What is fibromyalgia?
a common condition characterized by widespread pain in joints, muscles, tendons, and other soft tissues
- the cause of the disorder is unknown, physical or emotional trauma may play a role
- three months of widespread pain at 11 of 18 tender sites
- DX is clinical
What is the tx of fibromyalgia?
treat with tricyclics (TCAs) - cymbalta, SSRIs, neurontin, and exercise
-pregabalin (lyrica) is the only drug FDA approved to treat fibromyalgia
What is the American College of Rheumatology criteria for the classification of fibromyalgia?
- widespread pain for at least 3 months, defined as the presence of all of the following:
- pain on the right and left sides of the body
- pain above and below the waist (including shoulder and buttock pain)
- pain in the axial skeleton (cervical, thoracic, or lumbar spine; anterior chest)
- pain on palpation with a 4-kg force in 11 of the following 18 sites (9 bilateral sites, for a total of 18 sites):
- occiput: at the insertions of one or more of the following muscles: trapezius, sternocleidomastoid, splenius capitis, semispinalis capitis
- low cervical: at the anterior aspect of the interspaces between the transverse processes of C5 to C7
- trapezius: at the midpoint of the upper border
- supraspinatus: above the scapular spine near the medial border
- second rib: just lateral to the second costochondral junctions
- lateral epicondyle: 2 cm distal to the lateral epicondyle
- gluteal: at the upper outer quadrant of the buttocks at the anterior edge of the gluteus maximus muscle
- greater trochanter: posterior to the greater trochanteric prominence
- knee: at the medial fat pad proximal to the joint line
What is gout?
involves the accumulation of uric acid in the soft tissue of joints and bone
- altered purine metabolism and sodium urate crystal precipitation into the synovial fluid, M>W (9:1) until menopause (1:1)
- usually young, >30 yo, asymmetric; great toe; tophi
What are the s/s of gout?
MC = podagra (attack of MTP of the great toe) (70% of cases); pain, swelling, redness, exquisite tenderness
-in chronic gout = tophi
How is the dx of gout made?
diagnosis is by arthrocentesis - rod-shaped negatively birefringent
- serum uric acid level > 8 (not diagnostic)
- imaging: small, punched out lesions on XR = high likelihood diagnosis
What is the tx for gout?
lifestyle: elevation, rest, decrease purines (meats, beer, seafood, alcohol) weight loss, increase protein, limit alcohol
- pharm NSAIDs = drug of choice (indomethacin tid); colchicine = effective but bad GI s/e; steroid injections for those who can’t take NSAIDs, oral pred if other meds not tolerated
- thiazide diuretics and aspirin should be avoided
- the management between acute attacks: colchicine, allopurinol
- don’t start someone on allopurinol in an acute attack
What is pseudogout?
usually > 60 yo; large joints, lower extremity, no tophi
-similar gout symptoms
How is pseudogout dx?
rhomboid-shaped calcium pyrophosphate crystals - positively birefringent
-XR shows fine, linear calcification in cartilage
What is the tx of pseudogout?
NSAIDs, colchicine, intra-articular steroid injections
-colchicine = prophylaxis, NSAIDs = acute attack
What is polyarteritis nodosa?
a blood vessel disease characterized by inflammation of small and medium-sized arteries (vasculitis), which can restrict blood flow and damage vital organs and tissues
What are the characteristics of polyarteritis nodosa?
- most common in middle-aged men in their 40-50;s
- associated with Hepatitis B and C - increased microaneurysms with aneurysmal rupture leading to hemorrhage and thrombosis as well as organ ischemia or infarction
- damage to affected artery = hypertension, aneurysm, thrombosis, necrosis
- renal: HTN 2/2 increased renin production (may progress to renal failure)
- constitutional: fevers, myalgias, arthritis
- CNS: neuropathy, amaurosis fugax, peripheral neuropathy
- Dermatologic: livedo reticularis, purpura, ulcers, gangrene
How is polyarteritis dx?
required confirmation with either a tissue biopsy or angiogram
- biopsy demonstrates necrotizing arteritis or arteriography showing the typical aneurysms in medium-sized arteries
- increased ESR, most common in middle age men of 45 years old
- classic PAN is ANCA negative (P-ANCA positive in <20% cases)
- renal or mesenteric angiography: microaneurysms with abrupt cut-offs of small arteries)
What is the tx of polyarteritis?
steroids (prednisone) +/- cyclophosphamide if refractory
-plasmapheresis in patients with hepatitis B virus
What is polymyalgia rheumatica?
is an idiopathic inflammatory condition AFFECTING THE JOINTS causing PAINFUL synovitis, bursitis, and tenosynovitis - aching STIFFNESS of PROXIMAL JOINTS (shoulder, hip, neck) in patients > 50 years old
What are the characteristics of polymyalgia rheumatica?
-PMR is closely related to giant cell arteritis (temporal arteritis)
-joint pain versus muscle pain in polymyositis
-STIFFINESS versus the weakness of polymyositis
ESR is markedly elevated (usually > 50)
-temporal arteritis is confirmed by TEMPORAL ARTERY BIOPSY
How is polymyalgia rheumatica tx?
patient respond quickly to low-dose corticosteroid therapy, which may be required for up to 2 years and slowly tapered
-methotrexate may also be used
What is polymyositis?
a chronic, idiopathic inflammatory DISEASE OF THE MUSCLE causing symmetrical, proximal, PAINLESS (versus polymyalgia rheumatica) muscle weakness
- i.e quadriceps femoris
- different from dermatomyositis which is characterized by inflammatory and degenerative changes in the skin and muscles
- differentiate from Polymyalgia Rheumatica (PMR) by lack of pain which causes stiff joints usually of the shoulder, hip, and neck
How is polymyositis dx?
diagnosis of PM/DM involves a physical examination of muscle strength, blood tests for muscle enzymes, electrical tests of muscle and nerves and is confirmed by muscle biopsy
- increased enzymes: increased aldolase, creatine kinase; increased ESR (+) muscle biopsy, abnormal EMG
- (+) ANTI-JO 1 Ab: myositis-specific antibody-associated with interstitial lung fibrosis
- “mechanical hands” hyperkeratotic cracked hands with a dirty appearance
- (+) Anti-SRP Ab: signal recognition particle Ab
- (+) Anti-Mi-2 Ab: specific for dermatomyositis
- muscle biopsy: endomysial involvement with PM
What is the tx for polymyositis?
corticosteroids and sometimes other immunosuppressants (methotrexate/azathioprine)
What is reactive arthritis (Reiter syndrome)?
autoimmune response to infection in another part of the body (Chlamydia +/- gonorrhea MC)
- asymmetric inflammatory arthritis
- conjunctivitis, uveitis, urethritis, and arthritis (can’t see, can’t pee, can’t climb a tree)
- most commonly seen in Chlamydia (+/- gonorrhea and GI infections such as salmonella, Shigella, Campylobacter, Yersinia)
- Diagnosed by a history of infection, clinical exam, positive HLA-B27 (80%)
What is the tx for reactive arthritis (Reiter syndrome)?
NSAIDs are the mainstay of therapy, antibiotics to treat the infection that triggered the disease (Chlamydia)
What is rheumatoid arthritis?
MORNING JOINT STIFFNESS > 30 minutes after initiating movement and improves later in the day (vs OA which gets worse throughout the day and if morning stiffness is present will be <30 minutes)
- prodrome of constitutional symptoms including fevers, fatigue, weight loss, and anorexia
- Small joint stiffness (MCP, wrist, PIP, knee, MTP, shoulder, ankle) worse with rest
- symmetric arthritis: swollen, tender, and boggy joint
a. boutonniere deformity: flexion at PIP, hyperextension of DIP
b. swan neck deformity: flexion at DIP with joint hyperextension at PIP
c. ulnar deviation at MCP joint
d. Rheumatoid nodules
What are the diagnostic studies for rheumatoid arthritis?
- (+) Rheumatoid factor (sensitive but not specific); increased CRP and ESR
- (+) anti-citrullinated peptide antibodies (most specific for RA)
What is the tx for rheumatoid arthritis?
Prompt initiation of DMARDs
- methotrexate - methotrexate (MTX) is the cornerstone for therapy for RA and is effective as monotherapy for many patients
- Hydroxychlorquine (Plaquenil) - hydroxychloroquine (HCQ) may be added to a number of traditional DMARDs, including MTX, to improved response, HCQ is less effective as monotherapy
- Sulfasalazine - sulfasalazine (SSZ) is often used in combination with HCQ and MTX as part of the so-called “triple therapy” regimen, when used in this combination, response rates may rival those seen with biologic DMARDs
- Leflunomide - LEF is effective as monotherapy for both RA and spondyloarthritis, and therefore may be a good choice when the clinical diagnosis is less clear, diarrhea is a common side effect, however, and may limit its use
- other - other drugs with a much more limited role, due to the more effective and/or safer agents that are available including azathioprine, gold, and cyclosporine
- NSAIDs for pain control and low dose corticosteroids
- Biologic agents - the development of biologic agents represents an advance in the treatment of RA comparable to the introduction of glucocorticoids
- the cytokines interleukin (IL)-1 and tumor necrosis factor (TNF)-alpha both mediate inflammation and bone resorption in RA
- agents that inhibit B-cell function, T cell function, and the actions of proinflammatory cytokines (eg, the human recombinant IL-1 receptor antagonist, anakinra, and anti-TNF-alpha agents including entanercept, infliximab, and adalimumab) are all used for the treatment of RA
What is sjogren syndrome?
a relatively common chronic, autoimmune, systemic, inflammatory disorder of unknown cause attacking the exocrine glands
What are the characteristics of Sjogren syndrome?
characterized by dryness of the mouth, eyes, and other mucous membranes due to lymphocytic infiltration of the exocrine gland and secondary gland dysfunction
- salivary glands - xerostomia (dry mouth)
- lacrimal glands - dry eyes (keratoconjunctivitis sicca)
- parotid enlargement
How is Sjogren syndrome dx?
ANA (especially anti-SS-A (RO) and anti-SS-B (La)
- (+) Rheumatoid factor (RF)
- (+) schirmer test (<5 mm lacrimation in 5 min)
What is the tx for Sjogren syndrome?
treat with artificial tears, pilocarpine (cholinergic) for xerostomia
- pilocarpine: a cholinergic drug that increased lacrimation and salivation (side effects include diaphoresis, flushing, sweating, bradycardia, diarrhea, N/V, incontinence and blurred vision)
- cevimeline: stimulates muscarinic cholinergic receptors
What is systemic lupus erythematosus?
triad of join pain + fever + malar (butterfly rash) - fixed erythematous rash on cheeks and bridge of nose sparing nasolabial folds
- (+) anti-nuclear Ab (ANA): ANA best initial test (not specific)
- (+) anti-double-stranded DNA and Anti-Smith ab: 100% specific for SLE (not sensitive)
How do you dx systemic lupus erythematous?
4 or more of the following 11 criteria met
- malar rash (butterfly rash)
- discoid rash (chronic, can scar)
- photosensitivity (other rashes from sun exposure)
- mucosal involvement (ulcers, mouth, and nose)
- serositis (pleuritis, pericarditis)
- joint arthritis (2 or more)
- renal disorders (abnormal urine protein, diffuse glomerulonephritis)
- neurologic disorders (anemia, thrombocytopenia, leukopenia)
- other antibodies: Anti Smith, Anti-dsDNA, Anti-phospholipid (Anticardiolipin, lupus anticoagulant, Anti-B2 glycoprotein)
What is the tx for systemic lupus erythematous?
manage with sun protection, hydroxychloroquine (for skin lesions), NSAIDs or acetaminophen for arthritis
-pulse dose steroids; cytotoxic drugs (methotrexate, cyclophosphamide)
What is systemic sclerosis (scleroderma)?
systemic connective tissue disorder causing thickened skin (sclerodctyly), lung, heart, kidney, and GI tract
- tight, shiny, thickened skin due to fibrous collagen buildup
- limited cutaneous systemic sclerosis “CREST SYNDROME” - calcinosis cutis, Raynaud’s phenomenon, esophageal motility disorder, sclerodactyly (claw hand), telangiectasis)
- affects the face, neck as well as distal to the elbow and knees
- Raynaud’s phenomenon (60-70%) - worsens with smoking, gold, emotional stress, CCBs are the treatment of choice
- Diffuse cutaneous systemic sclerosis - skin thickening of the trunk and proximal extremities
What are the laboratory studies specific to scleroderma?
- (+) ANTI-CENTROMERE AB: associated with limited crest disease and better prognosis
- (+) ANTI-ACL-70 AB: associated with diffuse disease and multiple organ involvement (+) ANA
What is the tx for systemic sclerosis (scleroderma)?
acute management with DMARDs and steroids
-treat Raynaud’s with vasodilators (CCBs and prostacyclin)