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76 Cards in this Set
- Front
- Back
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What are the 4 levels of kidney lesions in SLE?
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1. Mesangial glomerulonephritis
2. Focal proliferative " " 2. Diffuse proliferative " " 4. Membranous glomerulonephritis |
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Why is diffuse proliferative glomerulonephritis the most serious of the SLE kidney lesions?
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Because it is associated with
-Hematuria -Proteinuria -Hypertension and Renal Failure. |
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What findings appear on urinalysis as a result of Diffuse proliferative glomerulonephritis in SLE?
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-RBC casts
-Waxy casts -Cellular casts |
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What is deposited in SLE that causes glomerulonephritis?
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IgG immune complexes
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What is the pathologic finding in joints in SLE?
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Nonerosive synovitis without deformity
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What pathology can be found in the CNS in SLE?
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Intimal proliferation causing inflammatory occlusion of small vessels
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What can the small vessel occlusion in the CNS lead to?
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Headaches and cognitive dysfunction.
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What are common findings in the lungs and heart in SLE patients?
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Lungs: pleuritis and pneumonitis
Heart: pericarditis and CAD |
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What can be found in the lymph nodes in SLE?
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Lymphadenopathy due to follicular hyperplasia
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How often are cardiac vegetations seen in SLE cases? Why?
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Rarely - because now steroids are used to treat SLE and prevent such heart valve disease.
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What is drug-induced lupus?
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The case where the ANA test is positive because of certain medications.
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What are 4 medicines that induce lupus erythematosus?
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1. Hydralazine
2. Procainamide 3. Penicillamine 4. Isoniazide |
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Do patients with drug induced LE have symptoms of SLE?
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no
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What is the characteristic antibody seen in drug-induced LE?
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Anti-histone
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What is the triad in Felty's syndrome?
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-Rheumatoid arthritis
-Splenomegaly -Neutropenia |
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What is the cause of the neutropenia in Felty's syndrome?
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An accompanying lymphocytosis of clonal Tcells.
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What is the pathologic mechanism in Sjogren's syndrome?
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Immunologic destruction of the lacrimal glands and salivary glands.
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How does Sjogren's syndrome often present?
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In conjunction with another autimmune disorder.
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What is it called when Sjogren's syndrome presents by itself?
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Sicca syndrome
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What are the 2 main symptoms of sjogren's syndrome?
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Dry eyes
Dry mouth |
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What age/sex are 90% of patients with Sjogren's syndrome?
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Females between 40-50 yr old
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What are the initial positive tests that suggest Sjogren's?
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-Pos ANA
-Pos Anti-RF |
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What are the more specific antibody tests that are positive in Sjogren's?
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Anti-SSA (ro)
Anti-SSB (la) |
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What is a high titer SS-A associated with?
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Systemic dissemination of the disease
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What genes predispose patients to Sjogren's syndrome?
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HLA alleles B8, DR3, DQB1, DQA1
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What is the featured morphology of Sjogren's syndrome?
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Periductal and Perivascular infiltration of salivary glands with lymphocytes
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What cells often initiate the autoimmune destruction of the lacrimal and salivary glands?
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Thelper cells
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When Sjogren's syndrome becomes systemic, what 4 tissues are usually involved?
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-CNS
-Skin -Kidneys -Lungs |
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Where will renal lesions primarily be seen if they arise in Sjogren's syndrome?
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In the TUBULES
-Tubulointerstitial nephritis (as opposed to the glomerulus as in SLE) |
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What is lymph node enlargement in Sjogren's syndrome due to?
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Polyclonal Bcell activation
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What are patients with Sjogren's syndrome at a 40X higher risk of developing?
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Bcell lymphoma
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What is the etiology of Systemic Sclerosis?
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It is unknown
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What is Systemic Sclerosis characterized by?
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Abnormal accumulations of Fibrous Tissue in the Skin and Multiple organs
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What organ is most commonly affected by systemic sclerosis?
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The skin
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What are 4 common causes of death in patients that die from Systemic Sclerosis?
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-Heart failure
-Kidney failure -pulmonary insufficiency -Intestinal malabsorption (starve) |
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What is the female:male ratio of Systemic sclerosis?
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3:1
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What precedes all other symptoms in 70% of systemic sclerosis cases?
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Raynaud's phenomenon
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What is the cause of intestinal malabsorption?
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-Dysphagia
-Gastroesophageal reflux -Esophageal hypomotility |
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What is the cause of pulmonary insufficiency?
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Pulmonary hypertension and fibrosis
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What is the cause of Cardiac failure?
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Myocardial fibrosis
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What is the cause of Renal failure?
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Hypertension
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What are 2 different flavors of systemic sclerosis?
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1. Diffuse Scleroderma
2. Limited Scleroderma |
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How can Diffuse scleroderma be identified?
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1. There will be WIDESPREAD SKIN INVOLVEMENT at the disease ONSET
2. Rapidly progresses and involves visceral organs early. |
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How can Limited scleroderma be identified?
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1. limited skin involvement
2. LATE visceral involvement |
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What do some patients with limited scleroderma have?
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CREST syndrome
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What does CREST stand for?
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-Calcinosis
-Raynaud's phenomenon -Esophageal dysmotility -Sclerodactyly -Telangiectasia |
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What antibody is characteristic of CREST & limited scleroderma?
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Anti-centromere
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What antibody is characteristic of disseminated scleroderma and systemic sclerosis?
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anti-Scl 70
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What causes the CD4+ Tcells to develop an immune reaction in systemic sclerosis?
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An UNIDENTIFIED ANTIGEN
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What inflammatory mediators are part of the SS pathology?
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IL1, IL2, TNF, PDGF, and TGF-beta
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What do we know TGF-beta does?
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Stimulates fibrosis and collagen deposition, which are the pathologic causes of Systemic sclerosis.
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How does microvascular injury develop in systemic sclerosis?
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Intimal fibrosis/capillary dilation -> widespread vascular narrowing/ischemic injury.
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What is anti-DNA topoisomerase I?
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Anti Scl-70
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What % of systemic sclerosis patients have a positive Anti-Scl 70?
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70%
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What is the Anti-Scl 70 specifically associated with? (symptoms)
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-Pulmonary fibrosis
-Peripheral vascular disease (so probably not limited) |
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What antibody is associated with scleroderma? In what percent of patients?
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Anti-centromere Ab
-In 36% of patients |
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What percent of patients with CREST syndrome have a positive Anti-centromere?
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96%
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What does subcutaneous fibrosis do to the fingers in systemic sclerosis?
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Causes flexion deformity
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What causes cutaneous ulcerations?
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Loss of blood supply
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What is "rubber hose" esophagus?
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Thickening of the epithelium in the GI tract in systemic sclerosis
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What does rubber hose esophagus cause in 80% of pateints?
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GERD and strictures
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What causes the hypertension in the kidneys in systemic sclerosis?
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Thickening of the interlobular arteries
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What is the MAJOR CAUSE OF DEATH IN SYSTEMIC SCLEROSIS?
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Interstitial fibrosis and pulmonary hypertension cause pulmonary insufficiency and suffocation.
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What is the treatment for early disseminated Systemic sclerosis?
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Immunosuppressive agents
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What is used for treating Raynaud phenomenon?
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Vasodilators
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What are 2 specific therapies for SS?
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-Anti-TNFalpha
-Anti-TGFbeta |
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What are the 2 inflammatory myopathy diseases?
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-Dermatomyositis
-Polymyositis |
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In what patients is Dermatomyositis seen?
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Adults and children
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What is the presentation of dermatomyositis?
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-Distinct skin discoloration
-Symmetric, proximal muscle weakness |
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What does Dermatomyositis put patients at a 40% increased risk of developing?
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Visceral cancers
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What is the pathology of dermatomyositis?
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1. Antibodies and complement attack capillaries
2. Ischemia to muscles causes necrosis and atrophy |
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How is polymyositis distinguished from dermatomyositis?
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There are no skin lesions and it mostly occurs in adults.
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What is the pathology of polymyositis?
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CTL mediated cell injury
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What is mixed connective tissue disease?
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A mishmash of all the systemic autoimmune diseases.
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What antibody suggests MCTD?
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Anti-U1-RNP
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What is RARELY found in MCTD?
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Renal involvement
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