MSK2-Table 1 Flashcards
What is systemic lupus erythematosus (SLE)?
A multisystem autoimmune connective tissue disease characterized by the presence of numerous autoantigens, autoantibodies, and circulating immune complexes with widespread immunologically determined tissue damage
What pt populations does SLE more frequently affect?
Non-white, AA more susceptible with severe disease, 90% female of child bearing age
When is SLE most frequently diagnosed?
Between 15-45 years old
What causes early death in SLE?
Active lupus and infection
What causes late deaths in SLE?
Sequelae of chronic lupus, chronic medications, CVD, infections
What is the most likely pathogenesis of SLE?
Likely genetic component in the setting of immune dysregulation, environment, and hormonal triggers
What environmental triggers are implied with SLE?
UV lights, microorganisms, tobacco, others
What are the 2 ways SLE autoantibodies cause tissue injury?
1) Formation of immune complexes and depostition of Ig at dermal- epidermal jxn and in renal tissue
2) damage or destruction of specific cells causing inflammation, activated PMNs, and release of oxygen radicals and lysozymes that damage and destroy tissues
What drugs can induce SLE?
Procainamide, hydralazine, isoniazid, methyldopa, quinidine, chlorpromazine, phenothiazines, OCPs, anticonvulsants
What are the clinical features of SLE?
Arthritis, arthralgia, and fever
Specifically polyarthralgia
What is the most common constitutional symptom in SLE?
Fatigue, occurs in >90%
What is the onset of symptoms like?
Gradual onset with flares and remission
What can happen with pregnancy and SLE?
May onset during pregnancy and may be present in women with hx of spontaneous abortion
Babies SGA
What vascular condition is common in SLE?
Raynauds
2/3 of pts with SLE have what?
Skin signs- lupus dermatitis
How might the skin conditions present?
vasculitis, purpura or periungual erythema. Classic malar butterfly rash and discoid lesions
What CNS manifestations present in SE?
Epilepsy, depression, dementia, psychosis, hemiplegia, chorea, ataxia, pripheral neuropathy
What are abdominal manifestations in SLE?
Ab pain, peritonitis, splenomegaly, pancreatitis, GI ulcers
What are ocular manifestations in SLE?
Keratoconjunctivitis, episcleritis, retinal vasculitis & retinal exudates
What is diagnostic criteria for SLE?
4 or more of the following during the course of the disease:
-—Malar Rash —Oral Ulcers
—Arthritis
—Serositis (heart/lungs)
—Renal Disease (proteinuria, cellular casts)
—Hematologic Disorders (hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia)
—Immune Disorders (including false + syphilis)
—Neurological Disorders (sz., psychosis)
—Discoid Rash
—Photosensitivity
—+ANA
what immunologic criteria is most common in lupus?
Positive ANA
How should SLE be managed?
REFER
- NSAIDS, exercise, sun protection, smocking cessation, antimalarials, corticosteroids
What is the mainstay tx for SLE and what needs to be monitored?
Antimalarials - Watch for retinal toxicity
- baseline and annual eye exams with visual field testing
When are corticosteroids mainstay?
If antimalarials are insufficient
What corticosteroid combo is superior for preventing renal failure in SLE?
Prednisone + cyclophosphamide
What should be used to tx SLE in pregnancy?
hydroxychloroquine and prednisone/prednisolone
What comorbid conditions are associated with SLE that need to be assessed for and tx?
- -Accelerated atherosclerosis
- -Pulmonary hypertension
- -Antiphospholipid syndrome
- -Osteopenia or osteoporosis
- -Non-Hodgkin lymphoma
What preventative measures can be taken with SLE?
—Sun protection (clothing and sunscreen)
—Vaccination (influenza and pneumococcal)
—Caution with live-attenuated vaccines and immunosuppression
—Suppress recurrent infections (UTIs)
—Prevent osteoporosis (steroids)
—Prevent and control CVD and obesity
What are the general characteristics of sjogrens syndrome?
Autoimmune disorder causing destruction of salivary and lacrimal glands- exocrine
What is the most common pt population for sjogrens syndrome?
Most common in middle aged females (9:1 ratio female: male)
What is the pathophys involved in sjogrens?
B lymphocyte hyperactivity
Infiltration of the exocrine glands by lymphocytes, resulting in inflammation and destruction of tissue
Infiltration of viscera and skin leading to extrglandular conditions
What are the cardinal clinical features of sjogrens?
Exocrine malfunction: dry mouth, dry eyes, enlargement of the parotid glands
Constitutional: fatigue, fever, wt loss, lymphadenopathy
Extraglandular: articular and raynaud phenomenon, B cell lymphomas
What is the diagnostic criteria for sjogrens?
4 of 6 of the following or 3 or 4 of the objective criteria ( III, IV, V, VI)
I. Ocular symptoms
II. Oral symptoms (dry mouth, swollen glands)
III. Ocular signs (Shirmer’s test, Rose Bengal)
IV. Histopathology (biopsy minor salivary glands)
V. Salivary gland testing
VI. Reactive antibodies to RO/SS-A or La/SS-B
What are other tests that can be done to diagnose sjogrens?
Shirmers test- evaluates tear secretions, less than 5mm in 5 min is positive
Corneal abrasion- stain with slip lamp evalv
Biopsy of lower lip mucosa- confirms lymphocytic infiltrate gland fibrosis
What is the treatment of sjogrens?
Symptomatic tx- artificial tears and saliva, ocular and vaginal lubricants, oral lubricants ,Protect eyes, Increase PO intake
NSAID- for arthralgia
Antimalarials/ methotrexate- 1st line for extraglandular
Nifedipine and prostacycline for raynauds
ACEI for HTN
What is systemic sclerosis (scleroderma)?
Multi-system autoimmune dz of unknown cause with chronic and progressive features
What is the hallmark of scleroderma?
Vasculopathy and excess collagen deposits
What does scleroderma lead to?
Fibrosis and degenerative changes of skin and internal organs
Ulcerations, ischemia, atrophy and gangrene of the skin and digits
What are early manifestations of scleroderma?
Raynauds and skin calcinosis
Wrist pain, wheezing, skin pigments, joint pain, hair loss and alopecia, eye burning itching or discharge
When it the typical age of onset in scleroderma?
30-50
What gender is most affected by scleroderma?
Females… 4.6: 1 ratio
What are the 2 types of systemic sclerosis?
Diffuse cutaneous
Limited cutaneous- crest syndrome
What does diffuse typically affect?
Skin, heart, lungs, GI tract, and kidneys
Typically worse!
What does crest syndrome affect?
Skin of the face, neck, elbows, knees
In late stages it may cause isolated pulmonary HTN
Raynauds associated with critical ischemia of the digits
What are the cardinal clinical features of scleroderma?
Skin – most begin with swelling in the fingers and hands, may spread to the trunk or the face
Raynauds
Polyarthralgias and esophageal dysfxn
Tendon or bursal friction rubs often seen prior to thickening of the skin
What are the systemic and MSK changes in scleroderma?
Arthritis, arthralgia, crepitus of tendon sheaths, non-progressive myopathy with weakness and atrophy
What are the skin changes in systemic sclerosis?
Non pitting edema- “sausage” swelling of fingers
Skin shiny and atrophied with fingertip ulcers
Decreased facial pigment and presence of telangiectasias
Tightening over bony prominences
Flexion contractures
Loss of adipose tissues
Symmetric and bilateral skin changes
What is the hallmark skin change in crest?
Telangiectasias
What is the mneumonic for CREST?
Calcinosis Raynauds Esophageal dysfxn Sclerodactyly Telangiectasias
What are GI changes associated with Systemic sclerosis?
Reflux esophagitis, hiatal hernia, loss of normal peristalsis, dilatation of large and small bowel, pain, constipation, diarrhea, primary biliary cirrhosis
What are some complications of scleroderma?
Cardiac failure, renal failure (emergency), pulmonary fibrosis, R heart failure, malabsorption, malnutrition, osteoporosis
How is scleroderma diagnosed?
Primarily a clinic diagnosis
Can scleroderma be cured?
No, only supportive and organ specific tx
What are the organ specific tx for scleroderma?
Antihistamines and low-dose steroids for early skin involvement
PPI for GERD
ACEI for Renal
Ca+ Channel Blockers or ARBs for Raynaud’s
Bosentan or sildenafil for pulmonary HTN
PT with daily stretching to avoid contracture
What is polymyalgia rheumatica?
inflammatory rheumatic condition characterized clinically by aching and morning stiffness in the shoulders, hip girdle, neck, and torso
What are 15% of pts with PMR also diagnosed with?
Temporal arteritis ( GCA)- complication is blindness
What gender is more commonly affected?
Female >male, 2-3:1
What is the typical pt population?
Female, northern European, age around 50 but peaks 70-80
What gene is PMR associated with?
HLA_DR4
What are the cardinal clinical features of PMR?
Stiffness is predominant symptom in shoulders, hip girdle, neck, and torso ÷Most severe after rest and in the AM ÷Stiffness may last more than 30 minutes ÷Bilateral, proximal, symmetrical ÷Onset may be abrupt
What are other symptoms of PMR?
systemic malaise, fatigue, depression, anorexia, weight loss, and low-grade fever
How is PMR diagnosed?
Empirical criteria:
- Age >50 at onset
- Proximally and bilaterally distributed aching and morning stiffness (lasting at least 30 minutes or more) persisting for at least two weeks. The stiffness should involve at least two of the following three areas: neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the thighs
- Erythrocyte sedimentation rate (ESR) ≥40 mm/hr
- Rapid resolution of symptoms with low-dose glucocorticoids. Symptoms are generally 50 to 70 percent better within three days in patients with PMR started on prednisone at a dose of 10 to 20 mg/day. The lack of response to initial therapy strongly suggests an alternative diagnosis
What MUST you assess for if your pt is diagnosed with PMR?
GCA- >50, fever, elevated ERS, anemia with temporal artery tenderness, HA
How is PMR treated?
Low dose corticosteroid therapy
- Prednisone 15mg PO Qday
- may need for up t 2 years
- higher dose plus ASA is GCA
What is fibromyalgia?
A controversial chronic pain syndrome characterized by persistent, widespread pain and tenderness to palpation at defined tender points in soft tissue structures
What is the prevalence of fibromyalgia?
Female : male = 9:1 ration
How is fibromyalgia diagnosed?
Diagnosis of exclusion
r/o: inflammatory disorders and thyroid by obtaining CBC, ESR, CRP, and TSH
What is fibromyalgia strongly associated with?
Mood disorders
What are the cardinal clinical features of fibromyalgia?
- -Non-articular, diffuse and poorly localized musculoskeletal pain, usually accompanied by fatigue and sleep disturbance- “Pain all over!”
- –Diffuse tenderness on exam
- —constellation of additional symptoms- fatigue, stiffness, poor sleep, panic disorder, depression, HA/ “migraine”, irritable bowel, dysmenorrhea, and parasthesias may be seen
Are narcotics indicated for tx of fibromyalgia?
NO
How is fibromyalgia managed?
Amytriptyline 10mg 1-3 hours prior to bed, titrate to 25-50mg
Cyclobenzaprine is an alternative
SNRIs may be helpful if fatigue is severe
Anti-convulsants- gabapentin and pregabalin (for sleep disturbance and pain)
Pregabalin dose- 25-50mg PO QHS and titrate up to 300-450mg QDAY
Aerobic exercise to improve conditioning is very important
— education and psych counseling
