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Sclerosis and SLE Flashcards

1
Q

What are the 3 types Systemic Slerosis

A
  • Diffuse Cutaneous Systemic Sclerosis
  • CREST (Limited cutaneous systemic sclerosis)
  • Systemic sclerosis sine scleroderma
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2
Q

The cutaneous fibrosis involvement in CREST mainly involves which area?

A 
Hands
Feet
Face/Neck
NO TRUNCAL INVOLVEMENT
Does not beyond elbows and knees
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3
Q

How do you differentiate Secondary Raynauds due to Diffue systemic sclerosis from primary Raynaud’s?

A

Check the nail fold capillaries- in diffuse systemic sclerosis have a decrease number of capillaries, capillaries with giant loops). In primary Raynaud’s disease there are no abnormal nail fold capillaries.

The abnormal nail fold capillaries correlates with the severity of scleroderma

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4
Q

What are the GI manifestations of Diffuse Sclerosis

A

This the mos common manifestations
-Esophageal dysmotility: Absent peristalsis and decrease LES pressure–>GERD=Rx Reglan, PPI

  • Gastric ectasia=Can lead to massive GI bleed; Rx is Argon laser photocoagulation during EGD
  • SBO leading to diarrhea and malabsorption
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5
Q

What is the mcc of mortality in systemic sclerosis?

A

Pulmonary involvement

  • ILD
  • Pulmonary HTN
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6
Q

Does steroids improve renal crisis in systemic sclerosis?

A

No steroids increase risk of renal crisis

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7
Q

What are the antibodies seen in diffuse systemic sclerosis

A

Antitopoisomerase (Anti-Scl-70)
Anti-U3- RNP (fibrillarin)
RNA polymerase III Ab

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8
Q

What are the antibodies seen in CREST?

A

Anti-Centromere

Anti-Th/To antibody

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9
Q

Which antibodies increase the risk of Pulmonary HTN in systemic sclerosis and which one increase risk for ILD?

A

Pulm HTN= Anti-Centromere,Anti-Th/To antibody, Anti-U3- RNP (fibrillarin)

ILD=Antitopoisomerase (Anti-Scl-70)

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10
Q

RNA polymerase III Ab is associated with what in sclerosis?

A

Renal Crisis

malignancies (Lung and Breast)

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11
Q

Scleroderma is associated with increase frequency of what lung cancer?

A

Bronchogenic carcinoma

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12
Q

What are the SCLICC and ACR/EULAR criteria forDx Lupus?

A

SLICC= 4/17 criteria with at least 1/11 clinical and 1/6 immunologial criteria OR B proven nephritis with positive ANA or ds DNA

ACR/EULAR= ANA> 1:80 plus 7 clinical and 3 immunological criteria

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13
Q

What antibody do we typically see in subacute cutaneous lupus?

A

Anti-RO/Anti-La

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14
Q

How does the arthritis of SLE present?

A

Its a polyarticular non-erosive arthritis
Can lead to a reversible deformity of PIP and MCP joint (Jaccoud’s arthritis). This is non-erosive and reducible because the tendon is involved and not the joint

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15
Q

What are the drugs approved for the management of SLE?

A

Aspirin
Hydroxychloroquine
Steroid
Belimumab

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16
Q

What is the most sensitive screening test for renal involvement in SLE?

A

Urinalysis with Micro looking for proteinuria, dysnorphic RBCs

These are seen prior to elevation in the creatinine

17
Q

How does acute lupus pneumonitis present?

A

Acute onset fever, SOB, Cough, Pleuritic chest pain that mimicks PNA

CXR Bilateral patchy infiltrates

18
Q

During flares which SLE antibodies increase?

A

anti-dsDNA

19
Q

Which SLE antibody is seen with nephritis and CNS diease?

A

Anti-Smith

20
Q

Anti-Ro (SSA)
Anti-La (anti-SSB)
is seen with what autoimmune diseases?

A

Subacute cutaneous lupus
Sjogrens
Neonatal lupus and Congenital heart blocks

21
Q

What antibody is seen in Mixed connective tissue disease and how does it present?

A

Anti-RNP

Features of :

  • SLE
  • Polymyositis
  • Scleroderma
22
Q

What drugs can lead to Drug-Induced Lupus and what organs are usually spared?

A

M CHIP

Methyldopa
Chlorpromazine
Hydralazine
INH
Procainamide
Quinidine
Penicillamine
PTU
Minocycline 
Anti-TNF agents (can be associated with anti-dsDNA)

Renal and CNS are usually spared

23
Q

Which Ab do we see in Drug-Induced Lupus?

A

Anti-Histone

24
Q

Management of SLE in pregnancy?

A

There is an increase risk of preeclampsia

RX: Prednisone, Hydroxychlorowuine, and Azathioprine

Women with recurrent spontaneous abortion in early pregnancy or 1 late pregnancy loss and antiphospholipid syndrome are treated with heparin and aspirin

If patients are Antiphospholipid negative OCP is safe to use in women with SLE

25
Women with SLE are at high risk for what vaginal disease?
Cervical dysplasia and should be vaccinated with HPV
26
What are the causes of death in SLE?
First 10 year=Renal involment then Infection After 10 years=CVD
27
EArly treament with hydroxychloroquine in SLE have what benefits?
1. Prevent disease flares 2. Decrease organ damage 3. Facilitate response to mycophenolate in renal lupus
28
What are the clinical features of Anti-Phospholipid syndrome?
BOTH Arterial (TIA, migraine, stroke, recurrent abortions) AND Venous (DVT, cerebral, or retinal vein thrombosis) thrombosis
29
What are the clinical and laboratory criteria of antiphospholipid and how is Dx?
Clinical: - Vascular thrombosis - Complication of pregnancy Laboratory Criteria: 1.Anti-cardiolipin Ab (IgG or IgM) on 2 or more ocassion at least 12 weeks apart 2. Lupus anti-coagulation 2 or more ocassion at least 12 weeks apart 3. Beta 2 Glycoprotein 2 or more ocassion at least 12 weeks apart Dx is made with at least 1 clinical and 1 lab criterion
30
What are the Complication of pregnancy of anti-phospholipid syndrome?
- One or more unexplained death of normal fetus at or after 10th week gestation - One or more premature births of morpholigically neonates at or before the 34th week of gestation - 3+ unexplained consecutive spontaneous abortions before 10th week or gestation
31
How is anti-phospholipid syndrome treated?
``` APLA with thrombosis; Arterial:Warfarin +/- Aspirin Venous: Warfarin (newer agents not used) INR goal is 2-3 ``` APLA w/o thrombosis=no treatment
32
What is catastrophic anti-phospholipid syndrome and how is it treated?
Acute onset thrombosis in at least 3 organs within 1 week + Microthrombosis It has a very high mortality Rx is high dose heparin, steroid, IV Ig/ plasmapharesis
33
What is eosinophilic fasciitis?
Inflammation followed by sclerosis of the dermis, subcutis, and deep fascia usually involving the forearm. Trigger is usually after strenuous physicial activity Patients get abrupt onset of symmetric tenderness and swelling of the extremities followed by induration leading to puckering of the skin. It spares the face and hands. eosinophilia is found early in the disease It is diagnosed with full thickness skin biopsy to the level of the fascia, treatment is with prednisone
34
Sjogren is confirmed with what?
Lip salivary gland biopsy
35
How is Sjogren with salivary, cutaneous and organt manifestation managed?
Steroid Hydroxychoroquine MTX Rituximab
36
What are the clues to lymphoma in a patient with Sjogrens
``` Persistent parotid enlargement Leukopenia Pupura Cryoglobulinemia Low C4 ```
37
What kind of lymphoma do we see in Sjogren?
Low grade marginal zone B cell (MALT) lymphomas

Rheumatology (4 decks)

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