Chapter 10*

Question 1

Buerger disease—thromboangitis obliterans is an occlussive inflammatory disease of the medium and small arteries of the ________ arms and legs. The etiologic role of ________ has been emphasized by the observation that cessation of smoking can be followed by remission.

Microscopic eval of affected vessels show polym ________ infiltrates extending to neighboring veins and nerves. Inflammation of the endothelium is associated with thrombosis and obliteration of the affected vessels.

Answer 1

Buerger disease—thromboangitis obliterans is an occlussive inflammatory disease of the medium and small arteries of the distal arms and legs. The etiologic role of smoking has been emphasized by the observation that cessation of smoking can be followed by remission.

Microscopic eval of affected vessels show polymorphonuclear infiltrates extending to neighboring veins and nerves. Inflammation of the endothelium is associated with thrombosis and obliteration of the affected vessels.

Question 2

Syphilitic aneurysm typically affects the ________ aorta. Microscopic exam typically shows obliterive endartitis and periarteritis of the ________ vasorum. The vasa vasorum ramify in the adventitia and penetrate the outer and middle third of the aorta.

In syphilitic disease, ________ vasorum become encircled by lymphocytes, plasma cells, and macrophages.

Obliteration of the vasa vasorum causes focal ________ and scarring of the media, with disruption and disorganization of the elastic lamellae. The inner surface of the affected aorta shows a ________ bark appearance.

Answer 2

Syphilitic aneurysm typically affects the ascending aorta. Microscopic exam typically shows obliterive endartitis and periarteritis of the vasa vasorum. The vasa vasorum ramify in the adventitia and penetrate the outer and middle third of the aorta.

In syphilitic disease, vasa vasorum become encircled by lymphocytes, plasma cells, and macrophages.

Obliteration of the vasa vasorum causes focal necrosis and scarring of the media, with disruption and disorganization of the elastic lamellae. The inner surface of the affected aorta shows a tree bark appearance.

Question 3

Malignant hypertension refers to an elevated blood pressure that may result in rapidly progressive vascular disease affecting the brain, heart, and kidneys. The disease injures ________ cells, causing increased vascular permeability, which leads to the insudation of ________ proteins into the vessel wall and morphologic evidence of fibrinoid necrosis.

In patients of Malignant hypertension, acute vascular injury is followed by ________ muscle proliferation with an increase in concentric layers of smooth mucle cells that yield the so-called onion skin appearance. Poor perfusion of the kidneys stimulates ________ release, which serves to elevate systemic blood pressure even further = renovascular hypertension.

Answer 3

Malignant hypertension refers to an elevated blood pressure that may result in rapidly progressive vascular disease affecting the brain, heart, and kidneys. The disease injures endothelial cells, causing increased vascular permeability, which leads to the insudation of plama proteins into the vessel wall and morphologic evidence of fibrinoid necrosis.

In patients of Malignant hypertension, acute vascular injury is followed by smooth muscle proliferation with an increase in concentric layers of smooth mucle cells that yield the so-called onion skin appearance. Poor perfusion of the kidneys stimulates renin release, which serves to elevate systemic blood pressure even further = renovascular hypertension.

Question 4

Polyarteritis ________ is an inflammatory process. PAN is an acute necrotizing vasculitis that affects medium-sized and smaller muscular ________. On occasion, PAN extends into larger arteries, such as the renal, splenic, or coronary arteries.

The most common morphological feature of affected arteries is fibrinoid ________, in which the medial muscle and adjacent tissue are fused into an eosinophilic mass that stains for fibrin.

PAN affecting small vessels is frequently associated with the presence of Perinuclear Anti-Neutrophil ________ Antibodies (P-ANCA). 15% of patients with PAN demonstrate either HBsAg or anti HCV antibodies (Hep B & C).

Answer 4

Polyarteritis nodosa is an inflammatory process. PAN is an acute necrotizing vasculitis that affects medium-sized and smaller muscular arteries. On occasion, PAN extends into larger arteries, such as the renal, splenic, or coronary arteries.

The most common morphological feature of affected arteries is fibrinoid necrosis, in which the medial muscle and adjacent tissue are fused into an eosinophilic mass that stains for fibrin.

PAN affecting small vessels is frequently associated with the presence of Perinuclear Anti-Neutrophil Cytoplasmic Antibodies (P-ANCA). 15% of patients with PAN demonstrate either HBsAg or anti HCV antibodies (Hep B & C).

Question 5

Hypersensitivity Agiitis refers to a broad spectrum of inflammatory lesions that represent a reaction to foreign materials (e.g., bacterial products or more commonly drugs).

Hypersensitivity Agiitis may be caused by ________ drugs.

When the vascular lesions are confined to the skin, the terms leukocytoclastic vasculities, cutaneous vasculitis, or cutaneous necrotizing venulitis are applied.

Answer 5

Hypersensitivity Agiitis refers to a broad spectrum of inflammatory lesions that represent a reaction to foreign materials (e.g., bacterial products or more commonly drugs).

Hypersensitivity Agiitis may be caused by sulfa drugs.

When the vascular lesions are confined to the skin, the terms leukocytoclastic vasculities, cutaneous vasculitis, or cutaneous necrotizing venulitis are applied.

Question 6

Giant cell temporal arteritis is the most common vasculitis. The disease is a local, chronic, granulomatous inflammation of the ________ arteries.

The average age of onset is 70 years.

Histologic examination shows ________ cell granulomatous inflammation, which destroy the media of the temporal artery and predisposes to thrombosis.

Headaches typically in the form of throbbing temporal pain and visual problems may appear. A palpable, tortuous, and swollen temporal artery may be the only finding on physical exam.

Answer 6

Giant cell temporal arteritis is the most common vasculitis. The disease is a local, chronic, granulomatous inflammation of the temporal arteries.

The average age of onset is 70 years.

Histologic examination shows giant cell granulomatous inflammation, which destroy the media of the temporal artery and predisposes to thrombosis.

Headaches typically in the form of throbbing temporal pain and visual problems may appear. A palpable, tortuous, and swollen temporal artery may be the only finding on physical exam.

Question 7

Wegener granulomatosis causes granulomatous inflammation, but ________ in temporal artery.

Answer 7

Wegener granulomatosis causes granulomatous inflammation, but NOT in temporal artery.

Question 8

Takayasu arteritis is an inflammatory disorder of the large arteries, classically the aortic ________ and its major branches. On gross exam, the aorta is ________, and the intima exhibits focal, raised plaques. The branches of the aorta often display localized stenosis or occlusion, which interferes with blood flow and accounts for the symptoms of pulseless disease.

Ischemic cerebrovascular episodes in a young woman and a ________ between the blood pressure in the left and right arm suggest a diagnosis of Takayasu. More than 90% of patients are women under 30.

Answer 8

Takayasu arteritis is an inflammatory disorder of the large arteries, classically the aortic arch and its major branches. On gross exam, the aorta is thickened, and the intima exhibits focal, raised plaques. The branches of the aorta often display localized stenosis or occlusion, which interferes with blood flow and accounts for the symptoms of pulseless disease.

Ischemic cerebrovascular episodes in a young woman and a differential between the blood pressure in the left and right arm suggest a diagnosis of Takayasu. More than 90% of patients are women under 30.

Question 9

Churg-Strauss disease is an idiopathic, systemic, granulomatous disease of small and medium sized arteries characterized by vasculitis of many organs, fluctuating eosinophilia, and late-onset asthma.

The majority of patients display ________ neutrophil cytoplasmic antibodies. The disease is also known as allergic granulomatoisis and angitis. Transbronchial lung biopsy shows granulomatous lesions in vascular and extravascular sites, accompanied by intense eosinophilia.

The vasculitis histologically resembles the lesions of polyarteritis nodosa and wegener granulomatoisis, but these diseases do not present with an ________ syndrome like in Churg-Strauss.

Answer 9

Churg-Strauss disease is an idiopathic, systemic, granulomatous disease of small and medium sized arteries characterized by vasculitis of many organs, fluctuating eosinophilia, and late-onset asthma.

The majority of patients display antineutrophil cytoplasmic antibodies. The disease is also known as allergic granulomatoisis and angitis. Transbronchial lung biopsy shows granulomatous lesions in vascular and extravascular sites, accompanied by intense eosinophilia.

The vasculitis histologically resembles the lesions of polyarteritis nodosa and wegener granulomatoisis, but these diseases do not present with an asthmatic syndrome like in Churg-Strauss.

Question 10

________ disease is a systemic vasculitis characterized by oral apthous ulcers, genital ulceration, and ocular inflammation, with occasional involvement of the nervous , cardio, and GI systems.

The mucocutaneous lesions show a nonspecific vasculitis of arterioles, capillaries, and venules.

The cause of the necrotizing inflammation of small blood vessels is not known, but an association with specific HLA subtypes suggests an immune basis.

Answer 10

Behcet disease is a systemic vasculitis characterized by oral apthous ulcers, genital ulceration, and ocular inflammation, with occasional involvement of the nervous , cardio, and GI systems.

The mucocutaneous lesions show a nonspecific vasculitis of arterioles, capillaries, and venules.

The cause of the necrotizing inflammation of small blood vessels is not known, but an association with specific HLA subtypes suggests an immune basis.

Question 11

________ or skin rash in a patient with a known autoimmune disease like Sjogren syndrome or system lupus erythromatosus is usually attributed to hypersensitivty vasculitis.

This vasculitis is caused by the deposition of immune complexes in ________ venules.

Answer 11

Purpura or skin rash in a patient with a known autoimmune disease like Sjogren syndrome or system lupus erythromatosus is usually attributed to hypersensitivty vasculitis.

This vasculitis is caused by the deposition of immune complexes in dermal venules.

Question 12

________ disease, or mucocutaneous lymph node syndrome, is a vasculitis of unknown etiology that presents with fever, skin rash, mucosal inflammation, and lymph node enlargement.

The disease usually has a self-limited course but may involve the coronary arteries and lead to ________ formation. Heart failure occurs in 1% - 2% of cases.

Answer 12

Kawasaki disease, or mucocutaneous lymph node syndrome, is a vasculitis of unknown etiology that presents with fever, skin rash, mucosal inflammation, and lymph node enlargement.

The disease usually has a self-limited course but may involve the coronary arteries and lead to aneurysm formation. Heart failure occurs in 1% - 2% of cases.

Question 13

Patients who have scleroderma may also get the ________ phenomenon.

Raynaud phenomenon refers to intermittent, bilateral attacks of vasospasm of the fingers or toes, sometimes affecting the ears or nose, resulting in pallor, paresthesias, and pain from ischemia.

The symptoms are precipitated by cold or emotional stimuli and are releived by ________. Raynaud’s may occur as an isolated disorder or as a feature of a number of systemic diseases including SLE and scleroderma.

Answer 13

Patients who have scleroderma may also get the Raynaud phenomenon.

Raynaud phenomenon refers to intermittent, bilateral attacks of vasospasm of the fingers or toes, sometimes affecting the ears or nose, resulting in pallor, paresthesias, and pain from ischemia.

The symptoms are precipitated by cold or emotional stimuli and are releived by heat. Raynaud’s may occur as an isolated disorder or as a feature of a number of systemic diseases including SLE and scleroderma.

Question 14

Intermittend claudication is associated with ________ vascular disease (atherosclerosis).

Answer 14

Intermittend claudication is associated with peripheral vascular disease (atherosclerosis).

Question 15

Chilblains represents itchy or tender red or purple bumps that occur as a reaction to ________.

Answer 15

Chilblains represents itchy or tender red or purple bumps that occur as a reaction to cold.

Question 16

Henoch-Schonlein purpura is the most common childhood vasculitis and is caused by vascular localization of immune complexes, containing predominantly Ig ________. Purpuric skin lesions and glomerulonephritis in a child sugges diagnosis of Henoch-Schonlein purpura.

Answer 16

Henoch-Schonlein purpura is the most common childhood vasculitis and is caused by vascular localization of immune complexes, containing predominantly IgA. Purpuric skin lesions and glomerulonephritis in a child sugges diagnosis of Henoch-Schonlein purpura.

Question 17

Scleroderma is characterized by vasculopathy and excessive ________ deposition in the skin and many internal organs. The disease occurs 4x more in women than men and mostly in people 20 - 25 years of age. Lesions in the arteries, arterioles. and capillaries are typical, and in some cases may be the first demonstratable pathologic finding in the disease.

The kidneys are involved in more than half of the patients with scleroderma, they show marked vascular changes often with focal hemorrhage and cortical infarcts. Among the most severly affected vessels are the interlobular arteries and afferent arterioles.

Early fibromuscular thickening of the subintima causes luminal narrowing, followed by thombosis.

Answer 17

Scleroderma is characterized by vasculopathy and excessive collagen deposition in the skin and many internal organs. The disease occurs 4x more in women than men and mostly in people 20 - 25 years of age. Lesions in the arteries, arterioles. and capillaries are typical, and in some cases may be the first demonstratable pathologic finding in the disease.

The kidneys are involved in more than half of the patients with scleroderma, they show marked vascular changes often with focal hemorrhage and cortical infarcts. Among the most severly affected vessels are the interlobular arteries and afferent arterioles.

Early fibromuscular thickening of the subintima causes luminal narrowing, followed by thombosis.

Question 18

Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterised by ________ (sclero) of the ________ (derma). In the more severe form, it also affects internal organs.

Answer 18

Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterised by hardening (sclero) of the skin (derma). In the more severe form, it also affects internal organs.

Question 19

Picture: question 8; what is the diagnosis?

Answer 19

Picture: question 8; what is the diagnosis? Buerger disease—thromboangitis obliterans

Question 20

Picture: question 9; what is the diagnosis?

Answer 20

Picture: question 9; what is the diagnosis? Obliterive endartitis and periarteritis of the vasa vasorum due to syphilitic aneurysm

Question 21

Picture: question 16; what is the diagnosis?

Answer 21

Picture: question 16; what is the diagnosis? Polyarteritis nodosa

Question 22

Picture: question 19; what is the diagnosis?

Answer 22

Picture: question 19; what is the diagnosis? Giant cell arteritis

Question 23

Picture: question 33; what is the diagnosis?

Answer 23

Picture: question 33; what is the diagnosis? Kawasaki disease

Question 24

Picture: question 34; what is the diagnosis?

Answer 24

Picture: question 34; what is the diagnosis? Raynoud phenomenon

Question 25

Picture: question 40; what is the diagnosis?

Answer 25

Picture: question 40; what is the diagnosis? Scleroderman—subintimal fibromuscular thickening

Question 26

Cryoglobulinemic Vasculitis is a _____ vessel vasculitis associated with serum cryoglobulins. This hypersensitivity vasculitis is characterized by cryoglobulin immune _____ in capillaries, venules, or arterioles, causing vasculitis. Cryoglobins are abnormal proteins in the blood that form crystals when the blood sample is _____ below 37 degrees Celsius (98.6 degrees Farenheit).

These cryoglobulins can be isolated monoclonal immunoglobulins (IgM) or IgM-IgG immunocomlexes. Ninety percent of Cryoglobulinemic vasculitis is caused by a heptatits _____ viral infection, but CV can also result from lymphoproliferative disorders (multiple myeloma or Waldenstrom macroglobulinemia) or autominnune diseases (systemic lupus erythematosus, rheumatoid arthritis, Sjogren syndrome). Patients are more frequently females 42-52 years old.

CV often has skin manifestations (most frequently lower extremity erythematous macules and purpuric papules), renal disease (most frequently membranoproliferative glomerulonephritis), and peripheral neuropathy.

The Meltzer _____ for identifying essential mixed cryoglobulinemia features purpura, arthralgia, and weakness. Cryoglobulinemia is treated with immune response suppression as well as treatment for the underlying cause. Positive Hepatitis _____ virus can be diagnostic and can be treated with INF-alpha and ribavirin. In Cryoglobulinemic Vasculitis C _____ is low & in Lupus, C3 & C4 are low.

Answer 26

Cryoglobulinemic Vasculitis is a small vessel vasculitis associated with serum cryoglobulins. This hypersensitivity vasculitis is characterized by cryoglobulin immune deposits in capillaries, venules, or arterioles, causing vasculitis. Cryoglobins are abnormal proteins in the blood that form crystals when the blood sample is cooled below 37 degrees Celsius (98.6 degrees Farenheit).

These cryoglobulins can be isolated monoclonal immunoglobulins (IgM) or IgM-IgG immunocomlexes. Ninety percent of Cryoglobulinemic vasculitis is caused by a heptatits C viral infection, but CV can also result from lymphoproliferative disorders (multiple myeloma or Waldenstrom macroglobulinemia) or autominnune diseases (systemic lupus erythematosus, rheumatoid arthritis, Sjogren syndrome). Patients are more frequently females 42-52 years old.

CV often has skin manifestations (most frequently lower extremity erythematous macules and purpuric papules), renal disease (most frequently membranoproliferative glomerulonephritis), and peripheral neuropathy.

The Meltzer triad for identifying essential mixed cryoglobulinemia features purpura, arthralgia, and weakness. Cryoglobulinemia is treated with immune response suppression as well as treatment for the underlying cause. Positive Hepatitis C virus can be diagnostic and can be treated with INF-alpha and ribavirin. In Cryoglobulinemic Vasculitis C4 is low & in Lupus, C3 & C4 are low.

Question 27

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) is a granulomatous, necrotizing small-vessel vasculitis. It is classified as a vasculitis of the _____- and _____-sized arteries, and is a multisystem disorder characterized by neuropathy, rhinosinusitis, pulmonary infiltrates, asthma, and prominent peripheral blood _____ philia.

The lungs and heart are particularly affected, as well as the skin. ANCA are found in 40-60 percent of patients with Churg-Strauss syndrome and a majority of patients that are ANCA-positive have _____ directed against myeloperoxidase with a perinuclear staining pattern, or P-ANCA.

There are no laboratory tests specific for Churg-Strauss syndrome, but _____ philia is characteristic, and a complete cell count, total eosinophil count, Ig _____ level, and ANCA test can be acquired. Diagnosis of Churg-Strauss syndrome is confirmed by lung biopsy or biopsy of other clinically affected tissues.

Answer 27

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) is a granulomatous, necrotizing small-vessel vasculitis. It is classified as a vasculitis of the small- and medium-sized arteries, and is a multisystem disorder characterized by neuropathy, rhinosinusitis, pulmonary infiltrates, asthma, and prominent peripheral blood eosinophilia.

The lungs and heart are particularly affected, as well as the skin. ANCA are found in 40-60 percent of patients with Churg-Strauss syndrome and a majority of patients that are ANCA-positive have antibodies directed against myeloperoxidase with a perinuclear staining pattern, or P-ANCA.

There are no laboratory tests specific for Churg-Strauss syndrome, but eosinophilia is characteristic, and a complete cell count, total eosinophil count, IgE level, and ANCA test can be acquired. Diagnosis of Churg-Strauss syndrome is confirmed by lung biopsy or biopsy of other clinically affected tissues.

Question 28

Henoch-Schӧnlein Purpura is a small vessel vasculitis caused by deposition of Ig _____ mediated immune complexes in capillaries, arterioles, and venules. It is a type of hypersensitivity vasculitis that most commonly affects _____.

Although the exact cause of HSP is unknown, it is usually triggered by _____ (e.g., group A strep, Shigella, Salmonella, EBV) or _____ exposure. HSP presents with a _____ of purpura, arthritis, and abdominal pain.

The purpura typically appear on the legs and _____ of the patient. Many patients have renal involvement which can progress to glomerulonephritis and renal failure. Treatment is primarily _____ and involves hydration, pain relief for arthritic/abdominal pain, and avoidance of any drugs that may have led to HSP symptoms.

Answer 28

Henoch-Schӧnlein Purpura is a small vessel vasculitis caused by deposition of IgA mediated immune complexes in capillaries, arterioles, and venules. It is a type of hypersensitivity vasculitis that most commonly affects children.

Although the exact cause of HSP is unknown, it is usually triggered by infection (e.g., group A strep, Shigella, Salmonella, EBV) or drug exposure. HSP presents with a triad of purpura, arthritis, and abdominal pain.

The purpura typically appear on the legs and buttocks of the patient. Many patients have renal involvement which can progress to glomerulonephritis and renal failure. Treatment is primarily supportive and involves hydration, pain relief for arthritic/abdominal pain, and avoidance of any drugs that may have led to HSP symptoms.

Question 29

Microscopic Polyangiitis:

Presentation: MPA can occur in patients of all genders, backgrounds, and ages (1). Patients may present with _____ itis, _____ with infiltrates in both lungs, and _____ uria due to glomerulonephritis (2). This disease can also cause peripheral nerve lesions, weight loss, skin lesions (palpable purpura), and fevers (1).

Mechanism: MPA is a _____ vessel vasculitis. The cause of MPA is unknown, but it seems to be an immune-mediated disorder. MPA is associated with anti-neutrophil cytoplasmic antibodies (_____-ANCA). In MPA, _____-ANCAs are typically directed against neutrophilic myeloperoxidase and proteinase 3 (1). _____-ANCAs destroy WBCs at “disease” sites and the release of WBC enzymes leads to damage within blood vessel walls (1). Ultimately, this leads to necrotizing vasculitis in many organs, but most notably the _____ and _____ (2).

Diagnosis: p-ANCA, ESR, and C-reactive protein (CRP) are _____ in this disease (1). However elevated p-ANCA and acute phase reactant levels are _____ disease specific. Urinalysis is may indicate renal involvement (hematuria and/or proteinuria) and CT may display lung infiltrates (1).

Significance to patient: Patients are treated with _____ (usually prednisone) and _____ or rituximab (1). These medicines carry major side effects like immunodeficiency (corticosteroids), hemorrhagic cystitis (cyclophosphamide), and a wide array of minimal toxicities (rituximab). Unfortunately, relapses are common (2).

Answer 29

Microscopic Polyangiitis:

Presentation: MPA can occur in patients of all genders, backgrounds, and ages (1). Patients may present with sinusitis, hemoptysis with infiltrates in both lungs, and hematuria due to glomerulonephritis (2). This disease can also cause peripheral nerve lesions, weight loss, skin lesions (palpable purpura), and fevers (1).

Mechanism: MPA is a small vessel vasculitis. The cause of MPA is unknown, but it seems to be an immune-mediated disorder. MPA is associated with anti-neutrophil cytoplasmic antibodies (p-ANCA). In MPA, p-ANCAs are typically directed against neutrophilic myeloperoxidase and proteinase 3 (1). p-ANCAs destroy WBCs at “disease” sites and the release of WBC enzymes leads to damage within blood vessel walls (1). Ultimately, this leads to necrotizing vasculitis in many organs, but most notably the lungs and kidneys (2).

Diagnosis: p-ANCA, ESR, and C-reactive protein (CRP) are elevated in this disease (1). However elevated p-ANCA and acute phase reactant levels are not disease specific. Urinalysis is may indicate renal involvement (hematuria and/or proteinuria) and CT may display lung infiltrates (1).

Significance to patient: Patients are treated with corticosteroids (usually prednisone) and cyclophosphamide or rituximab (1). These medicines carry major side effects like immunodeficiency (corticosteroids), hemorrhagic cystitis (cyclophosphamide), and a wide array of minimal toxicities (rituximab). Unfortunately, relapses are common (2).

Question 30

Takayasu arteritis is a _____, ANCA _____, vessel arteritis that usually affects _____ younger than 20 years. This is compared to Giant Cell arteritis, the other large vessel arteritis, that affects women older than 50 years. Takayasu affects the aortic _____ and it’s branch points. General vasculitis symptoms; fever, fatigue, myalgia, and signs of organ ischemia; can be seen.

Takayasu specific symptoms include _____ and neurological symptoms (due to the disease affecting the common carotid), but more classically: a weak or absent _____ in the upper extremities, due to vasculitis of the brachial artery. Thus, Takayasu is known as _____ less disease. Finally, as a sign of inflammation, the sed rate will be increased. Treatment for these patients is corticosteroids.

Answer 30

Takayasu arteritis is a large, ANCA negative, vessel arteritis that usually affects women younger than 20 years. This is compared to Giant Cell arteritis, the other large vessel arteritis, that affects women older than 50 years. Takayasu affects the aortic arch and it’s branch points. General vasculitis symptoms; fever, fatigue, myalgia, and signs of organ ischemia; can be seen.

Takayasu specific symptoms include visual and neurological symptoms (due to the disease affecting the common carotid), but more classically: a weak or absent pulse in the upper extremities, due to vasculitis of the brachial artery. Thus, Takayasu is known as pulseless disease. Finally, as a sign of inflammation, the sed rate will be increased. Treatment for these patients is corticosteroids.

Question 31

Polyarteritis Nodosa (PAN):

PAN is a necrotizing _____ vessel, ANCA _____, vasculitis involving many organs, but lungs are _____. There is hypertension via _____ artery involvement, abdominal pain with melena due to mesenteric artery involvement, neurologic disturbances, and skin lesions. PAN is associated with serum hepatitis _____ antigen.

Lesions of varying stages are present. Early lesions consist of transmural inflammation with fibrinoid necrosis, which eventually heals with fibrosis and produces a string of _____ appearance on imaging. Treatment = corticosteroids and cyclophosphamide (fatal if untreated). ESR & C-reactive protein will be elevated.

Answer 31

Polyarteritis Nodosa (PAN):

PAN is a necrotizing medium vessel, ANCA negative, vasculitis involving many organs, but lungs are spared. There is hypertension via renal artery involvement, abdominal pain with melena due to mesenteric artery involvement, neurologic disturbances, and skin lesions. PAN is associated with serum hepatitis B antigen.

Lesions of varying stages are present. Early lesions consist of transmural inflammation with fibrinoid necrosis, which eventually heals with fibrosis and produces a string of pearls appearance on imaging. Treatment = corticosteroids and cyclophosphamide (fatal if untreated). ESR & C-reactive protein will be elevated.

Question 32

Granulomatosis with polyangiitis (a.k.a. Wegener’s):
Wegner’s Granulomatosis is a _____ vessel necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys.

Classic presentation = middle aged male with _____ itis or nasopharyngeal ulceration, _____ with bilateral nodular lung infiltrates, and _____ due to glomerulonephritis. Serum _____-ANCA levels against proteinase 3 are elevated, & there is inflammatory urine sediment. Biopsy reveals large necrotizing granulomas with adjacent nectrotizing vasculitis. Treatment = steroids + cyclophosphamide. Relapses are common.

Answer 32

Granulomatosis with polyangiitis (a.k.a. Wegener’s):
Wegner’s Granulomatosis is a small vessel necrotizing granulomatous vasculitis involving the nasopharynx, lungs, and kidneys.

Classic presentation = middle aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to glomerulonephritis. Serum c-ANCA levels against proteinase 3 are elevated, & there is inflammatory urine sediment. Biopsy reveals large necrotizing granulomas with adjacent nectrotizing vasculitis. Treatment = steroids + cyclophosphamide. Relapses are common.

Question 33

Fibromuscular dysplasia, often abbreviated as FMD, is a non-atherosclerotic, non-inflammatory vascular disease that causes abnormal growth within the wall of an artery (dysplasia). It typically affects young women. FMD has been found in nearly every arterial bed in the body. However, the most common arteries affected are the _____ and _____ arteries.

• The main symptoms associated with renal FMD are _____ rtension and bruits audible over the abdomen or flanks. Complications such as aneurysms, dissections, or occlusion of the renal artery have been associated with renal artery FMD.
• Patients with FMD in the carotid arteries typically present around 50 years of age. Symptoms of craniocervical involvement include _____ (mostly migraine), pulsatile tinnitus, dizziness, and neck pain, although patients are often asymptomatic. On physical examination, one may detect _____ symptoms secondary to a stroke or transient ischemic attack (TIA), a bruit over an affected artery, and diminished distal pulses. Complications of cerebrovascular FMD include TIA, ischemic stroke, Horner’s syndrome, or subarachnoid hemorrhage.

Answer 33

Fibromuscular dysplasia, often abbreviated as FMD, is a non-atherosclerotic, non-inflammatory vascular disease that causes abnormal growth within the wall of an artery (dysplasia). It typically affects young women. FMD has been found in nearly every arterial bed in the body. However, the most common arteries affected are the renal and carotid arteries.

• The main symptoms associated with renal FMD are hypertension and bruits audible over the abdomen or flanks. Complications such as aneurysms, dissections, or occlusion of the renal artery have been associated with renal artery FMD.
• Patients with FMD in the carotid arteries typically present around 50 years of age. Symptoms of craniocervical involvement include headaches (mostly migraine), pulsatile tinnitus, dizziness, and neck pain, although patients are often asymptomatic. On physical examination, one may detect neurological symptoms secondary to a stroke or transient ischemic attack (TIA), a bruit over an affected artery, and diminished distal pulses. Complications of cerebrovascular FMD include TIA, ischemic stroke, Horner’s syndrome, or subarachnoid hemorrhage.

Question 34

Thromboangiitis obliterans (Buerger’s disease) is inflammatory thrombosis of _____ and _____ arteries and veins of the extremities in young adult _____. It is a necrotizing vasculitis involving the _____. _____ phenomenon is present; the vascular occlusion causes distal ischemia leading initially to digital ulceration and ultimately to gangrenous necrosis resulting over time in progressive amputation.

Unlike true vasculitis, there is no _____ to the vessel wall and there are _____ signs of a systemic inflammatory response. All of the inflammation, including the entire sequence from neutrophil accumulation through organization with granulation tissue and fibrosis, occurs within the thrombi. Though apparently a distinct clinical entity, there are no agreed upon criteria for diagnosis and it remains one of exclusion. Cessation of _____ stops the progression; no other therapy has been effective.

Answer 34

Thromboangiitis obliterans (Buerger’s disease) is inflammatory thrombosis of medium and small arteries and veins of the extremities in young adult smokers. It is a necrotizing vasculitis involving the digits. Raynaud phenomenon is present; the vascular occlusion causes distal ischemia leading initially to digital ulceration and ultimately to gangrenous necrosis resulting over time in progressive amputation.

Unlike true vasculitis, there is no damage to the vessel wall and there are no signs of a systemic inflammatory response. All of the inflammation, including the entire sequence from neutrophil accumulation through organization with granulation tissue and fibrosis, occurs within the thrombi. Though apparently a distinct clinical entity, there are no agreed upon criteria for diagnosis and it remains one of exclusion. Cessation of smoking stops the progression; no other therapy has been effective.