Connective Tissue Diseases

Question 1

Drug induced SCLE

Answer 1

HCTZ, PPIs

ACEI, CCB, IFNs,

TNFi as a trigger for psoriasiform SCLE—photosensitivity is a clue vs. PSO

Question 2

SCLE antibodies

Answer 2

Ro/SSA antigen

HLA DR3

Question 3

Neonatal LE

Answer 3

Associated with Ro/SSA antibodies La/SSB,

Only U1RNP do not develop heart blocks

50% of patients have permanent 3rd degree heart block

Cutaneous lesions: thrombocytopenia and hepatic disease

Risk of 2nd child with neonatal SLE is 25%

Question 4

Criteria for SLE

Answer 4

4 of 11:

• malar rash
• discoid rash
• photosensitivity
• oral ulcers
• arthritis
• proteinuria >0.5g/day or casts
• neurologic disorders
• pleuritis/pericarditis
• Blood abnormalities
• immunologic disorders
• positive ANA

Question 5

Bullous SLE

Answer 5

• Suberpidermal bulla and superficial dermal edema containing neutrophils in or below lamina densa

HLA-DR2 positive

Therapuetic effect with Dapsone vs. EBA which doesnt

Question 6

Cutaneous symptoms of lupus

Answer 6

red or spotted lunulae

periungal telangiectasia

Non scarring alopecia- chronic telogen effluvium

Conjunctivitis, episcleritis, nasal, oral, vaginal ulcers

erythema, petechaie, ulcerations on the hard palate

eruptive dermatofibromas

calcinosis cutis

papulonodular mucinosis

Question 7

Systemic lupus

APLA

Answer 7

higher rate of peripheral artery disease

2/2 to lupus anticoagulant

Associated with APLA: anticardiolipin antibodies, antiphosphatidylserine, antiphosphatidylinositol, antiphosphatodylserine

presence of APLAs indicate higher morality

false + for syphilis

Myocarditis- cardiomeglay, pericarditis, endocarditis

CNS- hemiparesis, epilepsy, diplopia, retinitis
–Livdeo reticularis marker for higher risk of CNS lesions

Question 8

Drug induced SLE

Answer 8

Antihistone antibodies and +ANA

Penicillamine unmasks true SLe

Question 9

dsDNA

Answer 9

high risk of renal disease

positive DIF in sun protected skin

Question 10

Anti-La antibodies

Answer 10

common in SCLE

Question 11

Anti-Ro

Answer 11

Common in patients with SCLE , neonatal LE

Question 12

Lupus band test

Direct cutaneous immunofluorescence

Answer 12

DLE: Continuous granular deposits of immunoglobulins and complement along the DEJ

positive test in normal, protected skin correlates with the presence of anti-dsDNA antibodies and renal disease

Question 13

Counseling points for Lupus patients

Answer 13

osteoperosis in women independent of steroid use
Supplement calcium and vitamin D

Vitamin D repletion can improve disease activity

Consider Bisphosphonates in women

photo protection for all

Question 14

Sjogren Syndrome

Answer 14

Lymphocytic infiltration of the exocrine glands
salivary and lacrimal glands

Sjögren syndrome alters the composition of saliva, producing a decrease in salivary amylase and carbonic anhydrase, along with an increase in lactoferrin, β2-microglobulin, cystatin C, sodium, and lysozyme C

2/3:

• xeropthalmia
• xerostomia
• associated autoimmune, rheum or lymphoproliferative disorder

Anti SSA. SSB, IgA and IgG antibodies against α-fodrin

aquaporin family of water channels (proteins freely permeated by water but not protons) appears to be an important target

Question 15

Diseases associated with RA

Answer 15

Erythema elevatum diutinum, pyoderma gangrenosum, Felty syndrome, IgA vasculitis, linear IgA disease, Sjögren syndrome, bullous pemphigoid, and yellow nail syndrome