Sclerosis and SLE Flashcards
What are the 3 types Systemic Slerosis
- Diffuse Cutaneous Systemic Sclerosis
- CREST (Limited cutaneous systemic sclerosis)
- Systemic sclerosis sine scleroderma
The cutaneous fibrosis involvement in CREST mainly involves which area?
Hands Feet Face/Neck NO TRUNCAL INVOLVEMENT Does not beyond elbows and knees
How do you differentiate Secondary Raynauds due to Diffue systemic sclerosis from primary Raynaud’s?
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
What are the GI manifestations of Diffuse Sclerosis
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
What is the mcc of mortality in systemic sclerosis?
Pulmonary involvement
- ILD
- Pulmonary HTN
Does steroids improve renal crisis in systemic sclerosis?
No steroids increase risk of renal crisis
What are the antibodies seen in diffuse systemic sclerosis
Antitopoisomerase (Anti-Scl-70)
Anti-U3- RNP (fibrillarin)
RNA polymerase III Ab
What are the antibodies seen in CREST?
Anti-Centromere
Anti-Th/To antibody
Which antibodies increase the risk of Pulmonary HTN in systemic sclerosis and which one increase risk for ILD?
Pulm HTN= Anti-Centromere,Anti-Th/To antibody, Anti-U3- RNP (fibrillarin)
ILD=Antitopoisomerase (Anti-Scl-70)
RNA polymerase III Ab is associated with what in sclerosis?
Renal Crisis
malignancies (Lung and Breast)
Scleroderma is associated with increase frequency of what lung cancer?
Bronchogenic carcinoma
What are the SCLICC and ACR/EULAR criteria forDx Lupus?
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
What antibody do we typically see in subacute cutaneous lupus?
Anti-RO/Anti-La
How does the arthritis of SLE present?
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
What are the drugs approved for the management of SLE?
Aspirin
Hydroxychloroquine
Steroid
Belimumab
What is the most sensitive screening test for renal involvement in SLE?
Urinalysis with Micro looking for proteinuria, dysnorphic RBCs
These are seen prior to elevation in the creatinine
How does acute lupus pneumonitis present?
Acute onset fever, SOB, Cough, Pleuritic chest pain that mimicks PNA
CXR Bilateral patchy infiltrates
During flares which SLE antibodies increase?
anti-dsDNA
Which SLE antibody is seen with nephritis and CNS diease?
Anti-Smith
Anti-Ro (SSA)
Anti-La (anti-SSB)
is seen with what autoimmune diseases?
Subacute cutaneous lupus
Sjogrens
Neonatal lupus and Congenital heart blocks
What antibody is seen in Mixed connective tissue disease and how does it present?
Anti-RNP
Features of :
- SLE
- Polymyositis
- Scleroderma
What drugs can lead to Drug-Induced Lupus and what organs are usually spared?
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
Which Ab do we see in Drug-Induced Lupus?
Anti-Histone
Management of SLE in pregnancy?
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
Women with SLE are at high risk for what vaginal disease?
Cervical dysplasia and should be vaccinated with HPV
What are the causes of death in SLE?
First 10 year=Renal involment then Infection
After 10 years=CVD
EArly treament with hydroxychloroquine in SLE have what benefits?
- Prevent disease flares
- Decrease organ damage
- Facilitate response to mycophenolate in renal lupus
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
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
- Lupus anti-coagulation 2 or more ocassion at least 12 weeks apart
- Beta 2 Glycoprotein 2 or more ocassion at least 12 weeks apart
Dx is made with at least 1 clinical and 1 lab criterion
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
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
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
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
Sjogren is confirmed with what?
Lip salivary gland biopsy
How is Sjogren with salivary, cutaneous and organt manifestation managed?
Steroid
Hydroxychoroquine
MTX
Rituximab
What are the clues to lymphoma in a patient with Sjogrens
Persistent parotid enlargement Leukopenia Pupura Cryoglobulinemia Low C4
What kind of lymphoma do we see in Sjogren?
Low grade marginal zone B cell (MALT) lymphomas
