tonystep1's version from 2017-07-27 08:58


Question Answer
Features common to both polymyositis and dermatomyositisSymmetrical proximal muscle weakness that develops over weeks or several months, myalgia, dysphagia
DermatomyositisHeliotrope rash, Gottron's papules (papular, erythematous, scaly lesions over the knuckles), V sign, Shawl sign, periungual erythema with telangiectases, subcutaneous calcifications in children.
Inclusion Body MyositisThere is early weakness and atrophy of quadriceps, forearm, flexors, and tibialis anterior muscles. Involvement is asymmetrical. Facial weakness occurs in one-third of patients, and dysphagia in one-half of patients.
Polymyalgia RheumaticaHip and shoulder muscle pain (bilateral) , abrupt onset, stiffness in shoulder and hip regions after a period of inactivity is the most prominent symptom. Profound morning stiffness is common. Constitutional symptoms are usually present. Signs and symptoms of temporal arteritis (if present)
FibromyalgiaStiffness, body aches (musculoskeletal), fatigue Sleep patterns are disrupted, and sleep is unrefreshing. Anxiety and depression are common

Myopathies and pain syndromes diagnostic lab studies

Question Answer
PolymyositisCK level is significantly elevated. LDH, aldolase, AST, ALT elevated ANA in over 50% Anti-synthetase antibodies (anti-Jo-1 antibodies Anti-signal recognition particle Anti-Mi-2 antibodies
Inclusion Body Myositisslight elevation of CK levels (relatively low)
Polymyalgia RheumaticaESR is usually elevated and aids in diagnosis

Myopathies and pain syndromes diagnostic criteria

Question Answer
PolymyositisSymmetric proximal muscle weakness//Elevation in serum creatine phosphokinase//EMG findings of a myopathy//Biopsy evidence of myositis//Characteristic rash of dermatomyositis
FibromyalgiaWidespread pain including axial pain for at least 3 months//Pain in at least 11 of 18 possible tender point sites

Treatment and Management of Myopathies/pain syndromes

Question Answer
PolymyositisCorticosteroids are the initial treatment. Continue until symptoms improve, and then taper very slowly (up to 2 years may be necessary) and Physical therapy
Refractory PolymyositisImmunosuppressive agents (for patients who do not respond to steroids)methotrexate, cyclophosphamide, chlorambucil
Polymyalgia RheumaticaResponse usually occurs within l to 7 days. Corticosteroids are not curative, but are effective in suppressing inflammation until the disease resolves itself.//After 4 to 6 weeks, begin to taper slowly.//Most patients (60% to 70%) can stop corticosteroids within 2 years. A few patients have symptoms for up to lO years.
FibromyalgiaAdvise the patient to stay active and productive.//Medications are generally not effective. SSRis and TCAs have shown some effect and may be beneficial. Avoid narcotics.//Cognitive-behavioral therapy, exercise, consider psychiatric evaluation


Question Answer
Ankylosing SpondylitisLow back pain and stiffness (secondary to sacroiliitis)-//Neck pain and limited motion in cervical spine//Enthesitis-inflammation at tendinous insertions into bone//spine becomes brittle and is prone to fractures with minimal trauma.//Chest pain and diminished chest expansion//Shoulder and hip pain//Constitutional symptoms//Extra-articular manifestations//Loss of normal posture as disease advances
Reactive ArthritisLook for evidence of infection (GI or genitourinary) l to 4 weeks before the onset of symptoms. \\ Asymmetric arthritis-New joints may be involved sequentially over days. Joints are painful, with effusions and lack of mobility.//Fatigue, malaise, weight loss, and fever are common//Joint pain may persist or recur over a long-term period
Reiter's SyndromeClassic triad of arthritis, urethritis, and ocular inflammation (conjunctivitis or anterior uveitis): "Can't see" (uveitis), "can't pee" (urethritis), "can't climb a tree" (arthritis).
Psioriatic ArthritisIt is typically gradual in onset. Patients usually have skin disease for months to years before the arthritis develops//Usually asymmetric and polyarticular//Upper extremities most often involved; smaller joints more common than large joints//

Diagnosis of Seronegative Spondyloarthropathies

Question Answer
Ankylosing SpondylitisImaging studies of lumbar spine and pelvis (plain film, MRI, or CT) reveal sacroiliitis-sclerotic changes in the sacroiliac area. Eventually, the vertebral columns fuse, producing "bamboo spine."Elevated ESR in up to 75% of patients (due to inflammation)-nonspecific HLA-B27 is not necessary for diagnosis.
Reactive ArthritisSend synovial fluid for analysis (to rule out infection or crystals).,organisms usually associated with reactive arthritis include Salmonella, Shigella, Campylobacter; Chlamydia, Yersinia; diagnosis is clinical
Psioriatic ArthritisClinical diagnosis following which is typically gradual in onset. Patients usually have skin disease for months to years before the arthritis develops.
Treatment of Seronegative SpondyloarthropathiesNSAIDs is first line, surgery maybe indicated for Patients with ankylosing spondylitis with severe spinal deformity.

VASCULITIS Clinical Features

Question Answer
Temporal ArteritisConstitutional symptoms Headaches Visual impairment (in only 25% to 50%) Jaw pain with chewing-intermittent claudication of jaw/tongue when chewing Tenderness over temporal artery; absent temporal pulse Palpable nodules 40% of patients also have polymyalgia rheumatica
Takayasu's ArteritisConstitutional symptoms Pain and tenderness over involved vessels Absent pulses in carotid, radial, or ulnar arteries; aortic regurgitation may be present Signs and symptoms of ischemia Severe complications include limb ischemia, aortic aneurysms, aortic regurgitation,stroke, and secondary HTN due to renal artery stenosis.
Wegener's GranulomatosisConstitutional symptoms Upper respiratory symptoms (e.g., sinusitis); purulent or bloody nasal discharge Oral ulcers (may be painful) Pulmonary symptoms (cough, hemoptysis, dyspnea) Renal involvement (glomerulonephritis-may have rapidly progressive renal failure) Eye disease (conjunctivitis, scleritis) Musculoskeletal (arthralgias, myalgias) Tracheal stenosis
Churg·Strauss SyndromeConstitutional symptoms (fever, fatigue, weight loss); prominent respiratory tract findings (asthma, dyspnea) ; and skin lesions (subcutaneous nodules, palpable purpura)
Polyarteritis Nodosa (PAN) Constitutional symptoms (fever, fatigue, weight loss) abdominal pain (bowel angina) Other findings are HTN, mononeuritic multiplex, and livedo reticularis
Behcet's Syndromerecurrent oral and genital ulcerations (usually painful), arthritis (knees and ankles most common), eye involvement (uveitis, optic neuritis, iritis, conjunctivitis), CNS involvement (meningoencephalitis, intracranial HTN) , fever, and weight loss
Buerger's Disease (Thromboangiitis Obliterans)ischemic claudication; cold, cyanotic. painful distal extremities; paresthesias of distal extremities; and ulceration of digits.
Hypersensitivity VasculitisConstitutional symptoms (fever, weight loss, fatigue) Small-vessel vasculitis that is a hypersensitivity reaction in response to a drug (penicillin, sulfa drugs) , infection, or other stimulus Skin is predominantly involved-palpable purpura, macules, or vesicles (common on lower extremities) can occur. Lesions can be painful.

Vasculitis associated risk factors and diagnostic studies

Question Answer
Temporal Arteritis risk factorspatient is >50 years of age; twice as common in women as men Associated with increased risk of aortic aneurysm and aortic dissection
Temporal Arteritis diagnostic testsESR elevated (but normal ESR does not exclude the diagnosis) .Biopsy of the temporal artery has a sensitivity o f 90%. A single negative biopsy does not exclude the diagnosis.
Takayasu's Arteritis risk factorsMost common in young Asian women
Takayasu's Arteritis diagnostic testsDiagnosed via arteriogram
Churg·Strauss Syndrome diagnostic testsDiagnosis is made by biopsy of lung or skin tissue (prominence of eosinophils) . It is associated with p-ANCA.
Wegener's Granulomatosis diagnostic testsChest radiograph is abnormal (nodules or infiltrates). Laboratory findings: markedly elevated ESR; anemia (normochromic normocytic); hematuria; positive c-ANCA in 90% of patients-sensitive and specific; thrombocytopenia may be present Open lung biopsy confirms diagnosis
Polyarteritis Nodosa (PAN) associated risk factorsCan be associated with hepatitis B, HIV, and drug reactions
Polyarteritis Nodosa (PAN) diagnostic testsDiagnosis is made by biopsy of involved tissue or mesenteric angiography ESR is usually elevated, and p-ANCA may be present. Test for fecal occult blood.
Buerger's Disease (Thromboangiitis Obliterans) associated risk factorsOccurs mostly in young men who smoke cigarettes , May lead to gangrene

Treatment and Management of Vasculitis

Question Answer
Temporal ArteritisIf temporal arteritis is suspected, begin prednisone and order a temporal artery biopsy. Follow up on ESR levels to monitor effectiveness of treatment. If visual loss is present, admit the patient to the hospital for IV steroids; otherwise, start oral prednisone. maintain steroid therapy for up to 2 to 3 years
Takayasu's ArteritisSteroids may relieve the symptoms. Treat HTN. Surgery or angioplasty may be required to recannulate stenosed vessels. Bypass grafting is sometimes necessary.
Churg·Strauss SyndromeThe prognosis is poor, with a 5-year survival of 25% (death is usually due to cardiac or pulmonary complications). With treatment (steroids) , the 5-year prognosis improves to 50%
Wegener's GranulomatosisThe prognosis is poor-most patients die within 1 year after the diagnosis. A combination of cyclophosphamide and corticosteroids can induce remissions in many patients, but a relapse may occur at any time. Consider renal transplantation if the patient has end-stage renal disease (ESRD).
Polyarteritis Nodosa (PAN)The prognosis is poor, but is improved to a limited extent with treatment. Start with corticosteroids. If PAN is severe, add cyclophosphamide.
Bechet's SyndromeTreatment is steroids, which are helpful
Buerger's Disease (Thromboangiitis Obliterans)Smoking cessation is mandatory to reduce progression
Hypersensitivity VasculitisWithdrawal of the offending agent and steroids are the treatments of choice.