02-Connective Tissue Flashcards
- Q: What are the forms of chronic cutaneous lupus
A: Discoid lupus, hypertrophic lupus, LE-LP overlap, Chilblain LE, Tumid LE, Lupus panniculitis
- Q: Who does DLE most commonly affect?
A: African American women (2:1), young adults
- Q: What does Langue au chat refer to?
A: cat’s tongue – refers to the carpet tack-like follicular plugging that occurs in DLE
- Q: What is the most common location for DLE to occur?
A: Above neck – commonly affects the concha bowl and scalp
- Q: What do you need to worry about in long-standing DLE?
A: Squamous cell carcinoma
- Q: Childhood DLE has a higher progression to?
A: SLE – Childhood DLE has an equal incidence of M:F
- Q: What percentage of patients with DLE have SLE?
A: 5-15%
- Q: What percentage of SLE patients have DLE?
A: 25%
- Q: What percentage of patients with DLE will have a positive DIF on histology?
A: 75%
- Q: LE-LP overlap syndrome most commonly affects what anatomical area?
A: palmoplantar involvement – also oral w/ scarring alopecia
- Q: What mutation has been reported in familial forms of chilblain lupus?
A: TREX1 mutation
- Q: How often does tumid lupus have overlying epidermal involvement?
A: It doesn’t
- Q: Tumid lupus is considered an overlap with what other disease entities?
A: REM (reticular erythematous mucinosis) and jessner’s lymphocytic infiltrate
- Q: What percentage of tumid lupus patients will eventually develop SLE?
A: 50%
- Q: What percentage of tumid lupus patients will have overlying DLE?
A: 1/3rd
- Q: What anatomical area does lupus panniculitis usually spare?
A: Typically does not affect the distal extremities
- Q: What percentage of patients with SCLE will have SLE?
A: 30%
- Q: What percentage of patients with SCLE will have a positive ANA?
A: 80%
- Q: What antibody is often present in 70% of SCLE patients?
A: Ro/SSA: 70%
- Q: 10-15% of SCLE patients will develop internal symptoms, what do these include?
A: Arthritis, leukopenia, renal, CNS, and vascular complications.
- Q: What is the common clinical description of SCLE lesions?
A: Sun exposed scaly, annular plaques in white women (15-40)
- Q: 30% of SCLE cases are drug-induced, what are the common causes?
A: HANG NAIL – HCTZ, Ace Inh, NSAIDs, Griseofulvin, Nifedipine (CCBs), Anticonvulsants, Interferon, Lamisil
- Q: What is the most important treatment for both DLE and SCLE?
A: Sunscreen!!!!
- Q: Neonatal lupus is due to transferring of ___ antibody across placenta from affected mothers.
A: Ro/SSA antibody
- Q: What is the risk that a second child of a mother with a previously affected neonatal lupus pregnancy?
A: 25%
- Q: What medication can lower the risk of neonatal lupus to ~ 7% if initiated prior to 10 weeks gestation?
A: Hydroxychloroquine
- Q: What is the most serious complication associated with neonatal lupus?
A: Congenital third-degree heart block
- Q: If only __ ab is present, then there is no risk of heart block in neonatal lupus?
A: U1RNP
- Q: What are the other associations important in neonatal lupus?
A: thrombocytopenia, hepatobiliary disease
- Q: What is the clinical presentation seen in neonatal lupus?
A: periocular edema and erythema (‘raccoon’s eyes’) – none at birth, presents in first few weeks of life
- Q:Who does SLE affect?
A: Young to middle aged women (6:1), and African Americans (4:1).