Rheumatology pearls

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Gout

Diagnostic Clues:  sudden onset of excruciating joint pain, most commonly affects first MTP joint of big toe (but may affect other joints depending on which are used most); affected joint is typically swollen, red, and tender may not be able to tolerate a bed sheet on the joint; in long standing (& undertreated) gout, may see tophi; MC in men 40-60 y/o

Aspiration of joint necessary on initial presentation to be sure of diagnosis

Gout:  needle-shaped crystals with negative birefringence 

Pseudogout:  rhomboid-shaped calcium crystals with positive birefringence; MC found in knees and wrists 

Rule out septic arthritis

Serum uric acid is not helpful in diagnosis

Causes:  overproduction vs. underexcretion of uric acid

Overproduction:  idiopathic, excessive dietary purine intake, alcohol, myeloproliferative and lymphoproliferative disorders

Underexcretion:  diuretics, idiopathic, dehydration, renal insufficiency

Precipitants of acute attack: dehydration, stress, EtOH, starving fasting OR feasting

Treatment:  

Acute:  high dose NSAIDs or steroids

Maintenance:  Allopurinol for overproduction, probenecid for undersecretion; avoid triggers (esp. dietary)

Avoid Aspirin and acetaminophen

Rheumatoid Arthritis

Chronic inflammatory disease involving the synovium of the joints; inflamed synovium can cause damage to nearby bones and cartilage

Age 20-40; MC in women (3:1)

S/Sx: Symmetrical inflammatory polyarthritis in almost every joint except DIP joints

MCC joints: PIP/MCP of hands, wrists, knees, ankles, hips, shoulders

Hand deformities: ulnar deviation of MCP; Boutonniere deformities; Swan-neck contractures

Morning stiffness that improves with activity

Possible C1-C2 spine involvement

Subcutaneous rheumatoid nodules over extensor surfaces.

Other: Cardiac involvement/effusions, Pulmonary effusions (typically fluid has very low glucose), soft tissue swelling

Dx: High titers of Rheumatoid Factor (RF), Narrowing of joint space, loss of juxta-articular bone mass, bony erosions, NO osteophytes

Tx: Prevent further destruction; exercise with resistance training; NSAIDs to control pain in symptomatic patient; Corticosteroids if NSAIDs fail – long term, low dose may alter the course of the disease

Disease Modifiers: reduce M&M by limiting complications, slowing disease, and preserving joint function

Methotrexate – first line therapy

Hydroxychloroquine – not as effective as MTX; may cause visual loss

Sjogren’s Syndrome

AI disease; lymphocytes infiltrate and destroy lacrimal and salivary glands

S/Sx: dry eyes (burning, itching, redness), dry mouth, arthritis, fatigue, arthralgias

Dx: ANAs present in 95% of patients

Tx: Pilocarpine, artificial tears

Scleroderma

Chronic connective tissue disorder that leads to widespread fibrosis; the high quality of the collagen causes the problems associated with the disease (composition of the collagen is normal)

More common in women, age 30-50s.

Raynaud’s Phenomenon

Present in all Scleroderma patients

Caused by vasospasm and thickening of vessel walls in the digits

Cold temps and stress cause color changes of fingers: blanching, cyanosis, then redness

Cutaneous fibrosis: tightening of skin in extremities (ex. sclerodactyly); the degree of skin involvement predicts prognosis – the more skin involved, the worse the prognosis

GI: dysphagia/reflux from esophageal immobility, delayed gastric emptying, pain, constipation/diarrhea

Pulmonary: MCC of death in scleroderma; interstitial fibrosis

Cardio: pericardial effusions

Diagnosis: Anticentromere antibody is very specific

Tx: no effective cure

Systemic Lupus Erythematosus (SLE)

More common in women, AA, childbearing age

Most common symptoms: fatigue, malar/butterfly rash, and joint pain

Diagnostic Clues:  must have at least four of the following:

Malar rash

Discoid rash

Photosensitivity

Oral/nasopharyngeal ulcers

Non-erosive arthritis – musculoskeletal pain often first symptom

Serositis (pleuritis, pleural effusion, pericarditis)

Proteinuria  may have nephritic syndrome

Seizures or psychosis

Hemolytic anemia

Positive ANA

Positive dsDNA Ab, anti-Smith Ab, anti-phospholipid Ab

Diagnostic Tools: Positive ANA (sensitive but not specific); Anti-dsDNA or Anti-Smith Ab – either is diagnostic of SLE; Anti-SS DNA; Antihistone Ab are present in 100% of drug-induced lupus

Diagnostic algorithm: Symptoms  Order ANA  if positive order additional tests (see above)

Causes:  HLA-linked, can also be drug induced (procainamide, hydralazine, and others)

Treatment:  Stop offending medication (if drug-induced), NSAIDS for joint symptoms, steroids for acute exacerbations, other treatments as needed; Life expectancy is decreased and MCC most common cause of deaths are opportunistic infection and renal failure

Ankylosing Spondylitis

Bilateral sacroilitis; MC in men (3:1); Onset in young adulthood

Strong association with HLA-B27

S/Sx: Low back pain and stiffness, neck pain and stiffness  both improve over the course of the day and a hot shower, worsen with rest or inactivity

Dx: Imaging studies of the lumbar spine and pelvis  bamboo spine (vertebral columns eventually fuse)

Tx: NSAIDs (indomethacin) for symptomatic relief

Temporal Arteritis

“Giant Cell Arteritis”

S/Sx: Women, >50 y/o presents with a new headache, change in vision, tenderness over the temporal artery, high ESR, and jaw claudication (sometimes “tooth pain”…may be referred by dentist)

Visual impairment (25-50% of patients) caused by ophthalmic artery involvement; may lead to blindness if not treated early and aggressively

Dx: Biopsy the temporal artery

Tx: High dose steroids IMMEDIATELY.  Do NOT wait for biopsy results.  If visual loss is present, admit patient to hospital and begin IV steroids immediately.

Takayasu’s Arteritis

Vasculitis of aorta and its branches; diagnosed via arteriogram

S/Sx: Patient is usually Asian woman, <40 y/o with constitutional symptoms, decreased/absent peripheral pulses, discrepancies of blood pressure in various sites of the body, absent pulses in carotid, radial, or ulnar arteries.

Tx: Steroids may relieve symptoms; Surgery needed to recannulate stenosed vessels.

Churg-Strauss Syndrome

Vasculitis that may involve many organ systems

Dx: biopsy of skin or lung

p-ANCA positive

Wegener’s Granulomatosis

Vasculitis predominately involving the kidneys, lungs, and upper/lower GI tract

S/Sx:  Upper respiratory symptoms (sinusitis, cough, hemoptysis, dyspnea), glomerulonephritis

Oral ulcers

Eye disease – conjunctivitis, scleritis

Muscle/joint pain

Dx: CXR shows nodules or infiltrates; markedly elevated ESR, c-ANCA positive; open lung biopsy confirms diagnosis

Poor prognosis – most patients die within one year

Polyarteritis Nodosa

Vasculitis of medium sized vessels esp in the nervous system and GI tract

Associated with Hep B, HIV, and drug reactions

Invasion of all layers and fibrinoid necrosis plus resulting intimal proliferation lead to reduced luminal area

S/Sx: fever, weakness, weight loss, myalgias/arthralgias, abdominal pain, HTN

Dx: must biopsy involved tissue, ESR usually elevated

Poor prognosis but is limited with corticosteroid treatment; if severe, add cyclophosphamide

PAN vs. Wegener;s: PAN has NO pulmonary involvement