8- Connective Tissue Diseases Flashcards by Jenica Dela Cruz

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

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[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

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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)

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Drug induced SCLE is most often related to what drug

Hydrochlorothiazide

Also: ACE inhibitors, CCB, interferon, anticonvulsant

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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

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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

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Difference between bullous lesions of lupus erythematosus (BLE) and epidermolysis bullosa acquisita

Dapsone effective in BLE and not in EBA

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Earliest changes that may be noted in SLE

May remain the sole symptom for some time

Transitory or migratory arthralgia

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Most common cause of death in SLE DURING the first 5 years

Inflammatory lesions and infections

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Most common cause of death in SLE AFTER the first 5 years

Thromboses

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Most frequent cardiac manifestation in SLE

Pericarditis

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This sign is a marker for SLE patients at risk for CNS lesions (Sneddon Syndrome)

Livedo reticularis

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Implicated causes of drig induced LE

HIP SPAM

Hydralazine
Isoniazid
Procainamide

Sulfonamides
Penicillin
Anticonvulsants
Minocycline

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This test is positive in 95% of cases of SLE

ANA

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This is specific but not sensitive to SLE

Indicates high risk of renal disease

Anti-dsDNA

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Immunologic test for SLE that has 10% sensitivity but specificity is very high

Anti-Sm

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Immunologic test that is common in SCLE and Sjögren

Anti-La

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(Immunologic test)

More commonly positive in SCLE, neonatal LE, late onset and Asian LE

Anti-Ro

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Single most effective local treatment in SLE

Triamcinolone acetonide 2.5-10mg/mL at 4-6 weeks interval

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Safest class of systemic agent for SLE

Antimalarials

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Drug of choice for bullous systemic LE

Dapsone

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First line systemic therapy in most forms of cutaneous LE

Hydroxychloroquine 6.5mg/kg/day

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WOF this side effect of hydroxychloroquine

Ocular toxicity

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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