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76 Cards in this Set

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What are the 4 levels of kidney lesions in SLE?
1. Mesangial glomerulonephritis
2. Focal proliferative " "
2. Diffuse proliferative " "
4. Membranous glomerulonephritis
Why is diffuse proliferative glomerulonephritis the most serious of the SLE kidney lesions?
Because it is associated with
-Hematuria
-Proteinuria
-Hypertension
and Renal Failure.
What findings appear on urinalysis as a result of Diffuse proliferative glomerulonephritis in SLE?
-RBC casts
-Waxy casts
-Cellular casts
What is deposited in SLE that causes glomerulonephritis?
IgG immune complexes
What is the pathologic finding in joints in SLE?
Nonerosive synovitis without deformity
What pathology can be found in the CNS in SLE?
Intimal proliferation causing inflammatory occlusion of small vessels
What can the small vessel occlusion in the CNS lead to?
Headaches and cognitive dysfunction.
What are common findings in the lungs and heart in SLE patients?
Lungs: pleuritis and pneumonitis
Heart: pericarditis and CAD
What can be found in the lymph nodes in SLE?
Lymphadenopathy due to follicular hyperplasia
How often are cardiac vegetations seen in SLE cases? Why?
Rarely - because now steroids are used to treat SLE and prevent such heart valve disease.
What is drug-induced lupus?
The case where the ANA test is positive because of certain medications.
What are 4 medicines that induce lupus erythematosus?
1. Hydralazine
2. Procainamide
3. Penicillamine
4. Isoniazide
Do patients with drug induced LE have symptoms of SLE?
no
What is the characteristic antibody seen in drug-induced LE?
Anti-histone
What is the triad in Felty's syndrome?
-Rheumatoid arthritis
-Splenomegaly
-Neutropenia
What is the cause of the neutropenia in Felty's syndrome?
An accompanying lymphocytosis of clonal Tcells.
What is the pathologic mechanism in Sjogren's syndrome?
Immunologic destruction of the lacrimal glands and salivary glands.
How does Sjogren's syndrome often present?
In conjunction with another autimmune disorder.
What is it called when Sjogren's syndrome presents by itself?
Sicca syndrome
What are the 2 main symptoms of sjogren's syndrome?
Dry eyes
Dry mouth
What age/sex are 90% of patients with Sjogren's syndrome?
Females between 40-50 yr old
What are the initial positive tests that suggest Sjogren's?
-Pos ANA
-Pos Anti-RF
What are the more specific antibody tests that are positive in Sjogren's?
Anti-SSA (ro)
Anti-SSB (la)
What is a high titer SS-A associated with?
Systemic dissemination of the disease
What genes predispose patients to Sjogren's syndrome?
HLA alleles B8, DR3, DQB1, DQA1
What is the featured morphology of Sjogren's syndrome?
Periductal and Perivascular infiltration of salivary glands with lymphocytes
What cells often initiate the autoimmune destruction of the lacrimal and salivary glands?
Thelper cells
When Sjogren's syndrome becomes systemic, what 4 tissues are usually involved?
-CNS
-Skin
-Kidneys
-Lungs
Where will renal lesions primarily be seen if they arise in Sjogren's syndrome?
In the TUBULES
-Tubulointerstitial nephritis

(as opposed to the glomerulus as in SLE)
What is lymph node enlargement in Sjogren's syndrome due to?
Polyclonal Bcell activation
What are patients with Sjogren's syndrome at a 40X higher risk of developing?
Bcell lymphoma
What is the etiology of Systemic Sclerosis?
It is unknown
What is Systemic Sclerosis characterized by?
Abnormal accumulations of Fibrous Tissue in the Skin and Multiple organs
What organ is most commonly affected by systemic sclerosis?
The skin
What are 4 common causes of death in patients that die from Systemic Sclerosis?
-Heart failure
-Kidney failure
-pulmonary insufficiency
-Intestinal malabsorption (starve)
What is the female:male ratio of Systemic sclerosis?
3:1
What precedes all other symptoms in 70% of systemic sclerosis cases?
Raynaud's phenomenon
What is the cause of intestinal malabsorption?
-Dysphagia
-Gastroesophageal reflux
-Esophageal hypomotility
What is the cause of pulmonary insufficiency?
Pulmonary hypertension and fibrosis
What is the cause of Cardiac failure?
Myocardial fibrosis
What is the cause of Renal failure?
Hypertension
What are 2 different flavors of systemic sclerosis?
1. Diffuse Scleroderma
2. Limited Scleroderma
How can Diffuse scleroderma be identified?
1. There will be WIDESPREAD SKIN INVOLVEMENT at the disease ONSET
2. Rapidly progresses and involves visceral organs early.
How can Limited scleroderma be identified?
1. limited skin involvement
2. LATE visceral involvement
What do some patients with limited scleroderma have?
CREST syndrome
What does CREST stand for?
-Calcinosis
-Raynaud's phenomenon
-Esophageal dysmotility
-Sclerodactyly
-Telangiectasia
What antibody is characteristic of CREST & limited scleroderma?
Anti-centromere
What antibody is characteristic of disseminated scleroderma and systemic sclerosis?
anti-Scl 70
What causes the CD4+ Tcells to develop an immune reaction in systemic sclerosis?
An UNIDENTIFIED ANTIGEN
What inflammatory mediators are part of the SS pathology?
IL1, IL2, TNF, PDGF, and TGF-beta
What do we know TGF-beta does?
Stimulates fibrosis and collagen deposition, which are the pathologic causes of Systemic sclerosis.
How does microvascular injury develop in systemic sclerosis?
Intimal fibrosis/capillary dilation -> widespread vascular narrowing/ischemic injury.
What is anti-DNA topoisomerase I?
Anti Scl-70
What % of systemic sclerosis patients have a positive Anti-Scl 70?
70%
What is the Anti-Scl 70 specifically associated with? (symptoms)
-Pulmonary fibrosis
-Peripheral vascular disease
(so probably not limited)
What antibody is associated with scleroderma? In what percent of patients?
Anti-centromere Ab
-In 36% of patients
What percent of patients with CREST syndrome have a positive Anti-centromere?
96%
What does subcutaneous fibrosis do to the fingers in systemic sclerosis?
Causes flexion deformity
What causes cutaneous ulcerations?
Loss of blood supply
What is "rubber hose" esophagus?
Thickening of the epithelium in the GI tract in systemic sclerosis
What does rubber hose esophagus cause in 80% of pateints?
GERD and strictures
What causes the hypertension in the kidneys in systemic sclerosis?
Thickening of the interlobular arteries
What is the MAJOR CAUSE OF DEATH IN SYSTEMIC SCLEROSIS?
Interstitial fibrosis and pulmonary hypertension cause pulmonary insufficiency and suffocation.
What is the treatment for early disseminated Systemic sclerosis?
Immunosuppressive agents
What is used for treating Raynaud phenomenon?
Vasodilators
What are 2 specific therapies for SS?
-Anti-TNFalpha
-Anti-TGFbeta
What are the 2 inflammatory myopathy diseases?
-Dermatomyositis
-Polymyositis
In what patients is Dermatomyositis seen?
Adults and children
What is the presentation of dermatomyositis?
-Distinct skin discoloration
-Symmetric, proximal muscle weakness
What does Dermatomyositis put patients at a 40% increased risk of developing?
Visceral cancers
What is the pathology of dermatomyositis?
1. Antibodies and complement attack capillaries
2. Ischemia to muscles causes necrosis and atrophy
How is polymyositis distinguished from dermatomyositis?
There are no skin lesions and it mostly occurs in adults.
What is the pathology of polymyositis?
CTL mediated cell injury
What is mixed connective tissue disease?
A mishmash of all the systemic autoimmune diseases.
What antibody suggests MCTD?
Anti-U1-RNP
What is RARELY found in MCTD?
Renal involvement