8- Connective Tissue Diseases Flashcards
Young adults
Dull red macules/indurated plaques with adherent scale—evolve with atrophy, scarring, pigment changes
Usually above the neck (scalp, nose, malar, lower lip ears)
Generalized- thorax, upper extremities, head and neck, may have alopecia
95% will remain confined to skin
Discoid LE
[Chronic cutaneous LE]
Large lesions, atrophic, hypopigmented, red or pink patches and plaques
Fine telangiectasia and scaling
Extensor/midline back usually affected
Prominent palmoplantar involvement
Lupus erythematosus-lichen planus overlap
Evolve as polycyclic annular lesions or psoriasiform plaques
Scale is thin and easily detached
Follicles not involved
Transient/migratory; no scarring
Tend to occur in sun exposed areas-face and neck, chest and back
3/4 have arthritis (+) anti Ro/SSA
Subacute cutaneous LE (SCLE)
Drug induced SCLE is most often related to what drug
Hydrochlorothiazide
Also: ACE inhibitors, CCB, interferon, anticonvulsant
SLE must have how many of the criteria to diagnose
4/11
At least 1 clinical and 1 immunologic
or must have biopsy proven lupus nephritis in the presence of ANA or anti-dsDNA antibodies
SLE criteria
SOAP BRAIN MD
Serositis (pleuritis/pericarditis)
Oral ulcers
Arthritis
Photosensitivity
Blood low (leuko, thrombo, anemia) Renal (protein >0.5g/day or casts) ANA positive Immunologic- dsDNA, anti-Sm Neurologic- seizures, psychosis
Malar rash
Discoid rash
Difference between bullous lesions of lupus erythematosus (BLE) and epidermolysis bullosa acquisita
Dapsone effective in BLE and not in EBA
Earliest changes that may be noted in SLE
May remain the sole symptom for some time
Transitory or migratory arthralgia
Most common cause of death in SLE DURING the first 5 years
Inflammatory lesions and infections
Most common cause of death in SLE AFTER the first 5 years
Thromboses
Most frequent cardiac manifestation in SLE
Pericarditis
This sign is a marker for SLE patients at risk for CNS lesions (Sneddon Syndrome)
Livedo reticularis
Implicated causes of drig induced LE
HIP SPAM
Hydralazine
Isoniazid
Procainamide
Sulfonamides
Penicillin
Anticonvulsants
Minocycline
This test is positive in 95% of cases of SLE
ANA
This is specific but not sensitive to SLE
Indicates high risk of renal disease
Anti-dsDNA
Immunologic test for SLE that has 10% sensitivity but specificity is very high
Anti-Sm
Immunologic test that is common in SCLE and Sjögren
Anti-La
(Immunologic test)
More commonly positive in SCLE, neonatal LE, late onset and Asian LE
Anti-Ro
Single most effective local treatment in SLE
Triamcinolone acetonide 2.5-10mg/mL at 4-6 weeks interval
Safest class of systemic agent for SLE
Antimalarials
Drug of choice for bullous systemic LE
Dapsone
First line systemic therapy in most forms of cutaneous LE
Hydroxychloroquine 6.5mg/kg/day
WOF this side effect of hydroxychloroquine
Ocular toxicity
When should Ophthalmology consult be done when administering hydroxychloroquine?
Before
After 4mos
After 6mos
Side effect of Quinacrine in SLE tx
Yellow discoloration-skin and conjunctivae
Blue black discoloration- hard palate, nail bed, ears, sclerae
Second line and third line agents for SLE
Retinoids
Systemic immunosuppresion
Inflammatory myositis and skin disease
Erythema and edema of face and eyelids, upper face—evolve to reticulated white scarring
Extensor surfaces may be similar to psoriasis
Photosensitivity
Dermatomyositis
Muscle group involved in dermatomyositis
Proximal muscle groups
Erythema and scale over shoulder area in dermatomyositis is called
Shawl sign
Erythema and scale over hip area in dermatomyositis is called
Holster sign
Less common lesions but highly characteristic of dermatomyositis
Gottron’s papules
In dermatomyositis, there is symmetrical muscle weakness, most commonly seen in
Shoulder girdle and pelvic region
Hands
Antibody positive in dermatomyositis
Anti-Jo-1
Diagnostic criteria for dermatomyositis must include:
How many
1 skin lesion + 4/8 other criteria
Polymyositis- 4/8 criteria, no skin lesions
Criteria for diagnosis of dermatomyositis
Skin lesions- heliotrope rash, gottrons papules
Other: 4/8 Proximal muscle weakness Elevated CK/ aldolase Muscle pain EMG changes Positive anti-Jo-1 Arthritis Systemic signs (fever, elevated CRP/ESR) Patho findings of myositis
Mainstay of acute treatment for dermatomyositis
Prednisone 1mg/kg/day
Circumscribed or diffuse, hard, smooth, ivory colored areas that are immobile and give appearance of hidebound skin
Face expressionless, mouth constricted, claw like hands
Scleroderma
This form of scleroderma is more common in women
Macules or plaques, bands or guttate lesions
Rosy or violaceous — yellowish white
More common on trunk, margins surrounded by lilac border or telangiectases- skin elasticity lost
Morphea
Linear lesions that extend the length of the arm or leg and may follow lines of Blaschko
Begins during 1st decade of life
Linear scleroderma
Lesion in scleroderma that occur on the frontal scalp and extend partly down the forehead
En coup de sabre
Variant of scleroderma that has the most favorable prognosis
CREST syndrome
CREST syndrome acronym meaning
Calcinosis cutis Raynaud phenomenon Eesophageal dysmotility Sclerodactyly Telangiectasia
First manifestation of progressive systemic sclerosis
Raynaud phenomenon
Generalized disorder of connective tissue- thickening of dermal collagen bundles, fibrosis and vascular abnormalities in internal organs
“Woody edema” of the hands
3rd-5th decade
Progressive systemic sclerosis (PSS)
Criteria for progressive systemic sclerosis
Proximal sclerosis and 2 or all of ff:
- sclerodactyly
- digital pitting scars/ loss of substance of distal finger pad
- bilateral basilar pulmonary fibrosis
90% of PSS patients have this symptom
Esophageal involvement
-distal 2/3: dysphagia and reflux esophagitis
Mainstay of medical treatment for Raynaud phenomenon
Vasodilators- CCB, ARB, topical nitrates and prostanoids
First line therapy for Raynaud phenomenon
Nifedipine 30-60mg/day
Defined by presence of: Raynaud phenomenon Arthralgia Swollen joints Esophageal dysfunction Muscle weakness Sausage like fingers
Mixed Connective Tissue Disease
MCTD
Antibodies present in MCTD
High anti RNP
Absent anti-Sm
ANA generally persists through periods of remission
Acute treatment for MCTD
Prednisone 1mg/kg/day
Chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands (salivary and lacrimal)
Sjögren syndrome
Vasculitis
Xerosis
Pruritus
Annular erythema
Decreased sweating
Sjögren syndrome
Most definitive test for Sjögren syndrome
Labial salivary gland biopsy
Classic criteria for Sjögren syndrome
2/3
Xerophthalmia
Xerostomia
Associated autoimmune, rheumatic or lymhoproliferative disorder
Antibodies present in Sjögren syndrome
80% anti-Ro/SSA
40% anti-La/SSB
Patients with Sjögren syndrome are predisposed to the development of this malignancy
Lymphoreticular- Non Hodgkin B cell lymphoma
Pharma agents for Sjögren that help stimulate salivation
Pilocarpine
Cevimeline
