path disease of immunity - formatted Flashcards
- 57.APRIL02 A 32-year-old woman with transfusion related HIV infection develops a cough, fever and pleuritic chest pain: She has a CD 4 count of 550-cells/ microlitre (standard units). Chest x-ray shows a non-specific opacity in the right lower lobe. The most likely diagnosis is
a. Mycobacterium avium-intracellulare infection
b. Pneumocystis carinii infection
c. Pneumococcus pneumonia infection
d. HIV-associated deep venous thrombosis/ pulmonary embolism
e. Metastatic Kaposi Sarcoma
c. Pneumococcus pneumonia infection – T – with count >500, would normally be asymptomatic, and pneumococcus pneumonia would be most likely, as would be in a non-HIV patient.
- 57.APRIL02 A 32-year-old woman with transfusion related HIV infection develops a cough, fever and pleuritic chest pain: She has a CD 4 count of 550-cells/ microlitre (standard units). Chest x-ray shows a non-specific opacity in the right lower lobe. The most likely diagnosis is: (TW)
a. Mycobacterium avium-intracellulare infection
b. Pneumocystis carinii infection – risk of PCP is extremely high in individuals with fewer than 200 CD4+ cells/uL.
c. Pneumococcus pneumonia infection – T – with count >500, would normally be asymptomatic, and pneumococcus pneumonia would be most likely, as would be in a non-HIV patient.
d. HIV-associated deep venous thrombosis/ pulmonary embolism
e. Metastatic Kaposi Sarcoma
CD4+ greater than or equal to 500 cells/ul are generally asymptomatic. Counts below 500 are associated with early symptoms, and below 200 with severe immunosuppression.
- 5.03.04 Patient with HIV and normal CD4 count, pathchy pulmonary opacities on CXR, which is MOST LIKELY?
a. CMV
b. Toxoplasmosis
c. Kaposi’s
d. Cryptococcus
e. PCP
c. Kaposi’s – T – about 3 % of the HIV positive patients who devleop Kaposi sarcoma still have relatively intact immunity with normal CD4 counts. Risk of KS is proportional to HIV viral load.
* LW: out of available options this is favored - RObbins states KS can occur in the early HIV phase before immune system is compromised.
- 5.03.04 Patient with HIV and normal CD4 count, patchy pulmonary opacities on CXR, which is MOST LIKELY? (TW) **** Opinions ** prev e. True
a. CMV – F – CMV pneumonia occurs primarily with advanced HIV infection and severe immunodeficiency (study: subjects with CMV pneumonia more likely to have low CD4 counts (below 12 cells/uL)). CMV pneumonia is rarely described in immunocompetent hosts.
b. Toxoplasmosis – F – extracerebral toxoplasmosis: most prominent risk factor is advanced immunosuppression (CD4 counts ~50).
c. Kaposi’s – T – about 3 % of the HIV positive patients who devleop Kaposi sarcoma still have relatively intact immunity with normal CD4 counts. Risk of KS is proportional to HIV viral load.
d. Cryptococcus – F – Presentation of pulmonary cryptococcosis in HIV infected persons appears to be more acute and severe than in other patient groups. Severity of symptoms and extent of dissemination are inversely proportional to the CD4 count, with most symptomatic cases occurring in patients with counts <100/uL. Has been described in immunocompetent hosts with radiographic findings of: (varied appearances) well-defined, non-calcified nodules, either solitary or a few. Other chest radiographs have shown lobal infiltrates, hilar and mediastinal adenopathy, and pleural effusions.
e. PCP – T – Generally PCP did not occur until the CD4 count dropped below 200/uL. While PCP is decreasing in frequence due to the use of PCP prophylasis and HAART, it is still the most common opportunistic infection in HIV-infected patients. PCP rarely occurs in patients without apparent immunodeficiency.
- 5.02.23 HIV positive patient with CD 4 count less than 100 has a mass on CT brain. What is likely diagnosis?
a. Lymphoma
b. Toxoplasmosis
c. Cryptosporidium
d. TB
e. PML
b. Toxoplasmosis – T – Toxoplasmosis is most common cause of focal lesion in HIV positive patient.
- 5.02.23 HIV positive patient with CD 4 count less than 100 has a mass on CT brain. What is likely diagnosis? (TW)
a. Lymphoma – F – second most common cause of focal CNS mass in HIV (primary CNS lymphoma).
b. Toxoplasmosis – T – Toxoplasmosis is most common cause of focal lesion in HIV positive patient.
c. Cryptosporidium – F – (note, not cryptococcus) Cryptosporidium is an intracellular protozoan parasite that is associated with GI diseases. Causes asymptomatic infection, through to severe enteritis.
d. TB
e. PML – F – multifocal, scalloping white matter hypodensities without mass effect or oedema. Polyoma virus (JC virus [John Cunningham])
- 5.02.38 Watery diarrhoea in HIV positive patient passing oocysts is compatible with infection by ?
a. Strongyloides
b. Entamoeba histolytica
c. Cryptosporidium
c. Cryptosporidium – T – Dx is primarily based on microscopic identification of the oocysts in stool or tissue. Affects small bowel (so large volume watery diarrhoea). Organism most closely associated with AIDS cholangiopathy is Cryptosporidium.
- 5.02.38 Watery diarrhoea in HIV positive patient passing oocysts is compatible with infection by ? (TW)
a. Strongyloides – F – detect rhabditiform larvae in concentrated stool.
b. Entamoeba histolytica – F – can have cysts in stool, but affects colon (so frequent, small volume, often painful stools).
c. Cryptosporidium – T – Dx is primarily based on microscopic identification of the oocysts in stool or tissue. Affects small bowel (so large volume watery diarrhoea). Organism most closely associated with AIDS cholangiopathy is Cryptosporidium.
- 5.03.20 In a patient with systemic sclerosis and hand changes MOST LIKELY IF ?
a. Female in 20’s and Raynauds
b. Female in 50’s and Raynauds
c. Female in 20’s with pulmonary fibrosis
b. Female in 50’s and Raynauds – T – primarily disease of women (3:1). Peak incidence 50-60yo. Raynaud phenomenon, manifested as episodic vasoconstriction of the arteries and arterioles of the extremities precedes all other symptoms in 70% of cases.
- 5.03.91 Patient with systemic sclerosis for renal artery doppler ?
a. Changes indistinguishable from FMD
b. Vasculitis of medium sized vessels
c. Fibrinoid necrosis
- *LJS - disagree. Occlusive vasculopathy (non-inflammatory) rather than vasculitis. I would choose c. fibrinoid necrosis - changes resembling malignant HTN incl fibrinoid necrosis
https: //www.ncbi.nlm.nih.gov/pmc/articles/PMC2952802/
*LW:
As always agree with LJS:
Systemic sclerosis related kidney disease primary histopathologic changes in the kidney are localized in the small arcuate and interlobular arteries and the glomeruli.
The characteristic finding is intimal proliferation and thickening that leads to narrowing and obliteration of the vascular lumen, with concentric “onion-skin” hypertrophy.
SRC is a thrombotic microangiopathy similar to malignant nephrosclerosis, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS), radiation nephritis, chronic transplantation rejection, and the antiphospholipid antibody syndrome.
b. Vasculitis of medium sized vessels – T – most prominent renal abnormalities are those in the vessel walls: interlobular arteries (150um to 500um diameter) show intimal thickening as a result of deposition fo mucinous or finely collagenous material (Robbins). The interlobular arteries are the arteries most often affected by small-vessel vasculitis (Heptinstall’s Path of Kidney). Robbins groups visceral arteries and branches / arterioles as medium-sized vessels.
- 5.03.91 Patient with systemic sclerosis for renal artery doppler ? Rob p226 (TW)
a. Changes indistinguishable from FMS – F – histological findings (in hypertensives with SS) can be difficult to Dx from other types of malignant hypertension.
b. Vasculitis of medium sized vessels – T – most prominent renal abnormalities are those in the vessel walls: interlobular arteries (150um to 500um diameter) show intimal thickening as a result of deposition fo mucinous or finely collagenous material (Robbins). The interlobular arteries are the arteries most often affected by small-vessel vasculitis (Heptinstall’s Path of Kidney). Robbins groups visceral arteries and branches / arterioles as medium-sized vessels.
c. Fibrinoid necrosis – F – can occur in SS patients with hypertension, but more a feature of malignant hypertension.
Vasculitides are defined by the presence of leucocytes in the vessel wall with reactive damage to mural structures.
Vasculitis secondary to connective tissue disorders most frequently involves the small muscular arteries, arterioles, and venules (UTD).
- 5.03.98 Systemic sclerosis and hypertension - MOST LIKELY cause ?
a. intimal proliferation of interlobar arteries
b. pipestem fibrosis of adventitia of proximal renal aa
c. malignant HTN secondary to accelerated atherosclerosis
a. intimal proliferation of interlobar arteries – T – interlobular arteries show intimal thickening as a result of deposition of mucinous or finely collagenous material. There is also concentric proliferation of intimal cells.
- 5.03.98 Systemic sclerosis and hypertension - MOST LIKELY cause ? (TW)
a. intimal proliferation of interlobar arteries – T – interlobular arteries show intimal thickening as a result of deposition of mucinous or finely collagenous material. There is also concentric proliferation of intimal cells.
b. pipestem fibrosis of adventitia of proximal renal aa
c. malignant HTN secondary to accelerated atherosclerosis – Hypertension occurs in 30% of patients with scleroderma (as result of a.), and in 20% it takes an ominously malignant course (malignant hypertension).
- Sep03.04 SLE
a. DsDNA
b. arterial & venous involvement
a. DsDNA – T – (assuming they are meaning antibodies to dsDNA). Anti-dsDNA and anti-Sm (Smith) antibodies are two autoantibodies that are highly specific for SLE.
- Sep03.04 SLE (TW)
a. DsDNA – T – (assuming they are meaning antibodies to dsDNA). Anti-dsDNA and anti-Sm (Smith) antibodies are two autoantibodies that are highly specific for SLE.
b. arterial & venous involvement – F – many of the clinical manifestations of SLE are mediated directly or indirectly by antibody formation and the creation of immune complexes. An acute necrotizing vasculitis involving small arteries and arterioles may be present in any tissue.
- Sep03.05 Systemic sclerosisa. Vasculitis late (t or f)
a. Vasculitis late – F – microvascular disease is consistently present early in the course of systemic sclerosis
- Sep03.84 Systemic sclerosis, most correct statementa. >8% have GIT involvement
a. >8% have GIT involvement – T – I guess this is true (but other options would probably be better, whatever they were).
* LW: likely incomplete recall as GIT is affected in approx. 90% patient with SS.
Nearly 90% of patients with either subtype of systemic sclerosis (Diffuse SSc or localized SSc) have some degree of GI involvement. Nearly half of these will be asymptomatic.
- Sep03.36 SLEa. Malar rash – is one of 11 diagnostic criteria
- Sep03.36 SLE (TW)
a. Malar rash – is one of 11 diagnostic criteria
Diagnostic criteria for SLE: Dx made if 4 or more manifestations present, either serially or simultaneously, during any interval of observation: Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis Serositis Renal disorder Neurologic disorder Hematologic disorder Immunologic disorder ANA
- Sep03.XX Regarding SLE, least likely associated with
a. M > F
b. Ab to double stranded DNA and Smith Ag
c. Venous and arterial thrombosis
d. 50% associated with renal disease
- Sep03.XX Regarding SLE, least likely associated with: (TW)
a. M.F FALSE: females > males. Women especially in 20s and 30s. 9x > in women of childbearing age, 2x > in female children.
b. Ab to double stranded DNA and Smith Ag – T – anti-dsDNA and anti-Sm (Smith) antibodies are highly specific for SLE.
c. Venous and arterial thrombosis TRUE - **LJS - recurrent venous and arterial thromboses - miscarriage, cerebral/ocular, hepatic vein
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3413961/#:~:text=Thrombosis%20in%20SLE,%2Drisk%20patients%20%5B5%5D.
(*LW - as always, agrees with LJS, UpToDate confirms, antiphospholipid AB increases risk of venous and arterial thrombosis).
d. 50% associated with renal disease – T – renal involvement becomes clinically apparent in approximately 50% of patients; however, most of the remaining patients have subclinical disease that can be demonstrated by renal biopsy.
- 5.03.88 Amyloid – which is FALSE?
a. In TB is AA type
b. Causes sago spleen and lardaceous spleen
c. Seen in 60% of patients with renal failure
c. Seen in 60% of patients with renal failure - F – depends on duration and if on hemodialysis: Amyloidosis in 21% patients on HD for less than 2y; 50% for 4-7yr, and 90% at 7-13y.
- 5.03.88 Amyloid – which is FALSE? Rob p158 (TW)
a. In TB is AA type – T – secondary (AA) amyloidosis, from chronic or recurring inflammation.
b. Causes sago spleen and lardaceous spleen – T – amyloid of the spleen is seen in 2 forms: sago spleen produced by amyloid degeneration of the organ where deposits are limited to the follicles (cross section shows scattered gray translucent bodies looking like grains of sago); and lardaceous spleen in which amyloid is depositied in the walls of the splenic sinusoids and spares the follicles.
c. Seen in 60% of patients with renal failure - F – depends on duration and if on hemodialysis: Amyloidosis in 21% patients on HD for less than 2y; 50% for 4-7yr, and 90% at 7-13y.
Most frequent types of amyloidosis are AL (primary) and AA (secondary) types.
- AL amyloid is due to deposition of protein derived from immunoglobulin light chain fragments. It is a plasma cell dyscrasia in which monoclonal immunoglobulin is detectable in the serum or monoclonal light chains in the urine in approx 80% of cases. AL amyloidosis can occur alone or in association with multiple myeloma or, much less often, Waldenstrom’s macroglobulinemia.
- AA amyloidosis may complicate chronic diseases in which there is ongoing or recurring inflammation, such as RA or spondyloarthropathy; chronic infections; or periodic fever syndromes.
- Amyloidosis as a disease was discovered by Dr Rokitansky, who called it “lardaceous change” (fucken Rokitansky again).
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it?
a. Type 1 hypersensitivity
b. Type 2
c. Type 3
d. Type 4
e. Non-immune reaction
c. Type 3 - T - Immunocomplex disease. Induced by antigen-antibody complexes that produce tissue damage as a result of their capacity to active the complement system. Can be generalized (immune complexes formed in circulation and deposited in many organs etc), or localized to particular organs such as kidney (GN), joints (arthritis) or the small blood vessels of the skin if the complexes are formed an deposited locally (the local Arthus reaction). See below.
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it? (TW)
a. Type 1 hypersensitivity - F- Anaphylactic type. Rapidly developing immunologic reaction developing within minutes after combination of antigen with antibody bound to mast cells or basophils in individuals previously sensitized to antigen
b. Type 2 - F - Cytotoxic type. Mediated by antibodies directed toward antigens present on surface of cells or other tissue component (subtypes: complement-dependent reactions; antibody-dependent cell mediated cytotoxicity; antibody mediated cellular dysfunction)
c. Type 3 - T - Immunocomplex disease. Induced by antigen-antibody complexes that produce tissue damage as a result of their capacity to active the complement system. Can be generalized (immune complexes formed in circulation and deposited in many organs etc), or localized to particular organs such as kidney (GN), joints (arthritis) or the small blood vessels of the skin if the complexes are formed an deposited locally (the local Arthus reaction). See below.
d. Type 4 - F - Cell mediated (delayed). Initiated by specifically sensitized T lymphocytes.
e. Non-immune reaction - F - unless someone is repeatedly extravasating a lot of contrast! Maybe fun, but not ethical.
Local immune complex disease (Arthus reaction) - type III hypersensitivity reaction. Localized area of tissue necrosis resulting from acute immune complex vasculitis, usually elicited in the skin. Reaction can be produced by intracutaneous injection of antigen in an immune patient having circulating antibodies against the antigen. Unlike IgE mediated type I reactions, which appear immediately, the Arthus lesion develops over a few hours and reaches a peak 4-10hrs after injection, when it can be seen as an area of visible oedema with severe hemorrhage followed occasionally by ulceration.
- Sep03.78 Amyloidosis is least likely to be associated with:
a. renal failure
b. cystic fibrosis
b. cystic fibrosis – amyloidosis is a rare complication of cystic fibrosis.
- Sep03.78 Amyloidosis is least likely to be associated with: (TW)
a. renal failure – commonly occurs in patients with renal failure on haemodialysis. Amyloidosis seen in 21% patients on HD for less than 2y; 50% for 4-7yr, and 90% at 7-13y.
b. cystic fibrosis – amyloidosis is a rare complication of cystic fibrosis.
