Section 2: SLE, Sjogren's syndrome, CREST Syndrome, Eosinophilic Fascitis Flashcards
Diagnosis
- Rash
- Joint pain
- Fatigue
Lupus
List the 11 criteria for systemic lupus erythromatosus (SLE)
Skin
- Malar rash
- Photosensitivity rash
- Oral ulcers rash
- Discoid rash
Arthralgias (Present in 90 percent of patients)
Blood: Leukopenia, thrombocytopenia, hemolysis; any blood involvement counts as 1 criterion
Renal: Varies from benign proteinuria to end stage renal disease
Cerebral: Behavioral change, stroke, seizure, meningitis
Serositis: Pericarditis, pleuritic chest pain, pulmonary hypertension, pneumonia, myocarditis
Serology
- ANA (95 percent sensitive)
- Double-stranded (DS) DNA (60 percent sensitive)
Each of the serologic abnormalities counts as 1 criterion. Hence, if the person has joint pain, a rash, and both an ANA and DS DNA, that patient would have 4 criteria.
Use the Mneumonic “DOPAMINE RASH”
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4368-4393). . Kindle Edition.
How many of the 11 diagnostic criteria need to be present to make a diagnosis of SLE
4
SLE
- Best initial test
- Most specific test
- ANA
- Anti-DS DNA or anti-Sm (Smith)
SLE on CCS:
- Complement levels
- anti-Sm, and anti-DS DNA should be performed on all patients
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4395-4412). . Kindle Edition.
What is the best test to follow the severity of a lupus flare-up?
Complement levels (drop in flare-up) and anti-DS DNA (rise in flare-up)
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4395-4412). . Kindle Edition.
As part of prenatal care, a woman with lupus is found to have a negative test for anticardiolipin antibodies, but she is positive for anti-Ro (SSA) antibody. What is the baby at risk for?
Heart block. The presence of anti-Ro or anti-SSA antibodies is a risk for the development of heart block.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4395-4412). . Kindle Edition.
The anemia of chronic disease is more common than hemolysis in SLE.
List the other findings in lupus that are not part of specific diagnostic criteria
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4395-4412). . Kindle Edition.
- Fatigue
- Hair loss
- Antiphospholipid syndrome
- Elevated sedimentation rate
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4395-4412). . Kindle Edition.
Rx of SLE
Acute flare-ups: Prednisone and other glucocorticoids
Joint pain: NSAIDs
Rash and joint pain not responding to NSAIDs:
- Hydroxychloroquine and antimalarials
Severe disease relapse upon cessation of steroids:
- Belimumab
- Azathioprine
- Cyclophosphamide
Nephritis: Steroids and mycophenolate mofetil (mycophenolate is superior to cyclophosphamide) Belimumab inhibits B cells as treatment of SLE. Drug-Induced Lupus The most common causes of drug-induced lupus are hydralazine, procainamide, and isoniazid. Keep the following in mind on Step 3: Drug-induced lupus always gives anti-HISTONE antibodies or a positive ANA. It never gives renal or CNS involvement. Complement level and anti-DS DNA are normal.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4413-4425). . Kindle Edition.
List the common causes of drug-induced lupus
- Hydralazine
- Procainamide
- Isoniazid
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4413-4425). . Kindle Edition.
Features of drug-induced lupus
- Always gives anti-HISTONE antibodies or a positive ANA
- It never gives renal or CNS involvement
- Complement level and anti-ds DNA are normal.
Diagnosis
Look for a woman (9: 1 female predominance) with dry eyes, dry mouth, and a sensation of “sand under the eyelid.” There is often loss of taste and smell from profound mouth dryness. (You need saliva to wet the food so you can taste it.) Look for loss of teeth at an early age, because saliva is critical to preventing dental cavities.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4427-4431). . Kindle Edition.
Sjogren’s Sydrome
- Most accurate test
- Describe Schirmer test
- Lip biopsy
- Decreased wetting of filter paper held to the eye shows decreased lacrimation.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4432-4433). . Kindle Edition.
List the serological tests in Sjogren’s syndrome
ANA: 95 percent sensitive but least specific
RF: 70 percent sensitive
Anti-Ro/ SSA: 50– 65 percent sensitive but fairly specific
Anti-La/ SSB: 30– 65 percent sensitive but fairly specific
CCS Tip: When you see anti-Ro (SSA) or anti-La (SSB), think Sjögren’s syndrome. They are present in a small number of people with lupus, and can help diagnose ANA-negative lupus.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4433-4445). . Kindle Edition.
Rx of Sjogren’s syndrome
- Keep the eyes and mouth moist
- Pilocarpine and cevimeline increase acetylcholine, which increases oral and ocular secretions.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4446-4448). . Kindle Edition.
Diagnosis
- Tight Skin + Heartburn + Raynaud’s
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4454-4455). . Kindle Edition.
Scleroderma
CF of scleroderma
- Skin: Look for a woman with tight, fibrous thickening of the skin that gives a tight face and tight, immobile fingers known as “sclerodactyly.”
- Raynaud’s phenomenon: This is a 3-phase vascular hyperreactivity, with the skin of the fingers becoming white, then blue, then red; it can be quite painful. Digital ulceration may occur from infarction of the skin. There might also be abnormal giant capillaries
- Joint pain:The pain is mild and symmetrical.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4451-4459). . Kindle Edition.
CF of diffuse scleroderma
- Diffuse scleroderma also presents with the following:
- Lung: Fibrosis and pulmonary hypertension (these are the leading cause of death)
- GI: Wide-mouthed colonic diverticula and esophageal dysmotility, leading to reflux and Barrett’s esophagus. There is primary biliary cirrhosis in 15 percent of patients.
- Heart: Restrictive cardiomyopathy
- Renal: May lead to malignant hypertension
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4462-4479). . Kindle Edition.
Diagnostic tests in scleroderma
There is no single diagnostic test
- ANA is present in 95 percent of cases but is nonspecific
- Antitopoisomerase (anti-Scl 70) is only present in 30 percent of patients. Treatment No treatment has been proven effective in stopping scleroderma. Penicillamine is not effective in delaying progression of this disease. One can use the following therapies: Renal involvement and hypertension: Use ACE inhibitors. Pulmonary hypertension: Use bosentan (endothelin antagonist), prostacyclin analogs (epoprostenol, treprostinil, iloprost), or sildenafil. Raynaud’s: Use calcium channel blockers. GERD: Regular use of PPIs Lung fibrosis is treated with cyclophosphamide. Interstitial lung disease is treated with cyclophosphamide.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4462-4479). . Kindle Edition.
Rx of scleroderma
No treatment has been proven effective in stopping scleroderma
- Penicillamine is not effective in delaying progression of this disease.
- Renal involvement and hypertension: Use ACE inhibitors.
- Pulmonary hypertension: Use bosentan (endothelin antagonist), prostacyclin analogs (epoprostenol, treprostinil, iloprost), or sildenafil.
- Raynaud’s: Use calcium channel blockers.
- GERD: Regular use of PPIs
- Lung fibrosis is treated with cyclophosphamide. Interstitial lung disease is treated with cyclophosphamide.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4462-4479). . Kindle Edition.
What is the characteristic serological findings in CREST?
CREST is characterized by anticentromere antibodies
CREST syndrome does not involve anti-Scl70
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4481-4493). . Kindle Edition.
CF of CREST syndrome (Scleroderma)
- Calcinosis of the fingers
- Raynaud’s
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
CREST does present with primary hypertension. It does not present with the following:
Joint pain
Heart involvement
Lung involvement (except for pulmonary hypertension)
Kidney involvement
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4481-4493). . Kindle Edition.
Eosinophilic fasciitis presents with thickened skin that looks like scleroderma. However, some features are absent. List them.
List two other characteristic features of eosinophilic fascitis
Rx?
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4495-4500). . Kindle Edition.
No hand involvement
No Raynaud’s phenomenon
No Heart, lung, or kidney involvement
There is marked eosinophilia and the appearance of an “orange peel” (peau d’orange)
Treatment is with corticosteroids.
Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kindle Locations 4495-4500). . Kindle Edition.
The most common glomerulonephritis in SLE
Membranous glomerulonephritis
Lab tests for acute lupus flare
Complement levels drop and abti-DS DNA levels rise
