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97 Cards in this Set
- Front
- Back
What are the clinical findings associated with neonatal lupus? |
Skin lesions Cardiac abnormalities (AV block, transposition of the great vessels) Valvular and septal defects |
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What are the most common initial findings in lupus? |
Malar rash, joint pain, and fatigue |
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How is SLE diagnosed? |
ANA screening test + - sensitive but not specific Anti-ds DNA and anti-smith Ab - diagnostic if either present Others: anti-ss DNA, antihistone Ab (drug induced lupus) SSA, SSB |
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What HLA type is associated with SLE? |
HLA-DR2 HLA-DR3 |
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What HLA type is associated with Sjogren's? |
HLA-DR3 |
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What HLA type is associated with RA? |
HLA-DR4 |
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What HLA type is associated with ankylosing spondylitis, Reiter's syndrome, and psoriatic arthritis? |
HLA-B27 |
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What is the treatment for acute SLE exacerbation? |
Local or systemic corticosteroids |
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What is the best long-term therapy for SLE? |
Antimalarials - hydroxychloroquine - for cutaneous, constitutional, and articular manifestations |
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What is the treatment for active glomerulonephritis in SLE? |
Cytotoxic agents - cyclophosphamide |
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What are the most common causes of death in SLE? |
Opportunistic infections Renal failure |
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What is drug-induced lupus? |
Lupus-like syndrome produced by certain drugs that is similar to SLE but doesnt affect CNS or kidneys If CNS or kidney involvement is NOT drug induced lupus |
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What drugs are common causes of drug induced lupus? |
Hydralazine Procainamide Isoniazide Chlorpromazine Methyldopa Quinidine |
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What is the pathophysiology of scleroderma? |
Cytokines stiulate fibroblasts causing abnormal amounts of collagen deposition High quantity of collagen causes problems associated with the disease (composition of collagen is normal) |
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What are the two forms of scleroderma? |
Diffuse - widespread skin involvement with rapid onset of symptoms and early visceral involvement Limited - skin involvement limited to distal extremities and face, delayed onset of symptoms, late visceral involvement |
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What is CREST syndrome? what does it stand for? |
Variant of limited scleroderma Calcinosis of digits Raynaud's phenomenon Esophageal motility dysfunction Sclerodactyly of fingers Telangectasias |
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What lab test is very specific for limited scleroderma? |
Anti-centromere antibody |
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What lab test is very specific for diffuse scleroderma? |
Anti-topoisomerase I (antiscleroderma-70) antibody |
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How is scleroderma treated? |
Symptomatically Musculoskeletal pain - NSAIDs Esophageal reflux - PPIs Raynaud's - avoid cold and smoking, keep hands warm, use CCBs |
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What is antiphospholipid antibody syndrome? and what are the lab findings associated with it? |
A hypercoaguable state that can be idiopathic or associated with SLE + Lupus anticoagulant Anticardiolipin antibody Prolonged PTT or PT not corrected by adding normal plasma |
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What is the treatment for antiphospholipid antibody syndrome? |
Long term anticoagulation INR goal 2.5-3.5 |
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What are the typical clinical findigns of antiphospholipid antibody syndrome? |
Recurrent venous thrombosis Recurrent arterial thrombosis Recurrent fetal loss Thrombocytopenia Livedo reticularis |
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What is Sjogren's syndrome? |
An autoimmune disease most commonly seen in women. Lymphocytes infiltrate and destroy the lacrimal and salivary glands |
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What is primary versus secondary Sjogren's syndrome? |
Primary - dry eyes, mouth, but patient w/o another rhheumatologic disease Secondary - Dry eyes and dry mouth along with a connective tissue disease (RA, systemic sclerosis, SLE, polymyositis) |
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What are patients with Sjogren's syndrome at risk for? and what is the MCC of death? |
At risk for non-Hodgkin lymphoma Malignancy is most common cause of death |
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How is sjogren's diagnosed? |
ANA - 95% SSA (55%) and SSB (40%) Increased ESR, normocytic anemia, leukopenia Salivary gland biopsy is most accurate, but not needed for diagnosis |
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How is Sjogren's treated? |
Pilocarpine/cevimeline - oral secretions Artifical tears Good oral hygiene Arthralgias - NSAIDS, steroids |
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What is mixed connective tissue disease? |
Overlap syndrome with features similar to SLE, RA, systemic sclerosis, and polymyositis |
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How is Mixed connective tissue disease diagnosed? |
Clinical findings of several other rheumatologic diseases over time Anti-U1-RNP Ab - key lab finding |
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What is rheumatoid arthritis? |
Chronic inflammatory autoimmune disease involving synovium of joints. Inflamed synovium can cause damage to cartilage and bone. Is a systemic disease that can have many extra-articular manifestations |
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What joints are most often involved in RA? |
Can be any joint in body - EXCEPT DIPS Common joints - PIP, MCP, wrists, knees, ankles, elbows, hips, shoulder Joint distribution is usually symmetric |
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What are the characteristic hand deformities in RA? |
Ulnar deviation of MCP joints Boutonniere deformities of PIP - PIP flexed, DIP hyperextended Swan neck contracture - MCP flexed, PIP hyperextended, DIP flexed |
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What are the constitutional symptoms of RA? |
Morning stiffness - improves as day progresses (>1 hour suggests inflammatory) Low grade fever, weight loss Fatigue |
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What are the lab findings in RA? |
High titer RF - more severe disease Anticitrullinated peptide ab (ACPA) Elevated ESR and CRP Normocytic normochromic anemia |
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What are the diagnostic criteria for RA? |
Inflammatory arthritis of three of more joints (not DIP) Symptoms lasting at least 6 weeks Elevated CRP and ESR Positive serum RF or ACPA Radiographic changes consistent with RA (erosions and periarticular decalcification) |
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How is RA treated? |
Symptomatic - NSAIDs for pain controls; low dose steroids for short term management DMARDs - reduce morbidity and mortality; initiate early Methotrexate - best initial DMARD Lefunomide Hydroxychloroquine Sufasalasine Anti-TNF agents (etanercept, infliximab) as add-on to MTX |
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What are the side effects of methotrexate? |
GI upset, oral ulcers, mild alopecia, bone marrow suppression, hepatocellular injury, idiosyncratic interstitial pneumonitis |
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What is Felty's syndrome? |
A variant of RA Anemia, neutropenia, splenomegaly, and RA |
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What is Juvenile RA? |
RA that begins before age 18 Extra-articular manifestations may predominate (Still's disease) or arthritis may predominate |
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What will be the result of synovial fluid analysis in non-inflammatory arthritis (OA, trauma)? (color, WBCs, PMNs) |
Color - clear, yellow; possibly red if traumatic WBCs <2,000 PMNs <25% |
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What will be the results of synovial fluid analysis in inflammatory arthritis (RA, gout, pseudogout, Reiter's syndrome)? (color, WBCs, PMNs) |
Color-Cloudy yellow WBCs >5,000 PMNs 50-70% Positive birefringent crystals - pseudogout Negative birefringent crystals - gout |
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What will be the results of synovial fluid analysis in septic arthritis (bacterial, TB)? (color, WBCs, PMNs) |
Color-turbid, purulent WBCs >50,000 PMNs >70% Synovial fluid culture positive for most cases except gonococcal (only 25% positive) |
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What are the different causes of gout? |
Increased production of uric acid - Hyppoxanthine-guanine phosphoribosyltransferase deficiency (lesh-nyan), phosphoribosyl pyrophosphate overactivity, increased cell turnover (cancer chemo, chronic hemolysis, hematologic malignancy) Decreased excretion of uric acid (>90% of cases) - renal disease, NSAIDs, diuretics, acidosis |
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What are some common precipitants of an acute gouty attack? |
Decrease in temperature Dehydration Stress (emotional or physical) Excessive alcohol intake Starvation |
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What are the clinical features of an acute gouty attack? |
Peak onset 40-60 years old Initial attack usually involves 1 joint of lower extremity - sudden onset exquisite pain, most often 1st MTP |
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What is intercritical gout? |
Asymptomatic period after an initial gout attack 60% have an attack in the next year Attacks tend to become polyarticular with increased severity over time |
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What is chronic tophaceous gout? |
Chronic gout occuring in pts with poorly controlled gout for 10-20 years Tophi - aggregates of urate crystals surrounded by giant cells in an inflammatory reaction; cause deformity and destruction of surrounding tissue Common locations - extensor surface of forearms, elbows, knees, achilles tendon, pinna of external ear |
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How is an acute gout attack diagnosed? |
aspiration of synovial fluid - needle shaped negative birefringent crystals Serum uric acid NOT helpful - can be normal even during acute attack |
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What are some complications of gout? |
Nephrolithiasis - small risk Degenerative arthritis |
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What are secondary causes of hyperuricemia that should be avoided in patients with gout? |
Meds that increase uric acid (thiazides and loops) Obesity Alcohol intake Dietary purine intake - limit seafood/red meat |
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What is the treatment for acute gout? |
Indomethacin/other NSAID = treatment of choice; relieves pain promptly (Colchicine is an alternative if can't take NSAIDs) Steroids if patient does not respond or cannot tolerate NSAIDs and colchicine |
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What are the side effects of colchicine? |
nausea, vomiting, abdominal cramps, severe diarrhea Indomethacin and other NSAIDs preferred over colchicine for acute gout for this reason |
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What is prophylactic therapy for chronic gout? and when should it be started? |
Wait until pt has at least 2 acute attacks (2/year) Uricosuric drugs (probenecid, sulfinpyrazone) - 24 hour urine uric acid <800 mg/day (contraindicated if hx of renal stones) Allopurinol (xanthine oxidase inhibitor) - if 24 hour urine uric acid is >800 mg/day - NEVER GIVE FOR ACUTE GOUT, makes it worse *when starting prophylaxis, add colcichine or NSAID for 3-6 months to prevent an acute attack* |
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What type of crystals are present in pseudogout? and what do they look like? |
Calcium pryophosphate crystals Weakly positively birefringent, rod-shaped and rhomboidal crystals |
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What are risk factors for pseudogout? |
Age, OA Hemochromatosis, hyperparathyroidism, hypothyroidism, Bartter's syndrome |
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How does pseudogout present clinically? |
Typically monoarticular Common joints are knees and wrists |
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What is the treatment for pseudogout? |
Treat underlying disorder if identified Sympomatic tx similar to goat - NSAIDs, colchicine, intraarticular steroid injections |
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What makes Inclusion body myositis the "oddball" inflammatory myopathy? |
Male > Female Absence of autoantibodies Distal muscle involvement Low CK Poor prognosis |
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What antibodies are associated with dermatomyositis and polymyositis? and which has the best prognosis? |
Antisynthetase antibodies (Anti-Jo-1) - does not respond well to therapy Antisignal recognition particle - common cardiac manifestations, poorest prognosis Anti-Mi-2 antibodies - better prognosis |
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What is the treatment for dermatomyositis/polymyositis? |
Corticosteroids - initial tx, taper after symptoms improve Immunosuppressive agents - MTX, cyclophosphamide, chlorambucil Physical therapy |
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What is polymyalgia rheumatica and what is the typical clinical course? |
Rheumatic disease that usually occurs in elderly patient (70), more common in women Unknown cause but may be autoimmune (HLA-DR4 association) Course is self-limited (1-2 years) |
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What are the clinical features of polymyalgia rheumatica? |
Bilateral hip and shoulder muscle pain - begins abruptly, stiffness after periods of inactivity, pain on movement, normal strength, morning stiffness Constitutional symptoms - malaise, fever, depression, weight loss, fatigue Joint swelling - knees, wrists, hands 10% develop temporal arteritis |
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How is polymyalgia rheumatica diagnosed? |
Clinically Labs - ESR elevated |
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How is polymyalgia rheumatica treated? |
Corticosteroids |
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What is the course of fibromyalgia? |
Chronic non-progressive course with waxing and waning in severity |
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What are the clinical features of fibromyalgia, and what is key to the diagnosis? |
Stiffness, body aches, fatigue - constant aching pain, aggravated by weather, stress, sleep deprivation, cold Key to diagnosis - multiple trigger points (tender to palpation), symmetrical |
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How is fibromyalgia treated? |
Advise pt to stay active and productive Meds not effective. SSRIs and TCAs may be beneficial. Avoid narcotics CBT, exercise, psych eval |
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What is ankylosing spondylitis? |
A seronegative arthropathy More common in males Strong associated with HLA-B27 |
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What disease is characterized by "fusion" of the spine in an ascending manner (lumbar to cervical)? |
Ankylosing spondylitis |
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What is the course of ankylosing spondylitis? |
slow progression, may have acute exacerbations Normal life expectancy |
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What are the clinical features of ankylosing spondylitis? |
Low back pain and stiffness - from sacroilitis Enthesitis - inflammation of tendinous insertions Brittle spine prone to fractures Consitutional - fever, weight loss, fatigue Extra-articular Eye involvement - anterior uveitis |
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What is seen on imaging of ankylosing spondylitis? |
Bamboo spine - caused by fusion of vertebral columns |
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What are some complicatiosn of ankylosing spondylitis? |
Restrictive lung disease Cauda equina syndrome Spine fracture with spinal cord injury Osteoporosis Spondylodiscitis |
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How is ankylosing spondylitis treated? |
Symptomatic relief - NSAIDs Anti-TNF meds (etanercept, infliximab) PT Surgery - if severe spinal deformity |
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What is reactive arthritis? |
Asymmetric inflammatory oligoarthritis of lower extremities (upper less common) Preceded by infectious process that is remote from site of arthritis (1-4 weeks prior), usually after enteric or urogenital infections Seen in HLA-B27 + individuals |
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What is Reiter's syndrome? and what is the classic triad? |
One form of reactive arthritis Triad - arthritis, urethritis, ocular inflammation (conjunctivitis or anterior uveitis) "Can't see, can't pee, can't climb a tree" |
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Infection with what organisms have been associated with reactive arthritis? |
Salmonella Shigella flexneri Campylobacter jejuni Yersinia enterocolitica |
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What are the clinical features of reactive arthritis? |
Evidence of GI or GU infection 1-4 weeks prior Asymmetric arthritis Fatigue, malaise, weight loss, fever |
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What is the treatment for reactive arthritis? |
1st line = NSAIDs If no response to NSAIDs, sulfasalazine and immunosuppresive agents like azathioprine |
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What are the clinical features of temporal arteritis? |
Constitutional - fatigue weight loss, fever Headaches Visual impairment (25-50%) Jaw pain with chewing Tenderness over temporal artery |
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How is temporal arteritis diagnosed? |
Elevated ESR Temporal artery biopsy - negative biopsy doesnt rule out |
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What is the treatment for temporal arteritis? |
High dose steroids - give early to prevent blindness. Do NOT wait for biopsy resuts |
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What is Takayasu arteritis? |
Vasculitis of aortic arch and its major branches seen most commonly in young asian women |
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What are the clinical features of Takayasu arteritis? |
Decreased/absent peripheral pulses Discrepancies in BP (arm vs leg) Arterial bruits |
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What are cocmplications of Takayasu arteritis? |
Limb ischemia, aortic aneurysm, aortic regurg, stroke, secondary HTN due to renal artery stenosis |
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How is Takayasu arteritis treated? |
Steroids for symptom relief Treat HTN MAy need surgery/angioplasty for stenosed vessels |
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What are the clinical features of Churg Strauss syndrome? |
constitutiona findigns, asthma, dyspnea, and skin lesions (subcutaneous nodules, palpable purpura) |
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How is churg strauss diagnosed? |
By biopsy of lung or skin Also associated with p-ANCA |
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What are the clinical features of Wegener's? |
Upper respiratory smptoms (sinusitis) Oral ulcers Pulmonary - cough, hempotsysis, dyspnea Renal - glomerulonephritis Eyes disease - conjunctivitis, scleritis Constitutional |
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How is Wegener's diagnosed? |
Nodules/infiltrates on CXR Elevated ESR, anemia, hematuria, C-ANCA open lung biopsy confirms |
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How is Polyarteritis nodosa distinguished from Wegener's? |
No pulmonary involvement |
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What is polyarteritis nodosa? |
Medium vessel vascultiis involving nervous system and GI tract |
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What is polyarteritis nodosa associated with? |
Hep B, HIV, drug reactions |
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What are the clinical features of PAN? |
Fever, weakness, weight loss, myalgia, arthralgia, abdominal pain (bowel angina) HTN Mononeuritic multiplex Livedo reticularis |
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What are the clinical features of Behcet's syndrome? |
Recurrent oral and genital ulcers - painful Arthritis Uveitis, optic neuritis, iritis CNS - meningoencephalitis, intracranial HTN Fever, weight loss |
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What is Buerger's Disease? |
AKA thromboangiitis obliterans Small and medium vessel vasculitis affecting arms and legs most common seen in young men who smoke |
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What are clinical features of Buerger's disease? |
Ischemic claudication Cold, cyanotic painful distal extemities Ulceration of digits |