What disease is associated with
- Autoimmune disorder of Multiple organ systems affected
- Mainly in young black women
- Spontaneous relapses and remissions
- Many symptoms are from trapping of antigen-antibody complexes in capillaries or to antibody destruction of host cells (platelets)
Systemic Lupus Erythematosus
Popcorn term for what disease?
- FATIGUE
- Butterfly rash”
- Photosensitivity
- Livedo reticularis
- Panniculitis
- Discoid plaques
- Periungual erythema
- Nail fold infarcts
- Alopecia
Systemic Lupus Erythematosus
What disease is diagnosed with 4/11 of the following?
Malar rash, Discoid rash, Photosensitivity, Oral ulcers, Arthritis
Serositis (pleuritis or pericarditis)
Kidney disease
Neurologic disease (Seizures, or Psychosis)
+ ANA, antiphospholipid antibodies based on (1) IgG or IgM anticardiolipin antibodies
Systemic Lupus Erythematosus
False positive Antiphosepholipid Syphilis
What other immune tests can be used to DIAG SLE besides +ANA?
Anti-dsDNA** (high specificity and sensitivity)
Anti-Sm (high specificity)
What disease is treated with
- Sun protection, exercise
- hydroxycholorquine
- Remember eye exams
- NSAIDS – prn for flares
- Corticosteroids – prn for flares
- methotrexate, azathioprine, Resistant to treatment: belimumab
Systemic Lupus Erythematosus
30% of patients with SLE are allergic to ________drugs
sulfonamide
Discoid lupus are inflammation and ______ Lesions on the _______.
Scarring
Face
What are the main drugs that cause a Drug-induced Lupus attack?
Think MD PHI
- Minocycline
- Diltiazem
- Procainamide
- Hydralazine
- Isoniazid
What antibodies cross the placenta in SLE?
Anti SSA and Anti SSB
What disease is associated with
- Recurrent pregnancy loss in the first trimester
- DVT
- Pulmonary emboli
- CVAs
- Budd-Chiari syndrome
- Cerebral vein thrombosis
- Myocardial or digital infarctions
Antiphospholipid Antibody Syndrome
What disease is diagnosed with
1 Clot + A positive antibody test
Anti-cardiolipin antibodies (false-positive test for syphilis)
Antibodies to beta-2-glycoprotein
Lupus anticoagulant antibody
Antiphospholipid Antibody Syndrome (APS)
What disease is treated with
- Anticoagulation for life (warfarin)
- Teratogenic so give Subcutaneous heparin
- Low-dose aspirin
- IV heparin
- High dose corticosteroids
- IVIG
- Plasmapheresis
Antiphospholipid Antibody Syndrome
What disease has paroxysmal digital ischemia.
- Initial phase: excessive vasoconstriction
- Recovery phase: vasodilation
- Triggered by emotional stress, cold
Raynaud Phenomenon
What does Raynaud’s look like?
Think ischemic digital
- well demarcated digital pallor or cyanosis
- Numbness, stiffness, pain decreased sensation
- Recovery phase: intense hyperemia and rubor
- Intense throbbing, paresthesia, pain
- Primarily affects fingers
What ischemic finger disease is treated with wear gloves, stop smoking, and heat the body?
* Avoid Sympathomimetics (decongestants, diet pills, amphetamines)
Raynaud Phenomenon
What disease is treated with
Calcium channel blockers - first line
Nifedipine, amlodipine, felodipine
Other options: PDE-5 inhibitor (sildenafil), topical nitroglycerin, prazosin, losartan, prostaglandin
OR a Sympathectomy?
Raynaud Phenomenon
What disease has diffused collagen deposits on the SKIN and other organs?
AND
Has the CREST syndrome?
Scleroderma
CREST
- Calcinosis cutis
- Raynaud phenomenon
- Esophageal dysfunction
- Sclerodactyly
- Telangiectasias
T/F can you Diagnose Scleroderma with
+ ANA in 90% of patients with diffuse scleroderma
+ anti-centromere antibody in CREST syndrome
+ anti-SCL-70 antibody in diffuse disease; indicates poorer prognosis
True
Is there a cure for Scleroderma?
No cure but you can monitor HTN, PPI (reflux), ACE, CCB, or immunospuressants.
What disease has Inflammed Striated Muscle, Muscle weakness, and is associated with Malignancy (>50 Paraneoplastic Syndrome)?
Polymyositis & Dermatomyositis
What is the Skin Rash in Dermatomyositis?
(HMS Gottron’s)
Her Majesty’s Ship - Gottron’s
Polymyositis & Dermatomyositis
How can you Diagnose and Treat Polymyositis & Dermatomyositis?
(think Striated muscle disease)
Diag: Muscle Biopsy
CK, Aldolase, MRI
Tx: HIGH DOSE STEROID, DMARDS
What disease was called SICCA syndrome
Is a Connective tissue disorder where auto antibodies destroy salivary and lacrimal glands?
Sjӧgren Syndrome
This disease presents with
- Dry mouth (xerostomia)
- Dry eyes (xerophthalmia or keratoconjunctivitis sicca)
- Enlarged parotid glands
And is Diagnosed with the Schirmer Test
Sjӧgren Syndrome
