or women more?
women
Patho of dermatomyositis?
humoral attack to muscle capillaries and small arterioles; activation of C3 forms C3bNEO and MAC; B cells and Cd4 cells activated; destroys capillaries; causes microinfarction in the muscles
Patho of polymositis
T-cell mediated cytotoxic process against muscle antigens
Genetic component of polymyositis and dermatomyositis?
HLA types– DR3, DR5, DR7
Immunologic etiology of polymyositis and dermatomyositis
abnormal t-cell activity
Infectious etiology of polymyositis and dermatomyositis
viruses (coxsackievirus, parvovirus, HIV), toxoplasma species, borrelia species
Drug etiology of polymyositis and dermatomyositis
statins, interferon, quinidine
CM of polymyositis and dermatomyositis
skin is the initial manifestation in dermatomyositis only; proximal muscle weakness (pelvic girdle, upper body weakness, neck extensor muscles); difficulty climbing stairs/walking/standing up from seated position/combing hair/lifting things; possible muscle tenderness to palpation
Systemic manifestations of polymyositis and dermatomyositis
arthralgias and arthritis (knees, wrist, hands), fatigue, weight loss, fever, Raynaud’s, dysphagia, reflux esophagitis, constipation, exertional dypsnea, interstitial lung disease, rhythm disturbances, conduction defects, CHF, pericarditis
PE of dermatomyositis
possible photo-sensitivity over face, chest, hands; Gottron’s papules; rash in V neck distribution; calcinosis
Calcinosis is seen in?
Dermatomyositis and Scleroderma
What’s Gottron’s papules?
raised, scaly palpable lesions on extensor surfaces; MCP, PIP, elbows, upper eyelids
Labs for dermatomyositis/polymyositis
CBC (possible leukocytosis or thrombocytosis), ESR (elevated), creatine kinase (elevated), AST/LDH (may be abnormal), autoantibody studies: ANA (positive), anti-jo-1 (polymyositis), anti-Mi2 and anti-MDA5–dermatomyositis
Which autoantibody study is specific to polymyositis?
anti-jo-1
which autoantibody study is specific to dermatomyositis?
anti-Mi2 and anti-MDA5
Imaging for dermatomyositis/polymyositis
MRI to evaluate for amount of muscle involvement; EMG (abnormal; not done as frequently as MRI); muscle biopsy
What’s necessary for definitive dermatomyositis/polymyositis diagnosis?
muscle biopsy
What are important non-diagnostic testing for dermatomyositis?
colonoscopy, pap smear, mammogram, CA-125, CA 19-9
Diagnostic criteria of dermatomyositis
[Gottron’s papules or muscle biopsy finding of perifasicular atrophy] OR all of the following are present: suggestive skin involvement, proximal weakness (subacute/chronic), muscle biopsy or skin biopsy (perimysial or perivascular inflammation)
Diagnostic criteria of polymyositis?
all of the following: subacute/chronic proximal weakness, elevated serum creatine kinase, muscle biopsy (endomysial inflammation), response to immunotherapy or exclusion of limb-girdle muscular dystrophies
Treatment of dermatomyositis/polymyositis?
high protein diet, daily exercise, avoid UV exposure/use sunscreen, corticosteroids (topical), hydroxychloroquine, methotrexate
What’s the first line treatment (Drug) for dermatomyositis/polymositis?
corticosteroids
Dosage of prednisone for dermatomyositis/polymositis?
prednisone 1 mg/kg/day x 4-8 weeks (occasionally up to 3 months)
When do you discontinue corticosteroid treatment for dermatomyositis/polymyositis?
when CK level is in normal range
Methotrexate dosage for dermatomyositis/polymyositis
7.5 mg po weekly increase by 2.5 mg to 20 mg po weekly as needed (take with folic acid 1 mg)
what is fibromyalgia?
chronic widespread pain and tenderness
What’s the patho of fibromyalgia?
abnormal central sensory processing within the CNS; amplification of peripheral sensory stimuli; descending inhibitory control deficit in midbrain; persistent neuronal hyperexcitability
Etiology of fibromylagia
unknown maybe hormones, sleep disorders, depression, viral infections, rare complication of hypothyroidism, RA, or sleep apnea
Does fibromylalgia impact men or women more often?
women
Age range for women with fibromyalgia?
20-50; increases with age
S/S of fibromyalgia
widespread pain and stiffness (neck, shoulders, low back, hips), fatigue, disordered sleep (non-restorative sleep, restless leg syndrome), cognitive dysfunction, depression, chronic HA, IBS, TMJ pain, chronic pelvic pain, overactive bladder syndrome
Labs for fibromyalgia
CBC with diff, BMP, thyroid stimulating hormone, ESR, CRP, ANA, RF, CPK
Treatment for fibromyalgia
education, counseling/behavior modification, pain, treat causes of fatigue/sleep difficulties, eliminate stressors, deconditioning (PT/OT), maybe treat endocrine dysfunction
Medication treatments for fibromyalgia
analgesics (acetaminophen, tramadol); muscle relaxants (cyclobenzaprine); anti-anxiety; antidepressants; anticonvulsants
Tramadol dosage for fibromyalgia
50 mg 1 6-8 hours PRN (short term)
Cyclobenzaprine dosage for fibromylagia
5 mg po q8 hours PRN
ex of anti-anxiety meds for fibromyalgia
alprazolam, buspiron, clonazepam, zolpidem, zaleplon
ex of antidepressants for fibromyalgia
amitriptyline, duloxetine, venlafaxine
Anticonvulsant ex for treatment of fibromyalgia
gabapentin, pregabalin
What’s CFS?
myalgic encephalomyelitis
Cause of CFS?
viral association? EBV, coxsackievirus B
Are men or women more commonly impacted by CFS?
women
CM of CFS
sudden onset abrupt, debilitating fatigue (impacts ADL, relationships, work funcitons); possible flu-like prodrome; generalized MSK pain, cognitive dysfunction, IBS, nonrestorative sleep
How long does the fatigue have to be to be diagnosed with CFS?
persistent chronic fatigue >6 months or intermittent, unexplained chronic fatigue (relapses and definite start) and not the result of exertion and is not relieved by rest
Outside of fatigue, what other criteria can be met for CFS (4/8)
recently impaired memory/concentration, pain on swallowing, painful axillary/cervical lymph nodes, muscle pains, joint pain without swelling, HA, sleep that does not improve after rest, post-exertional discomfort lasting >24 hours
What disorders cause fatigue?
heart disease, thyroid disease, chronic anemia, liver and renal disease, psychiatric illness, fibromyalgia
Work up for CFS?
r/o
Treatment of CFS?
supportive and symptomatic
What is complex regional pain syndrome?
affects the extremities (pain, swelling, and vasomotor dysfunction) following trauma/surgery/soft tissue disorder/immobilization
Who does complex regional pain syndrome impact more?
women
Age of average complex regional pain syndrome?
30-60 with mean age at 40
CM of complex regional pain syndrome
pain out of proportion, edema (disproportionate), redness/cyanosis/pale, stiffness/decreased ROM, muscle weakness/tremors, excessive sweating/dryness/shiny/wrinkled/temp changes, flexion contractures, frozen shoulder, joint fibrosis
How many extremities does CRPS impact?
usually only one
what are the three clinical stages of CRPS?
acute, subacute, chronic
duration of acute CRPS
3 months
describe acute CRPS
burning pain, swelling, redness, hyperhidrosis, coolness to touch, functional limitations, bone demineralization
duration of subacute CRPS
9 months
describe subacute CRPS
severe pain, fixed edema, pallor and cyanosis, dry skin, loss of function (joint fibrosis)
Duration of chronic CRPS
1 year after onset
describe chronic CRPS
variable pain, decreased edema, dry/pale/cool/shiny skin, osteoporosis, loss of function (fibrosis)
how long does CRPS last?
chronic can last for years or be permanent
labs for CRPS
nothing specific
imaging for CRPS
pain films (bone demineralization), triple phase bone scan (increased uptake in articular and periarticular structures)
treatment of CRPS
patient education, meds (NSAIDs/corticosteroids), sympathetic nerve block, PT/OT, surgical last resort
surgical treatment of CRPS
upper thoracic/lumbar symphathectomy, chemical sympathectomy, spinal cord stimulator implantation
Genetic dominance of Marfan
AD
S/S of Marfan
lens displacement, risk of retinal detachment, increased limb and finger length/sternal deformity, ligamentous laxity, arched palate, crowded dentition, malocclusion, MVP, aortic root dilation, spontaneous pneumothorax, sleep apnea, striae atrophy, cystic organs
What’s Ehlers-Danlos syndrome?
heterogenous disorder of connective tissue; affects structure, production, or processing of collagen
Hallmarks of Ehlers-Danlos
tissue fragility, joint hypermobility, skin hyperextensibility
Ehlers-Danlos s/s
joint hypermobility, skin atrophy/velvety skin, medium and large vessel rupture, easily bruises, organ rupture (uterus, large bowel, pneumothorax), premature rupture of fetal membranes
treatment of ehlers-danlos syndrome
manifestations of the syndrome, care with suturing, prophylactic beta blocker to protect vessels maybe
Osteogenesis imperfecta
hereditary osteopenia due to defects in bony protein matrix
s/s of osteogenesis imperfecta
recurrent fractures with deformity, impaired mobility, short stature, restrictive lung disease (vertebral column deformity/rib fractures), compression of brain stem, abnormal teeth, BLUE SCLERA
Tx of OI
Risedronate to increase bone mineral density
