DCNP - Connective Tissue Disease Flashcards
SLE epidemiology
10x more females
African American > Caucasian
SLE serologies
CBC (platelet count)
ESR
+/- CRP,
ANA panel -
titer 1:320 3% false pos
anti-dsDNA & anti-Sm (highly specific)
anti-RNP, anti-Ro (SS-A), anti-La (SS-B)
histone if suspect drug-induced
urinalysis w/ microscopy
SLE presentation
insidious onset (fatigue, fever, arthralgia, weight change in early onset)
Eczematous plaques
Photosensitivity
Myalgia and arthralgia in 90%
Renal involvement develops in 50%
Raynaud’s phenomenon
Vasculitis (usu. small/med vessel w/ purpura)
Hair
Alopecia - non-scarring, diffuse hair loss
Broken hairs frontal hair line (“lupus hairs”)
Skin
Telangiectasias
Erythema (between MCPs)
Oral ulcerations
Lesions vary significantly dependingon the type of lupus: papules,plaques, vesicles/bullae, scale or smooth, hyper- and hypopigmented, scarring.
Nails
Dilated capillary loops proximal nail folds
ACR 1997 Systemic Lupus Diagnostic Criteria
Patient must have 4 out of 11 signs/symptoms present
“SOAP BRAIN MD”
Serositis (lungs, heart, etc.)
Oral ulcers
Arthritis
Photosensitivity
Blood disorders (anemia, thrombocytopenia, leukopenia, HSM, etc)
Renal involvement
Antinuclear antibodies
Immunologic phenomena (i.e. dsDNA, anti-Smith antibodies, antiphospholipid antibodies)
Neurologic disorder
Malar rash
Discoid rash
Acute Cutaneous Lupus Erythematosus
“Butterfly rash” (spares NLF)—although HALLMARK, only occurs in 10-50%—is not the most common cutaneous sign
erythematous patches/plaques
may be bullous, discoid or erosions
extensor extremities
spares IP in fingers/hands
multisystem organ involvement (pts are sick)
8:1 females to males
onset in 30-40s
lasts hrs-days
multisystem involvement w/ cutaneous flares (pts sick)
95% + ANA
risk of SLE >90%
subacute cutaneous lupus erythematosus
Annular, polycyclic w/central clearing, and psoriasiform
in photodistribution
sudden onset
lasts weeks to months
limited organ involvement
risk of SLE < 50% & usually milder
ANA + 60-80%
Drugs assoc w/ SCLE
beta blockers, CCB, ACE inhibitors, thiazide diuretics, hydralazine, isoniazid, procainamide, terbinafine, statins, tamoxifen, PPIs, NSAIDS, minoxycline, and anti-TNF agents
lupus mgmt
Topical - class 2-3 TCS, TCI, retinoids
Systemic - hydroxychloroquine (dec flares, risk of thromboembolism, time to SLE; dec efficacy in smokers)
Alt - DMARDs, slow prednisone taper
severe - CsA, tacrolimus, belimumab or rituximab
discoid lupus
AA, female, > 40 y.o.
exacerbated by uv & trauma
thick, dark, scaly, coin-shaped plaques
Atrophy, hypo- or hyperpigmentation, telangiectasia, usually scarring
tx: intralesional/higher potency steroids, immunomodulators, antimalarials
pediatric/neonatal SLE
transplacental autoantibodies
Polycyclic, annual or bullous rash (scalp, periorbital)
Owl eyes
photosensitivity
chilblain lupus/perniosis
(not lupus pernio)
Recurrent tender, itchy, erythematous papules/nodules
on acral sites like ears, nose, fingers and toes
d/t exposure to cold
neg. cryoglobulins/cold agglutinins
tx: avoid cold & tight clothing
TCS, prednisone, pentoxifylline, hydroxychloroquine, calcium channel blockers, mycophenolate, nicotinamide
lupus pernio
misnomer
actually cutaneous sarcoidosis
Purple, “apple jelly”, or urticarial papules & nodules
Acral locations (If nose or mouth, must evaluate pulmonary)
Spontaneous remission for most
tx: topical/intralesional steroids
antimalarials
Hydroxychloroquine, chloroquine, & quinacrine
4-6 wks onset
increases digoxin, CsA, penicillamine
decreases botulinum toxin
increased by cimetidine
may exacerbate psoriasis
Bluish-gray to black hyperpigmentation 10-30% pt tx for >3 mos (shins, face,palate, nails)- reversible
Corneal deposits (reversible)
retinopathy (irreversible, >1000g cumulative, baseline exam, annual after 5 yrs)
contraindications: myasthenia gravis; G6PD insufficiency (hemolysis unlikely at therapeutic dose so screening not necessary)
systemic scleroderma
Sclerosis of visceral organs, connective tissue, and skin
Possibly immune disruption triggered by infections, hormonal changes or environment
collagen deposition & fibrotic changes
Vasospasms, vasoconstriction and hardening of arteries
Genetic predisposition but not inheritable
May have CREST (60%)
rapidly progressive in blacks and females
Circumscribed/diffuse, hardening, fixed plaques
Abrupt, diffuse, stiffness/edema hands/feet (Raynaud’s)
Lipoatrophy (bird-face)
Can have digital ulcers & hair loss
Edema and pruritus often precede sclerosis
Salt & Pepper skin (hyper- or depigmentation)
Arthritis & tendonitis
CBC w/diff, creatinine, creatinine kinase, UA w/microscopy
ANA panel incl. anti-Scl-70 (high anti-Scl-70 & anti-RNA have >95% specificity but 20-50 sensitivity)
Managed by rheumatology
Diffuse - 50% 5yr survival
morphea
localized scleroderma
Circumscribed ivory colored
Sclerotic fixed plaques or violaceous border
Linear- extremities, forehead
Face (en coup de sabre)
Perry-Romberg- facial hemi-atrophy, leaves hyperpigmentation>atrophy to SQ/bone
Morphea/Lichen sclerosus et atrophicus overlap
tx: may burn out, TCS, topical retinoids, calcipotriene, nbUVB, Mtx, MMF, hydroxychloroquine, infliximab
