Automimmune And Rheumatic Diseases

Question 1

What is the most frequent clinical manifestation of LE? What is the second most frequent clinical manifestation?
A. Skin disease
B. Joint inflammation
C. Alopecia
D. Headache

Answer 1

B. Joint inflammation (p1038)
A. Skin disease

Question 2

Which of the following is NOT classified as LE-specific skin disease?
A. Chilblain lupus
B. Lupus erythematosus gyrates repens
C. Malar rash
D. Urticarial vasculitis

Answer 2

D. Urticarial vasculitis (p1038)

A. Chilblain lupus-CCLE
B. Lupus erythematosus gyrates repens-Annular SCLE
C. Malar rash - localized ACLE

Question 3

Which of the following is TRUE of classic DLE?
A. Primarily a disease of white females
B. Mean age of onset in the 5th decade
C. 5% of patients with isolated localized DLE will subsequently develop SLE
D. Constitutes 7% to 27% of LE patients

Answer 3

C. P1039

All the other statements describe SCLE lesions. DLE is present in 15%-30% of SLE populations and common between 20-40yo, prevalent in blacks.

Question 4

Which of the following is NOT included in the 1982 Revised Criteria for Classification of SLE?
A. Fixed erythema, flat or raised over the malar eminences and nasolabial folds
B. Painless oral ulceration
C. Seizure or psychosis in the absence of offending drugs or known metabolic derangements
D. Abnormal titers of anti-dsDNA, anti-Smith antigen and ANA

Answer 4

A. Sparing of NL folds (p1039)

Question 5

What is considered a proximal and driving event in lupus pathogenesis?
A. Inappropriate type 1 IFN activity
B. Genetic predisposition
C. Abnormal hormonal milieu
D. Excessive UVR exposure

Answer 5

A. P 1040
UVB irradiation is the most important environmental factor in the induction phase of SLE esp LE specific skin disease; releases the cutaneous T cell-attracting chemokine that activate autoreactive T cells and type 1 IFN
Smokers>non-smokers—treatment resistant CLE
Epstein Barr virus
Drugs (see table 61-4)

Question 6

A 32-year-old female presents with finger pain when exposed to cold temperatures.

She has a history of tobacco use and drinks three cups of coffee per day. She notices the pain when she reaches into her freezer or when she’s at the grocery store. She also notes the color change to her fingers, first they turn white, then blue, then red. She now must wear gloves when she goes outside, even in only mildly cold temperatures. She has no other symptoms and on exam she has no skin thickening notes.

Which of the following is a reasonable first step in treating her Raynaud’s?
A. Aspirin
B. Lifestyle modification (tobacco cessation, less caffeine consumption)
C. Acetaminophen
D. Warfarin

Answer 6

B. Lifestyle modification-first step in treating Raynaud’s

Question 7

Which of the following is NOT a characteristic feature of SCLE?
A. Commonly involves the neck, shoulders, upper extremities and trunk
B. Tend to be transient
C. Sharply demarcated, coin-shaped, hypopigmentation, atrophic dermal scarring, follicular plugging, and adherent scale.
D. Heal with more pigmentary changes

Answer 7

C. Feature of DLE p.1044

Question 8

Which of the following is NOT a feature of DLE?
A. Reversible, nonscarring alopecia
B. May involve below the neck, extensor aspects of the arms, forearms, and hands
C. Recalcitrant to standard therapy
D. Can involve the malar areas with sparing of nasolabial folds

Answer 8

A. P1045; SLE causes reversible, nonscarring alopecia

Question 9

What is the Second Line of Treatment for Lupus Erythematosus-Specific Skin Disease?
A. Topical calcineurin inhibitor
B. Hydroxychloroquine
C. Methotrexate
D. Dapsone

Answer 9

B. P1054 Table 61-6

Question 10

Which of the following is NOT a Major criteria for Dermatomyositis?
A. Gottron papules
B. Gottron sign
C. Shawl sign
D. Heliotrope sign

Answer 10

C. P1064

Question 11

What cutaneous finding is specific to DM and is valuable in differentiating DM from Cutaneous Lupus?
A. Reticulated white macules surrounded by telangiectatic red macules
B. Presence of atrophy, hyperpigmentation, hypopigmentation, and telangiectasias
C. Calcinosis
D. Non-scarring and diffuse alopecia

Answer 11

A. “Red on white” p. 1067

Question 12

Which of the following is seldom necessary to establish the diagnosis of DM in a clinic setting?
A. EMG
B. MRI
C. Muscle biopsy
D. Laborating testing for autoantibodies and muscle enzymes

Answer 12

C. P.1074

Question 13

What is the hallmark autoantibody in a patient with diffuse skin sclerosis, pulmonary fibrosis, secondary PAH, increased SSc-related mortality rate?
A. CENP proteins speckled pattern
B. Topoisomerase-1 speckled pattern
C. RNA polymerase III speckled pattern
D. U1RNP speckled pattern

Answer 13

B. Scl-70 p1090

A. Centromere-limited skin sclerosis, severe gut disease, isolated PAH, calcinosis
C. RNAP III-diffuse skin sclerosis, hypertensive renal crisis, with higher mortality rate
D. nRNP-Overlap features of SLE, arthritis

Question 14

Digital ulceration on the finger- or toe-tips, the major external feature of structural vessel disease, can be seen in what percentage of patients with SSc?
A. 20%
B. 30%
C. 40%
D. 50%

Answer 14

D. 50% p1090

Question 15

What is the most common cause of disease-related death in patients with SSc?
A. Gastric antral vascular ectasia
B. Pulmonary arterial hypertension (PAH)
C. Scleroderma Renal Crisis (SRC)
D. Pulmonary fibrosis (PF)

Answer 15

B. PAH p1090

Question 16

The first inflammatory infiltrates in SSc lesional skin are predominantly cells of the monocyte lineage. Which of the following are considered important contributors of cytokines which influence the fibrotic response?
A. T cells
B. Macrophages
C. B cells
D. Mast cells

Answer 16

B. Macrophages p1094

Question 17

What is the mainstay of therapy for critical digital ischemia?
A. Sildenafil
B. Clopidogrel
C. Iloprost
D. Nifedipine

Answer 17

C. Prostacycline derivatives by IV infusion p1100

A. Phosphodiesterase Type 5 inhibitors
B. Antiplatelet agents
D. Calcium channel blockers

Question 18

Which of the following is NOT True regarding Morphea?
A. Self-limiting and recurrences are rare occurring in <25% of patients with linear morphea of extremities
B. Assessment via MRI and ultrasonography is useful for determining lesion activity and depth
C. Generalized morphea is characterized by more than or equal to 4 lesions in at least 2 of 7 different anatomic sites
D. Methotrexate is considered first-line systemic treatment for morphea especially for deep, rapidly progressive, or disabling morphea

Answer 18

A. High risk for recurrences (31%) for linear morphea of extremities p1112

Question 19

How many years after cessation of UVA-1 phototherapy can recurrences develop in 50% of cases?
A. 1 year
B. 2 years
C. 3 years
D. 4 years

Answer 19

C. 3years p1115

Question 20

Which of the following is NOT considered an effective treatment for genital LS?
A. Mometasone furoate 0.1%
B. Clobetasol propionate 0.05%
C. Tacrolimus
D. Topical progesterone

Answer 20

D. Progesterone and topical androgens p. 1121

Clob and mometasone had comparable efficacy in treatment of vulvar LS bid for 2-3 mos in tapering doses (od x 4 weeks, eod x 4weeks, 2x a week for 4 weeks)
Shift to calcineurin inhibitors if there is cutaneous atrophy from steroids

Question 21

Which of the following is considered a potential trigger of Dermatomyositis?
A. Adrenal fatigue
B. Low estrogen levels in women
C. Viral infections
D. Vitamin B12 deficiency

Answer 21

C. Viral infections (Coxsackie virus, enterovirus, SARS Cov-2)

Question 22

RA should be suspected in patients with polyarticular, symmetric arthritis, particularly if the wrists and 2nd and 3rd metacarpophalangeal joints are involved. Alternative diagnoses should be suspected in which of the following?
A. Patients with Rheumatoid nodules
B. Patients with NO DIP joint symptoms
C. Low RF titers
D. Patients with prominent lumbar symptoms

Answer 22

D. Prominent lumbar symptoms — need to investigate other possible diagnosis