Pathology 1 - Block 2 - Part 1

davidwurbel7's version from 2016-02-22 19:10


Question Answer
The presence of anti-double stranded DNA (ds), anti- Smith (Sm) and antiphospholipid antibodies is suggested of thisSystemic Lupus Erythomatosis
Leads to persistence and uncontrolled activation of self-reactive lymphocytesImmunological Factors
autoantibodies specific for red cells can lead toHemolytic Anemia
autoantibodies specific for white cells can lead toLeukopenia
autoantibodies specific for platelets can lead toThrombocytopenia
Free and/or exposed damaged nucleiLE Bodies
Any phagocytic leukocyte that has engulfed denatured nucleus of an injured cellLE Cell
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Question Answer
Exposure to sunlight accentuates the erythemaButterfly Rash
Vacuolar degeneration of basal cells in epidermis. Dermis show edema, perivascular inflammation. Vasculitis with fibrinoid necrosisSLE Skin Changes
Non-bacterial verrucous endocarditis (fibrinous). A pericardial friction rub on auscultationLibman-Sacks Endocarditis
Multisystem disease usually presents in young woman presenting with butterfly rash. recurrent early fetal loss (APL). photo sensitivity. Fever, headaches, seizures (due to intracerebral bleed due to thrombocytopenia) , joint pain with no deformity. Renal involvement is serious. Recurrent spontaneous abortionsSystemic Lupus Erythomatosis
This condition is diagnosed by anti nuclear antibodies (ANA). high titer of IgG antibodies to double stranded DNA & antibodies to Sm antigen (most useful). LE cell: neutrophil containing phagocytosed altered DNA. immunofluoresence in skin/renal biopsies. proteinuriaSystemic Lupus Erythromatosis
Specialized macrophages with phagocytic activity within jointsType A Synoviocytes
Similar to fibroblasts and synthesize hyaluronic acid and various proteins within jointsType B Synoviocytes
A plasma filtrate containing hyaluronic acid that act as a viscous lubricant and provides nutrition for articular hyaline cartilageSynovial Fluid
Chronic inflammatory disease, characterized by symmetric, peripheral polyarthritis. Inflammatory synovitis resulting in destruction of articular cartilage and ankylosis (fusion) of joints. Increases between 25 and 55 years, more in females. Small joints of hands and feet (peripheral joints). symmetric involvement of proximal interphalangeal (PIP) and metacarpophalangeal (MCP) joints producing Ulnar Drift. Distal interphalangeal joint (DIP) is usually spared. Extra articular lesions may involve skin, heart, blood vessels, lungsRheumatoid Arthritis
This cytokine is from TH1 cells which activate macrophages and resident synovial cellsIFN-ɣ
This cytokine is from TH17 cells which recruits neutrophils and macrophagesIL-17
These cytokines are from macrophages stimulate resident synovial cells to secrete proteases that destroy hyaline cartilageTNF and IL-1
This is expressed on activated T cells stimulate bone absorptionRANKL
Autoantibodies directed against citrullinated peptides (CCPs) are diagnostic for thisRheumatoid Arthritis
Serum IgM and/or IgA autoantibodies is the same as thisRheumatoid Factor
This gene is linked to Rheumatoid arthritisHLA-DRB1
This gene is a hallmark of Rheumatoid arthritisPTPN22
Pathologic hallmarks of this are synovial inflammation and proliferation, focal bone erosions, and thinning of articular cartilageRheumatoid Arthritis
Chronic inflammation leads to synovial lining hyperplasia and the formation of thisPannus
A thickened cellular membrane of granulation–reactive fibrovascular tissue that grows over the articular cartilage and causes erosionPannus
Sites of pressure- ulnar aspect of the forearm, elbows, occiput, lumbosacral area. They are firm, non-tender round to oval skin lesionsRheumatoid Nodules
Popliteal cyst develops posteriorly and inferiorly to the knee as a distention of a local bursa. Diagnosis may be made by aspiration of mucinous fluidBaker Cyst
Chronic, slowly progressive autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in xerostomia, and dry eyes (keratoconjunctivitis)Sjögren Syndrome
Dry mouthXerostomia
Dry eyeKeratoconjuctivitis
Lymphocytic infiltrate and fibrosis of lacrimal and salivary gland decreases in tears and saliva productionSjögren Syndrome
SS-A (Ro) and SS-B (La) autoantibodies are presentSjögren Syndrome
Sjögren Syndrome is diagnosed by the presence of these antibodiesSS-A (Ro) and SS-B (La)
The morphology of the salivary gland periductal and perivascular lymphocytic infiltration. Lymphoid follicles with germinal centers may appear. ductal lining cells show hyperplasia, this leads to obstruction. Later there is acinar atrophy, fibrosis, and hyalinizationSjögren Syndrome
B- Cell Lymphoma is a well known risk for this conditionSjögren Syndrome
Characterized by chronic inflammation (autoimmune), widespread damage to small blood vessels, and progressive interstitial and perivascular fibrosis in skin and multiple organs. GI symptoms include progressive atrophy and collagenous fibrous replacement of the muscularis may develop at any level of the gut but are most severe in the esophagus lower two thirds of the esophagus often develops a rubber-hose–like inflexibility – leading to GERD. Lower esophageal sphincter gives rise to gastroesophageal reflux and its complications, including Barrett esophagitis (metaplasia). Kidney symptoms include vascular lesions (interlobular arteries show intimal thickening with mucinous or collagenous material) concentric proliferation of intimal cells, similar to those seen in hypertension. Lungs - pulmonary hypertension, & interstitial fibrosis. Heart - pericarditis with effusion, myocardial fibrosis. thickening of intramyocardial arteriolesSystemic Sclerosis (Scleroderma)
T-cells produce this which activates fibroblasts to produce collagenTGF-β
T-cells produce TGF-β which activates these to produce collagenFibroblasts


Question Answer
Calcinosis cutis, Raynaud’s phenomenon, esophageal dysfuntion, sclerodactly (tapered fingers, claw-like) and telangiectasiaCREST Syndrome
CREST syndrome is most often seen associated with this conditionSystemic Sclerosis (Scleroderma)
An episodic vasoconstriction in the fingers and toes that occurs in virtually every patient with scleroderma start with pallor, followed by cyanosis of variable duration eventually erythema develops spontaneously or with rewarming of the digitRaynaud's phenomenon
The two anti-nuclear antibodies (ANA’s) that are diagnostic for this condition are DNA topoisomerase I (anti-Scl 70) and Anticentromere antibodySystemic Sclerosis (Scleroderma)
Systemic Sclerosis (Scleroderma) is diagnosed by the presence of these two anti-nuclear antibodiesDNA topoisomerase I (anti-Scl 70) and Anticentromere Antibody
Heterogeneous group of genetically determined autoimmune disorders that predominately target the skeletal musculature and/or skin and typically result in symptomatic skeletal muscle weakness and/or cutaneous inflammatory disease Dermatomyositis
Immunologic disease in which there is damage to small blood vessels contributing to muscle injury. Vascular findings are present as telangiectasia (dilated capillary loops) seen in nail folds, eyelids, and gums. Biopsy of muscle/skin show deposits of complement: C5b-9Dermatomyositis
Anti-Mi2 antibodies: associated with Gottron papules. Anti-Jo1 antibodies: associated with interstitial lung disease, non-erosive arthritis, skin rash. Anti-P155/P140 antibodies: associated with paraneoplastic & juvenile cases. Muscle biopsy: mono-nuclear inflammatory cells, mostly around perimysial connective tissue and around blood vessels this leads to perifasicular atrophy of muscle. Immunohistochemistry: inflammatory cells stain positive for CD4+ T-helper cells, and deposition of C5b-9 in capillary vesselsDermatomyositis
Confluent macular violaceous erythema, most pronounced over the metacarpophalangeal/interphalangeal joints, extending in a linear array overlying the extensor tendons of the hand and fingersGottron Sign
Clinical features of this condition include muscle weakness, slow onset often accompanied by myalgias. Getting up from chair, climbing stairs become difficult. elevated serum creatinine kinase levels. lilac colored discoloration of upper eyelids (heliotrope rash). scaling erythematous eruption or dusky red patches over knuckles/elbows/knee - Gottron papules. dysphagia. interstitial lung disease (10%)Dermatomyositis
Mixed connective tissue disease may progress to this conditionSLE


Lab Diagnostics
Question Answer
The two anti-nuclear antibodies (ANA’s) that are diagnostic for this condition are DNA topoisomerase I (anti-Scl 70) and Anticentromere antibodySystemic Sclerosis (Scleroderma)
Systemic Sclerosis (Scleroderma) is diagnosed by the presence of these two anti-nuclear antibodiesDNA topoisomerase I (anti-Scl 70) and Anticentromere Antibody
SS-A (Ro) and SS-B (La) autoantibodies are presentSjögren Syndrome
Sjögren Syndrome is diagnosed by the presence of these antibodiesSS-A (Ro) and SS-B (La)
Rheumatoid arthritis is diagnosed by the presence of these antibodiesRF factor and Anti-citrullinated peptides (CCPs)
RF factor and Anti-citrullinated peptides (CCPs) presence is diagnostic in this conditionRheumatoid Arthritis
Anti-Mi2 antibodies, Anti-Jo1 antibodies and Anti-P155/P140 antibodies presence is diagnostic in this conditionDermatomyositis
Dermatomyositis is diagnosed by the presence of these antibodiesAnti-Mi2 antibodies, Anti-Jo1 antibodies and Anti-P155/P140 antibodies