Last Rheum Sections Flashcards
What are the features that are characteristic of spondyloarthropathies?
It involves sacroiliac joints (uncommon in rheumatoid arthritis)
Peripheral arthritis that is usually asymmetric and oligoarticular
Absence of rheumatoid factor
Associated with HLA-B27 in more than 90% of the cases
It’s an enthesopathic (bone attachment) disorders
What are the four diseases associated with an increased frequency of HLA-B27 and the frequency in each disease?
Ankylosing spondylitis (HLA-B27 90%) Reactive arthritis (HLA-B27 80%) Enteropathic spondylitis (HLA-B27 75%) Psoriatic spondylitis (HLA-B27 50%)
What are the joints affected in ankylosing spondylitis?
Affects the sacroiliac joints, the spring and the peripheral joints
What are the Sx of ankylosing spondylitis?
It is a chronic systemic inflammatory disease. Low back pain and decreased spinal motion and reduced chest expansion. Insidious onset, duration of more than 3 months, morning stiffness, improvement with exercise, family history, involvement of other systems.
What is the age of onset of ankylosing spondylitis?
15-40years old
What are the radiographic findings of ankylosing spondylitis?
sacroiliac involvement with erosion, “pseudo widening” of joint space, sclerosis (both side of sacroiliac joint) and fusion. Spine involvement with squaring of superior and inferior margins of vertebral body, syndesmophytes and bamboo spring.
What are the lab findings of ankylosing spondylitis?
Increased erythrocyte sedimentation rate. An anemia of chronic disease and rheumatoid factor is absent
What are the extraspinal involvement seen in ankylosing spondylitis?
Enthesopathic involvement is characteristic of ankylosing spondylitis and the other spondyloarthropathies and consists of plantar fasciitis, Achilles tendinitis and costcochondritis
Hip and shoulder involvement are common (50%)
What are the extraskeletal involvement seen in ankylosing spondylitis?
Fatigue, weight loss, osteoporosis, low grade fever and iritis (not in rheumatoid arthritis)
Late complication can include traumatic spinal fracture leading to cord compression, cauda equine syndrome, fibrotic changes in upper lung fields and aortic insufficiency
What are the Tx of ankylosing spondylitis?
Physical therapy (upright position), exercise (swimming), cessation of smoking, genetic counseling and drug therapy with NSAIDs (indomethacine) and TNF α inhibitor
What are the organisms of Reactive arthritis?
Salmonella, shigella, Yersinia, campylobacter, chlamydia and Ureaplasma
What are the joints involved with Reactive arthritis?
Toes (sausage toes), asymmetric large joints in the lower extremities and distal interphalangeal joints in the hands
What are the extra-articular conditions of Reactive arthritis?
Cardiac conduction disturbances and aortitis can develop, Sacroiliitis can occur.
What are the Tx of Reactive arthritis?
NSAIDs (indomethacin).
Sulfasalazine and methotrexate are used in patients with chronic disease.
Tetracycline or erythromycin ab to decrease the duration and severity of illness caused by chalamydia triggered reactive arthritis
What are the joints involved with psoriatic arthritis?
finger and toes
What are the Sx of psoriatic arthritis?
Pitting of nails. Patients with more severe skin disase are at higher risk. “Sausage” finger or toe is characteristic of psoriatic arthritis. It develops in patients with psoriasis (7% or less)
What are the radiological findings of psoriatic arthritis?
Distal interphalangeal joint with erosions. It can also cause “pencil-in-cup” deformity of the distal interphalangeal and proximal interphalangeal joints.
What are the criteria for the diagnosis of SLE?
Malar rash
Discoid lupus
Photosensitivity
Oral ulcers
Nonerosive arthritis
Proteinuria ( protein > 0.5g/day) or cellular casts
Seizures or psychosis
Pleuritis or pericarditis
Hemolytic anemia, leukopenia, lumphopenia or thrombocytopenia
Antibody to native DNA, antibody Smith, IgG or M antiphospholipid ab, positive test for lupus anticoagulant or false-positive result of VDRL test
Positive results of fluorescent antinuclear ab test.
What are the most frequently affected gender, race, and age at onset of SLE?
Gender: Female during reproductive year
Race: American blacks, Native Americans and Asians.
Age at onset of SLE: second and fourth decades of life
What are the human histocompatibility complexes seen with increased frequency seen in SLE?
HLA-B8
HLA-DR2
HLA-DR3
What are the clinical manifestations of SLE?
Fever
What are the articular manifestations of SLE?
It’s inflammatory but nondeforming and nonerosive. Avascular necrosis of one occurs and not only in patients taking steroids. The femoral head, navicular one and tibial plateau are most commonly affected.
What are the cardiopulmonary manifestations of SLE?
Pericarditis, myocarditis, valvular involvement, accelerated coronary atherosclerosis and coronary vasculitis. There is an association between SLE and coronary artery disease. Risk factor for hypertension and hyperlipidemia.
Pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, hemorrhage and diaphragmatic dysfunction.
What are the neuropsychiatric manifestations of SLE?
Impaired cognitive function, seizures, long tract signs, cranial neuropathies, psychosis and migraine like attack happens in CNS lupus. Immune complexes in the choroid plexus happen in both CNS and non-CNS lupus. Increased CSF protein IgG, pleocytosis and antineuronal antibodies.
What are the findings on lumbar puncture and MRI of neuropsychiatric SLE?
Lumbar puncture: Normal results of CSF
MRI: Shows areas of increased signal in the periventricular white matter, similar to those found in MS. It is usually nonspecific and sometimes can be seen in patients who have SLE w/o CNS manifestation
Pregnant women with SLE have higher chance of what?
Spontaneous abortion
What are the lab findings and clinical manifestations in infants of SLE mothers?
Lab findings: Anti-SS-A (Ro) + but there is no HLA association in the child.
Clinical manifestations in infants: Thromboyctopenia and leukopenia can develop. They also can have transient cutaneous lesions and complete heart block.
What are the lab findings of SLE including anemias, ITP, ESR, and hypocomplementemia?
Anemia of chronic disease and hemolytic anemia (coombs positive) can occur. Anti-lymphocyte ab causes lymphopenia in SLE. Idiopathic thrombocytopenic purpura (ITP) with the presence of platelet Ab is initial manifestation of SLE.
Polyclonal gammopathy due to hyperactivity of the humoral immune system is common
The erythrocyte sedimentation rate (ESR) usually correlates with disease activity.
Hypocomplementemia (CH50, C3,C4) usually correlates with active disease and hypocomplementemia with increased anti-native DNA abs usually implies renal disease or skin disease.
A positive result of an antinuclear antibody test (ANA) is by no means specific for lupus.
Why can patients with SLE may have false positive results of VDRL testing?
It will have false positive results of the VDRL test as a result of ab to phospholipid, which cross-reacts with VDRL
What are the two drugs that are strongly implicated in drug-induced lupus?
Procainamide
Hydralazine
What are the clinical features of drug-induced lupus.?
Arthralgia and polyarthritis. Malaise is common and 40% of the patients have fever. Cardiopulmonary involvement is common and 30% of patients have pleural-pulmonary manifestations as their presenting symptoms. Pericarditis-20% of pts. Diffuse interstitial pneumonitis. No involvement of CNS or renal.
What are the lab findings of drug-induced lupus.?
Have antinuclear Abs. Abs to native DNA, Anti smith, SS-A, SS-B and RNP are not really found in drug induced lupus but found in SLE. Serum totally hemolytic complement C3 & C4 are usually normal in drug induced lupus in contrast to SLE. The frequency of antihistone Abs is high in drug induced. Positive LE preparation, positive Coombs test, positive rheumatoid factor, false positive result of serologic test for syphilis, circulating anticoagulants and croglobulins are less common abnormal laboratory results
What are the Tx of drug-induced lupus.?
Stop using the drug. One can also take NSAID or possibly low dose prednisone if need.
What are the Ig’s involved with antiphospholipid syndrome?
IgG or IgM
What are the lab findings for antiphospholipid syndrome?
Prolongation of all phospholipid dependent coagulation tests in laboratory is hallmark of the syndrome
Activated partial thromboplastin time is prolonged and not corrected by adding normal plasma but it is corrected with the addition of platelet rich plasma, this is the laboratory hallmark of a lupus anticoagulant
What are the clinical features seen in antiphospholipid syndrome?
There is an association between the presence of the lupus anticoagulant & antiphospholipid Ab and recurrent venous or arterial thrombosis. (they all provoke blood clots)
Stroke, MI, transient ischemic attack, brachial artery thrombosis, deep venous thrombophlebitis, retinal vein thrombosis, hepatic vein thrombosis resulting in Budd-Bhiari syndrome & pulmonary hypertension.
Recurrent fetal loss, thrombocytopenia, positive Coombs test, migraines, chorea, epilepsy, chronic leg ulcers, livedo reticularis and progressive dementia from cerebrovascular accidental. Acquired valvular heart disease, especially aortic insufficiency has been described.
What is the treatment of antiphospholipid syndrome including the INR goal and duration?
Warfain is prescribed in doses sufficient to yield international normalized ratio (INR) values close to 3 and will need to be taken for life.
Low-dose aspirin and subcutaneous heparin have been used in pregnant women to prevent fetal loss
How do you treat antiphospholipid syndrome in pregnancy?
Low-dose aspirin and subcutaneous heparin have been used in pregnant women to prevent fetal loss
What are the symptoms of Raynauds?
pallor, cyanosis, erythema accompanied by pain and numbness in the hands or feet.
What is a common precipitating factor for Raynauds?
Cold
What is the treatment of Raynauds?
Smoking cessation
Wearing gloves
Biofeedback
2% nitrol paste
Antihypertensive agents ( Prazosin or Ca+ ch blockers)
Stellate ganglion block, digital nerve block or surgical digital sympathectomy if ischemia is severe.
What is the gender, age at onset, symptoms, precipitating factors of primary Raynaud disease?
Females are usually affected Onset is at menarche Usually all digits are involved Attacks are very frequent Severity of symptoms is mild to moderate They can be precipitated by emotional stress Digital ulceration and finger edema are rare, as is periungual erythema Livedo reticularis is frequent
What is the gender, age at onset, symptoms, precipitating factors of secondary Raynaud disease?
Both male and females are affected
Mid 20s of later
Beings in a single digits and attacks are usually infrequent (0-5 a day)
It is moderate to severe
Not precipitated by emotional stress
Digital ulceration, finger edema and periungual erythema are frequent
Livedo reticularis is un common
It is caused by chemotherapeutic agents, toxins, vibration induced injuries, vascular occlusive disorders, CT disease or increased blood viscosity, cold agglutinin and cryglobulinemia.
What is criteria needed for the diagnosis of Scleroderma?
One major criterion or two or more minor criteria needs to be present.
Major criterion: symmetric induration of the skin of the fingers and the skin proximal to metatarsophalangeal or metatarsophalangeal joints
Minor criterion: sclerodactyly, digital pitting scars or loss of substance from the finger pad and bibasilar pulmonary fibrosis.
What are the clinical manifestations affecting the skin in scleroderma?
Risk for development of rapidly progressive acral and trunk skin thickening and early visceral abnormalities. Raynaud phenomenon occurs in almost all pts.
What are the clinical manifestations affecting the joints in scleroderma?
Non deforming symmetric polyarthritis similar to rheumatoid arthritis. Pts can have both articular erosions and nonarticular bony resorptive changes in ribs, mandible, radius, ulna and distal phalangeal tufts which are unique to systemic sclerosis.
What are the clinical manifestations affecting the tendons in scleroderma?
Up to 60% of pts have “leathery” crepitation of the tendons of the wrist
What are the clinical manifestations affecting the cardiopulmonary systems in scleroderma?
Considerable decrease in diffusing capacity present with a normal radiography. Diffuse interstitial fibrosis occurs in 70% of pts and is the most common pulmonary abnormality. Pleuritis is very rare.
Cardiac abnormalities in 70% of pts. Pulmonary hypertension with cor pulmonale is a serious potential problem. But pulmonary hypertension by itself is more common in pts with CREST variant.
What is esophageal involvement and Tx in scleroderma?
Esophageal dysfunction is the most frequent GI abnormalities and is asymptomatic. Lower esophageal sphincter incompetence with acid reflux may produce esophageal strictures or ulcers.
Tx: Medications to reduce acid production. Reduced motility: Metoclopramide, cisapride or erthromycine
What is small bowel hypomotility and Tx in scleroderma?
Small bowel hypomotility may be associated with pseudo-obstruction, bowel dilatation, bacterial overgrowth and malabsorption
Tx: Tertracycline. Promotility agents are less effective
What is renal involvement and Tx in scleroderma?
Renal involvement may result in fulminant hypertension, renal failure and death if not treated aggressively.
Tx: aggressive early antihypertensive therapy
Renal involvement with hyperreninemia treatment is angiotensin-converting enzyme inhibitor
What is the lab finding seen in Scleroderma and treatment?
Antinuclear antibodies positive
Antitopoisomerase I Ab (Anti-Scl-70) found in 25% pts
Anticentromere Abs 10-20% pts
Tx: No remissive or curative therapy is available. Aggressive nutritional support and hyperalimentation for GI disease associated with systemic sclerosis.
What are the characteristic findings of CREST?
Calcinosis cutis Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telangiectasias
Lung involvement is in what % of CREST pts?
70%
When is the onset of Raynauds in CREST?
< 2 yrs
What is the lab finding most commonly associated with CREST?
Anticentromere Ab present, Antiscleroderma-70 ab in 10% pts and increased incidence of piary biliary cirrhosis
What is a aggravating factor for eosinophilic fasciitis?
Polyvinyl chloride disease, organic solvents jackhammer disease, silicosis and toxic oil syndrome
What are the Sx for eosinophilic fasciitis?
Tight bound-down skin of the extremities, characteristically sparing the hands and feet. Peau d’orange skin changes can develop. Onset after vigorous exercise is common. Raynaud phenomenon does not occur and there is not visceral involvement. Flexion contractures and carpal tunnel syndrome can develop.
What are the lab findings for eosinophilic fasciitis?
Peripheral eosinophilia, increased sedimentation rate and hypergammaglobulinemia
What are the Dx for eosinophilic fasciitis?
Findings of inflammation and thickening o the fascia on deep fascial biopsy
What are the Tx for eosinophilic fasciitis?
Prednisone (40mg/daily)
What are the clinical manifestations and lab findings of polymyositis?
Inflamed myopathy characterized by proximal muscle weakness.
What shows on the electromyogram seen in inflammatory myopathies?
It is characteristic but not diagnostic.
It shows decrease amplitude and increased spike frequency it is polyphasic and conduction speed is normal.
What shows on the muscle biopsy findings seen in inflammatory myopathies?
Degeneration, necrosis and regeneration of myofibrils with lymphocytic and monocytic infiltrate in perivascular or interstitial distribution
What is the treatment of polymyositis including dosage?
Prednisone (60mg daily) for 1-2months then dosage is reduced.
In severe or steroid-resistant cases: azathioprine (1-2mg/kg daily) or methotrexate
What are the presentation and lab findings seen in drug-induced myopathy?
Associated with colchicine mimics polymyositis. Muscle weakness and increased creatine kinase level.
What are the drugs implicated in drug-induced myopathy?
Colchicine Statins Zidovudine D-penicillamine Hydroxychloroquine Heroin or cocaine Corticosteroids
What are the causes of nongonococcal bacterial arthritis and the patients at risk?
It is caused by hematogenous spread of bacteria, direct inoculation (trauma), or extension of soft tissue infection with osteomyelitis into the joint space.
Elderly or immunosuppressed pts.
Pts with cancer, diabetes mellitus, chronic renal failure, liver disease, or sick cell anemia
Pts with chronic inflammatory and degenerative arthritis
What is the presentation of septic arthritis in a patient with arthritis?
Single joint flare that is out of proportion to the rest of their joint symptoms. There is possibility of infectious endocarditis, other septic joints or a disk space infection
What is the most common pathogen involved in adults?
S. aureus
What is the most common pathogen involved in sickle cells anemia?
Salmonella
What is the most common pathogen involved in cat/dog bites?
Pseudomonas
What is the most common pathogen involved in human bites?
Anaerobic infection
What are the most common pathogens involved in IV drug users and the atypical joints that are involved?
IV drug users: Pseudomonas or Serratis @ sternoclavicular or sacroiliac joints
What is the presentation of disseminated gonococcal arthritis syndrome?
Septic arthritis in younger, sexually active person.
Fever, dermatitis and an inflammatory tenosynovitis. 50% have inflammatory arthritis (knee, wrist or ankle). Rash sometimes with pustules or hemorrhagic vesicles is common
What is the treatment of gonococcal arthritis?
Later third generation cephalospsorin: ceftriaxone 1.0g daily and antichlamydial antibiotic.
What are the clinical presentation, radiographic finding, and diagnostic testing of infected joint prostheses?
Clinical presentation: No fever, hard to detect. Return of pain and evidence of prosthetic loosening after some time.
Radiographic finding: Lytic changes around the prosthesis. Negative bone scan.
Diagnostic testing: Increased erythrocyte sedimentation rate. Aspiration of fluid from prosthetic joint to confirm infection.
What are the organisms involved and symptoms of early versus late infected joint prostheses and know when these occur?
Before 6months after the replacement it is usually staph aureus and staph epidermidis.
After 6months, it’s gram negative bacterial.
Early infection: hard to detect and no fever.
Late infection: return of pain and evidence of prosthetic loosening after some time
What are the states where Lyme disease is endemic?
Connecticut Delaware Maryland Massachusetts New Jersey New York **Pennsylvania Rhode Island Minnesota Wisconsin California Nevada Oregon Utah
(NOT CALIFORNIA. HA. HAHA. HAHAHAHA)
What are the symptoms occurring at each of the stages of Lyme disease?
Stage I: (several days to a month)
Erythema chronicum migrans
Flu like symptoms (fever, headache, malaise and adenopathy)
Stage II (wks~months)
Dissemination infection in the skin, MSK system, heart and nervous system
Neurologic symptoms (Bell palsy, meningoencephalitis and sensory & motor radiculoneuritis) 15%
Cardiac abnormalities including heart block (5% of pts)
Arthritis (knees) with baker cysts 50%
Stage III (several years)
Arthritis that becomes chronic
What are the diagnostic testing, results in patients previously treated with an antibiotic, and confirmatory testing used in Lyme disease?
Measure ab to spirochete via ELISA (other disease can be positive as well) so if ELISA is positive, confirm it with Western blot assay
If patients are treated early with antibiotic, the results might never be positive
What are the various treatments used in Lyme disease?
Early tx: Oral tetracycline or doxycycline or amoxicillin in children
Pt w/ neurologic, cardiac and arthritic symptoms: add ceftriaxone
What is the clinical presentation of poststreptococcal reactive arthritis?
It affects 2/3 of patients with rheumatic fever. Arthritis in large joints (knees, ankles, elbows and wrist). Arthritis may be migratory with each joint remaining inflamed for approx. 1 week. Repeated attacks may result in Jaccoud deformity (metacarpophalangeal joints are in ulnar deviation)
What is the Tx of poststreptococcal reactive arthritis?
Joint symptoms without carditis may be treated with high-dose salicylates. Corticosteroids may be required if pts do not respond to salicylates.
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