Rheum part 1 Flashcards
Pain and stiffness in shoulders and hips/pelvic girdle lasting several weeks with no other cause, worse with inactivity, resulting in nocturnal pain and morning predominance w/ morning stiffness >45 min
No blindness or cranial symptoms
Fever, malaise, weight loss, pain in lower back
Difficulty combing hair, putting on coat, rising from chair
NOT causing true muscle weakness
polymyalgia rheumatica
What is polymyalgia rheumatica commonly associated with?
giant cell arteritis
Is polymyalgia rheumatica or temporal arteritis associated with blindness?
temporal arteritis
Clinical → labs = elevated acute phase reactants (ESR, CRP, anemia)
PE: strength is NORMAL
polymyalgia rheumatica
how do you treat polymyalgia rheumatica?
None of the following”above head” symptoms (headache, jaw pain, scalp tenderness, visual symptoms): Prednisone daily with tapering after 2-4 weeks and low dose for one year
Methotrexate for more successful taper
Refractory = biologics
Headache, jaw claudication, visual abnormalities, scalp tenderness, throat pain, jaw pain with chewing, throat pain
Temporal artery
CAN cause blindness (occlusion of posterior ciliary branch of ophthalmic artery) + large artery complications – amaurosis fugax, diplopia
Dry cough, FUO, rigor and sweats, painful paralysis, vague tongue, nose, ear pain
temporal arteritis
Affects medium and large sized vessels
Nonclassical presentation = aortic regurgitation, arm claudication, dry cough, mononeuritis multiplex, FUO, vague pain
If older with FUO with elevated ESR/CRP and normal WBC, must be considered even w/o typical features
temporal arteritis
PE: Temporal artery normal or nodular, enlarged, tender, pulseless
Fundoscopic exam may be normal for first 24-48 hours after blindness occurs
Asymmetry of pulses in arms, aortic regurgitation murmur, bruits near clavicle (subclavian artery stenosis)
→ aneurysm of thoracic aorta
LABS:
Normal WBC
ESR > 50
CRP slightly more sensitive, elevated but can be normal
Normochromic, normocytic anemia
Thrombocytosis
Alkaline phosphatase elevated
→ temporal artery biopsy for confirmation
US = “halo sign” on temporal artery
temporal arteritis
when using an US on the temporal artery, a halo sign indicates
temporal arteritis
how do you treat temporal arteritis
Prednisone started immediately for at least one month and then taper
If vision loss → IV methylprednisolone (usually vision loss is permanent :()
Tocilizumab - IL-6 antibody to reduce flares and steroid use
How do you confirm temporal arteritis diagnosis?
MRI or CT angiography can help confirm diagnosis with narrowing, thickening or aneurysmal dilation
those with temporal arteritis are also at higher risk for
TAAs, regurgitation, dissection
Chronic and widespread aching pain and stiffness most commonly in neck, shoulders, low back and hips with fatigue, sleep problems, subjective numbness, chronic headaches
Minor exertion worsens pain and fatigue
Higher intramuscular pressure
fibromyalgia
fibromyalgia is seen in
women 20-50 years, often correlated with IBS
Syndrome of chronic, widespread MSK pain and stiffness w/ unknown cause - consider sleep disorders, depression, viral infections, rare complication of other illness (hypothyroidism, RA, OSA)
fibromyalgia
PE: normal except for “trigger points” of pain produced with palpation of various areas (ex: trapezius, medial fat pad of knee, lateral epicondyle of elbow)
Dx of EXCLUSION – TSH helpful due to hypothyroidism risk
Rule out other diagnoses!
WPI and SS scales to dictate diagnosis
fibromyalgia
whats the criteria for fibromyalgia
1) Widespread pain index (WPI) score of 7 or higher and symptom severity scale score of 5 or higher
(OR WPI 3-6 and SS 9 or higher)
2) experienced this at least 3 months
3) no other disorder could explain the symptoms
How do you treat fibromyalgia?
Multidisciplinary:
Patient education, CBT, meditation
Exercise programs, massage, therapy
Cannabis, treat depression and anxiety
Amitriptyline, fluoxetine, duloxetine, chlorpromazine, milnacipran, cyclobenzaprine, pregabalin, gabapentin, low-dose naltrexone
FDA approved: pregabalin, duloxetine, milnacipran
Insidious onset with fever, malaise, weight loss weeks-months
Pain in extremities - arthralgia, myalgia (calves), neuropathy (mononeuritis multiplex) w/ foot drop MC
Skin = livedo reticularis (could be from cold), subcutaneous nodules, ulcers, gangrene, commonly in lower extremity near malleoli
With abdominal involvement = N/V, diffuse abdominal pain from eating (postprandial)
Can cause perforation, HOTN
polyarteritis nodosa
polyarteritis nodosa is ass with
vasculitis neuropathy (mononeuritis multiplex)
Necrotizing arteritis/vasculitis of medium-sized vessels affecting skin, peripheral nerves, mesenteric vessels, heart, brain
Can be caused by hep B, rarely genetic (adenosine deaminase 2)
Does NOT affect the lungs, but can cause MI secondary to vasculitis/myocarditis
polyarteritis nodosa
HTN due to renal artery dysfunction
LABS: Anemia, leukocytosis, ESR and CRP elevated
ANCA negative
Rh factor or ANA present but nonspecific
Hep B serologic tests
If childhood onset – genetic testing
Tissue biopsy or angiogram required for confirmation of diagnosis of symptomatic sites
Angiogram = aneurysmal dilations in renal, mesenteric, hepatic arteries (careful)
polyarteritis nodosa
What is required for diagnosis of polyarteritis nodosa?
tissue biopsy or angiogram of symptomatic sites
How do you treat polyarteritis nodosa?
Mild = NSAIDs or colchicine
Severe = IV methylprednisolone
Cyclophosphamide or other immunosuppressive agents lower risk of disease-related death (methotrexate or azathioprine)
Steroids in high doses to help control symptoms
Hep B = short course of prednisone + HBV therapy and plasmapheresis
Genetics = TNF inhibitors
Progressive or abrupt muscle weakness weeks-months involving proximal and symmetric muscle groups of upper + lower extremities and neck
Difficulty climbing stairs, rising from chair, generally preceding upper
NO facial or ocular weakness
Pain and tenderness of muscles
Dysphagia
Late = muscle atrophy and contractures
50-60 years, women>men
polymyositis
LABS: serum muscle enzymes CK and aldolase are elevated
LFTs elevated
ESR and CRP, Hbg normal
ANA elevated
Autoantibodies present –
anti-Jo-1
anti-Mi-2
anti-MDA5
anti-155/140 (cancer)
Anti-140 (juvenile)
anti-SAE (cancer)
Biopsy of clinically involved muscle
MRI helps to detect early involvement, EMG abnormalities
polymyositis
How do you treat polymyositis?
Prednisone, often long term
Monitor muscle strength and muscle enzymes, immunosuppressive drugs may reduce need for steroids
(methotrexate, azathioprine, mycophenolate)
Refer!
Admit if signs of rhabdomyolysis, dysphagia, respiratory insufficiency
polymyositis is infiltration of
endomysium
polymyositis is a type of
idiopathic inflammatory myopathy
Fever, anorexia, malaise, weight loss, skin lesions, fatigue
“Butterfly” malar rash
Panniculitis, discoid lupus, fingertip lesions, periungual erythema, nail fold infarcts and splinter hemorrhages
Alopecia
Lesions of mucous membrane lesions, Raynaud phenomenon
Joint symptoms as earliest manifestation → swan neck deformity
EYES: conjunctivitis, photophobia, transient/permanent blindness, blurry vision
LUNGS: pleurisy, pleural effusion, pneumonia, pneumonitis is frequent
HEART: HF, arrhythmia, endocarditis, pericarditis, mitral regurgitation
Mesenteric vasculitis
NEUROLOGIC: psychosis, cognitive impairment, seizures, peripheral/cranial neuropathies, stroke
Glomerulonephritis and interstitial nephritis (kidney involvement!)
lupus
common in women, after menarche and before menopause
Black > white
Genetically passed through maternal line
Sunlight exposure
Increased estrogen (reproductive age)
lupus
Inflammatory autoimmune disorder - autoantibodies to nuclear antigens
Suspect in any patient with multisystem disease and + ANA test (rule out drug induced!)
Can be induced by hydralazine (if taken >1 year), isoniazid, sulfasalazine, quinidine, minocycline, procainamide – discontinue agent!
lupus
What drugs can cause lupus
Can be induced by hydralazine (if taken >1 year), isoniazid, sulfasalazine, quinidine, minocycline, procainamide – discontinue agent!
PE: cotton wool spots on retina
LABS:
ANA + – initial screening TOC
anti-Sm antibodies (specific)
Anti-double-stranded DNA antibody levels = disease activity/response to treatment
SS-A/Ro, SS-B/La, ribonucleoprotein (RNP) phospholipid
Lupus nephritis → abnormal urinary sediment (urinary RBCs, w or w/o casts, proteinuria frequent)
Flare = high ESR
Entry criteria: + ANA
If yes → additive criteria, need >/= 10 points
lupus
SOAP BRAIN MD
Serositis
Oral or nasal ulcers
Arthritis in >2 joints
Photosensitivity
Blood disorders
Renal involvement
Ana +
Immunologic
Neurologic symptoms
Malar rash
Discoid rash
How do you treat lupus
Avoid sun exposure, use sunscreen
Tailor to specific patient and severity!
Skin lesions = topical steroids
Joint pain = rest and NSAIDs
Hydroxychloroquine for initial therapy of rash and joint symptoms
Steroids for controlling complications (acute exacerbations), more severe
Immunosuppression with mycophenolate, azathioprine, methotrexate
Biologics
Flu vaccine annually and pneumococcal vaccine every 5 years
Refer
Admit if rapidly progressing, severe complications, infection
Initial: excessive vasoconstriction with digital pallor or cyanosis
Recovery: vasodilation with hyperemia and rubor, intense throbbing, paresthesia, pain, slight swelling
Fingers but can affect toes, nose, ears
Asymptomatic between attacks
Numbness, stiffness, diminished sensation, aching pain
Young women 15-30y
Rheumatic disease
Raynaud syndrome
Paroxysmal digital ischemia, exaggerated response of digital arterioles to cold or emotional stress
Scleroderma can cause secondary RP with digital pitting, ulceration, gangrene (CREST syndrome)
Raynaud syndrome
How do you treat Raynaud syndrome
Keep warm: wearing warming clothing will help prevent vasospasms
Protect hands from injury, wounds heal slowly and increase infection
Lubricating lotion
Stop smoking
Avoid sympathomimetic drugs (decongestants, diet pills, amphetamines)
Calcium channel blockers (Slow-release nifedipine, amlodipine)
ARBs, sympatholytics, topical nitrates, PDE-V inhibits, SSRIs
IV prostacyclin if threat of digital loss
Sympathectomy if frequent and severe
Initial sign = Raynaud phenomenon and may precede others by YEARS
Polyarthralgia, weight loss, myalgias, malaise (diffuse)
Non-pitting subcutaneous edema associated w/ pruritus becoming shiny, skin becoming hardened involving trunk and proximal extremities
Telangiectasias, pigmentation, depigmentation
Ulceration of fingertips and calcification
Dysphagia and reflux symptoms
GI = fibrosis and decreased motility, diarrhea/constipation, diverticuli
Pulmonary fibrosis and vascular disease
Pericarditis, heart block, myocardial fibrosis, RHF (pulm HTN)
Renal crisis
30-50y
women>men
scleroderma
Diffuse fibrosis of skin and internal organs – chronic with unknown cause
Limited (80%) confined to face, neck (more risk for ischemia), distal extremities or diffuse forms
Limited = CREST syndrome
Calcinosis cutis-calcium salts deposited in skin and tissue
Raynaud phenomenon
Esophageal motility disorder
Sclerodactyly
Telangiectasia
Either can have small/large bowel hypomotility with constipation/diarrhea, malabsorption, obstruction, distention
Diffuse = trunk involved with proximal extremities, tendon friction rubs over forearm/shins (leathery, crepitus feel) w/ lungs, heart, GI tract, kidneys
scleroderma
CREST indicates
scleroderma
CREST means
Calcinosis cutis
Raynaud phenomenon
Esophageal motility disorder
Sclerodactyly
Telangiectasia
LABS: mild anemia, renal crisis (microangiopathic hemolytic anemia, proteinuria)
ANA
Anti-SCL-70 = diffuse
Anticentromere antibodies
anti-RNA polymerase III antibodies
Consider clinically: skin thickening involving fingers, skin manifestations, Raynaud’s, SS-related antibodies
scleroderma
How do you treat scleroderma?
Calcinosis can be observed, but topical sodium thiosulfate, colchicine, minocycline can help
Symptomatic + supportive
Raynaud’s = CCB
Esophageal symptoms = liquid/crushed meds only, avoiding late night meals, + PPI
GI bacteria: abx
HTN renal crisis = captopril or other ACE-I
Interstitial lung disease = mycophenolate mofetil or cyclophosphamide
Methotrexate for skin disease
Digital ulceration = bosentan
Pulmonary HTN = sildenafil or prostaglandins
Immunoablative therapy
