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Secondary amyloidosis
epidemiology, causes
- Extracellular deposit of insoluble polymeric protein fibrils in tissues and organs
- Elevated amyloid A
- 2/2 chronic inflammatory conditions: Inflammatory arthritis, chronic infections, inflammatory bowel disease, malignancy, vasculitis
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Secondary amyloidosis
presentation
- Asymptomatic proteinuria or nephrotic syndrome
- Cardiomyopathy with heart failure
- Hepatomegaly
- Mixed sensory & motor peripheral neuroapthy, autonomic neuropathy
- Visible organ enlargement
- Bleeding diathesis
- Waxy thickening, easy bruising of skin
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Secondary amyloidosis
diagnosis and treatment
- Dx: abdominal fat pad aspiration biopsy
- Tx: underlying condition, and colchicine for prevention and treatment
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Reactive arthritis
- seronegative spondyloarthropathy
- resulting from enteric or GU infections
- findings: urethritis, conjunctivits, mucocutaneous lesions, enthesitis (achilles tendon pain), asymmetric oligoarthritis
- Tx: NSAIDs
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Fibromyalgia
- middle-aged women
- widespread pain, fatigue, congnitive/mood disturbances
- Tx: aerobic exercise, good sleep hygiene, tricyclic antidepressants
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Hydroxychloroquine
- tx for SLE with isolated skin and joint involvement
- AE: retinopathy
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polymyositis
- symmetrical proximal muscle weakness
- Increasing difficulty climbing stairs, getting up from a chair
- Labs: elevated muscle enzymes (CK, aldolase, LDH, AST
- Electromyography abnormal
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polymyalgia rheumatica
- age > 50
- Aching, morning stiffness
- Synovitis, bursitis, decreased range of motion
- no significant muscle tenderness
- possible systemic symptoms
- Clinical diagnosis, significantly elevated ESR
- Sx improve with corticosteroids
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Enthesitis
- inflammation and pain where tendons and ligaments attach to bone
- HLA-B27-associated arthropathies: ankylosing spondylitis (AS), psoriatic arthritis, reactive arthritis
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Ankylosing spodylitis
- seronegative spondyloarthropathies
- male:female is 2:1
- morning stiffness that lasts >30min, back typically improves with exercise
- Dx: AP x-ray of sacroiliac joints, fusion of the sacroiliac joints and/or bamboo spine
- 90% have HLA-B27
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avascular necrosis
- corticosteroid-induced
- progressive pain without restriction of motion
- normal radiograph on early stages
- Dx: MRI
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Cyclophosphamide
- immunosuppressant in SLE, vasculitis and certain cancers
- Side effects: acute hemorrhagic cystitis, bladder carcinoma, sterility, and myelosuppression
- prevent these by drinking fluids
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Pseudogout
- calcium pyrophosphate dehydrate (CPPD) crystals from sites of chondrocalcinosis (calcification of articular cartilage) into the joint space
- rhomboid-shaped, positively birefringent crystals is diagnostic
- Attacks: often occur in setting of trauma, surgery, or medical illness
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gout
- urate crystals
- firm, yellowish nodules
- needle-shaped, negatively birefringent crystals
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Gonococcal infection
triad
- polyarthralgia
- tenosynovitis
- painless vesiculopustular skin lesions
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Rheumatoid arthritis
tx
- Disease-modifying antirheumatic agents (DMARDs)
- 1. Nonbiologic agents: MTX, hydroxychloroquine, sulfasalazine, leflunomide, azathioprine
- 2. Biologics: etanercept, infliximab, adalimumab, tocilizumab, rituximab
Symptomatic relief: NSAIDs, COX-2 inhibitors
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Sarcoidosis
- African american women in the 3-4th decades of life
- incidental CXR findings: bilateral hilar adenopathy and reticular opacities
- Sx: cough, dyspnea, fever, weight loss; can also have skin, eye, joint, or other organ involvement
- Pathology: noncaseating granulomas
- Elevated angiotensin converting enzyme
- Tx: glucocorticoids (in symptomatic pts)
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Erythema nodosum
- painful, subcutaneous nodules develop on the anterior surface of the lower legs
- benign vs early sx of more serious disease
- most common cause: recent streptococcal infection
- Other causes: sarcoidosis, TB, histoplasmosis, inflammatory bowel disease
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hypercalcemia
sx
- constipation, fatigue, excessive urination
- Abdominal pain, urinary stones, mental status changes
- osteoporosis
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Dermatomyositis
- autoimmune condition
- proximal extensor muscle inlammatory myopathy
- cutaneous findings: violaceous poikiloderma, periorbital edema (heliotrope sign)
- Lichenoid papules overlying the joints (grotton's papules)
- Females: 6 times more common
- Anti-Mi-2
- Risk: internal malignancies (ovarian; breast, lung, female urogenital cancers)
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Whipple's disease
- presentation: h/o chronic malabsorptive diarrhea, protein-losing enteropathy, weight loss, migratory non-deforming arthritis, lymphadenopathy and low-grade fever
- Can also damage the eye, CNS, myocardium
- Tropheryma whippelii (gram-positive bacillus)
- Small intestine biopsy shows PAS-positive macrophages in the lamina propria containing non-acid-fast gram-positive bacilli
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