Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

PBR: Rheumatology > Vasculidities > Flashcards

Vasculidities Flashcards

1
Q

What causes small vessel vasculitis?

A

Small vessel vasculitis is caused by immune complexes.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List four examples of small vessel vasculitis.

A

Drug reactions, serum sickness, Henoch-Schonlein purpura (HSP), and granulomatosis with polyangiitis (GPA; formerly known as Wegener’s).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

List two examples of medium vessel vasculitis.

A

Polyarteritis nodosa and Kawasaki disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the classic example of a large vessel vasculitis?

A

Takayasu arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the most common vasculitis in childhood?

A

Henoch-Schonlein purpura is the most common vasculitis in childhood.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the typical age range for Henoch-Schonlein purpura?

A

The mean age at diagnosis is 4 years, and >75% of those affected are <7 years of age. The age range is typically 3-15 years, but it can be seen in older adolescents and adults as well.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the gender distribution for Henoch-Schonlein purpura?

A

HSP affects boys more frequently, with a 2:1 male:female ratio.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

At what time of year is Henoch-Schonlein purpura more prevalent?

A

There are more cases of HSP in the winter and spring.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What immune molecule mediates HSP?

A

HSP is an immune-mediated leukocytoclastic vasculitis with neutrophil infiltration and deposition of primarily IgA in vessel walls, along with small amounts of IgG and Complement 3.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is Henoch-Schonlein purpura?

A

HSP is an immune-mediated small vessel vasculitis of childhood that presents with purpura and involvement of multiple organ systems, including joints, the GI system, and the renal system most commonly.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is a predisposing factor in 50% of HSP cases?

A

The specific cause of HSP is unknown, but in about 50% of cases, an upper respiratory tract infection precedes the onset of disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Describe the classic skin findings in patients with Henoch-Schonlein purpura.

A

Skin lesions are present in all HSP patients. The rash begins as small wheals or red maculopapules that progress to petechiae and palpable purpura. They are generally found in dependent, pressure-bearing areas such as the lower extremities and buttocks, but can be located in other places as well. The skin lesions last anywhere from 4 days to 4 weeks.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How common is joint involvement in patients diagnosed with Henoch-Schonlein purpura?

A

Joint involvement occurs in 50-80% of patients with HSP.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Describe the typical pattern of joint involvement in patients with Henoch-Schonlein purpura.

A

The arthritis/arthralgia is transient and mainly involves the large joints, particularly the knees and ankles. Joint effusions do not usually occur.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the most common musculoskeletal manifestation of Henoch-Schonlein purpura?

A

Periarthritis, with edema around the joints and inflammation involving the tendon sheaths.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the most typical GI manifestation in patients with Henoch-Schonlein purpura?

A

The most typical GI manifestation is abdominal pain that is colicky and sometimes involves vomiting.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How common are GI bleeds in patients with Henoch-Schonlein purpura?

A

Occult bleeding is common in those with abdominal pain, and melena can occur. Hematemesis can occur but is less typical. Major GI bleeds are uncommon.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What do you do if a child with HSP has persistent, severe, abdominal pain? What do you suspect as the etiology?

A

Perform an ultrasound to evaluate for intussusception, which can occur in 2-14% of patients.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What system are you concerned about for up to 6 months after diagnosis of HSP? What is the recommended course of action?

A

The renal system. Serial urinalyses should be performed for at least 3-6 months after HSP is diagnosed to monitor for renal involvement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How do the renal manifestations of HSP usually present?

A

Renal manifestations are usually mild and transient. They appear as isolated microscopic hematuria or hematuria + proteinuria on urinalysis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What causes the renal manifestations of HSP?

A

IgA deposition in the kidneys.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Which patients with HSP are at higher risk for development of permanent renal damage?

A

Children with purpura that lasts >1 month; children with severe, persistent GI symptoms; and children who have decreased Factor 13.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

How does one make the diagnosis of Henoch-Schonlein purpura?

A

HSP is a clinical diagnosis without specific confirmatory lab tests. Look for the classic purpura in addition to renal, GI, and joint manifestations.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the typical management of HSP?

A

There is no specific therapy for HSP, and management is mostly supportive. Corticosteroids may be used for ulcerated skin lesions. NSAIDs may be used for pain control if kidney function isn’t a concern. Systemic corticosteroids are reserved for patients with severe abdominal pain or severe scrotal swelling/edema.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is the typical duration of symptoms in patients with HSP?

A

HSP is a self-limited condition and lasts 4 weeks in about 65% of children.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

How common are recurrences of HSP during the first two years?

A

HSP recurs in up to 40% of patients from 6 weeks to 2 years after initial presentation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What is Granulomatosis with Polyangiitis?

A

GPA is very rare in children. It is characterized by necrotizing granulomatous vasculitis of small vessels involving the upper and lower respiratory tracts and kidneys (pulmonary-renal syndrome).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

List 8 common symptoms in patients with granulomatosis with polyangiitis.

A

Fever, weight loss, arthralgia or migratory large joint arthritis, cough, nasal stuffiness, epistaxis, resistant ear infections, and persistent sinusitis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the nasal deformity seen in granulomatosis with polyangiitis?

A

Saddle nose ***include image 20-5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

List four possible lung findings in patients with granulomatosis with polyangiitis.

A

Nodules, infiltrates, hemoptysis, and pleuritis.

31
Q

What laboratory test is useful in diagnosing granulomatosis with polyangiitis?

A

Finding cytoplasmic ANCA (c-ANCA specific for PR3 [protein 3]) aids in the diagnosis, as >90% of patients with diffuse disease and ~50% of those with limited disease have c-ANCA positivity.

32
Q

Chronic use of which drug prevents relapses and infection in patients with granulomatosis with polyangiitis?

A

Trimethoprim/sulfamethoxazole decreases the risk for PCP pneumonia and prevents relapses in patients with GPA.

33
Q

What four drugs may be used in the treatment of granulomatosis with polyangiitis?

A

Steroids, methotrexate (especially for limited disease), rituximab, and cyclophosphamide.

34
Q

What is the leading cause for acquired heart disease in children in the U.S.?

A

Kawasaki disease.

35
Q

Which age group is most commonly affected by Kawasaki disease?

A

Kawasaki disease generally occurs in children <5 years of age.

36
Q

What is the male:female ratio in Kawasaki disease?

A

Boys are affected more commonly than girls, with a ratio of 1.5:1.

37
Q

At what time of year is Kawasaki disease most likely to occur?

A

Kawasaki disease occurs year-round, but typically has clusters in the winter and spring.

38
Q

Which ethnic group is at highest risk for development of Kawasaki disease?

A

Incidence is highest in children of Asian descent.

39
Q

What is the main cause of death in Kawasaki disease? When does this usually occur?

A

Myocardial infarction is the main cause of death and most commonly occurs during the first year after onset of illness.

40
Q

What are the diagnostic criteria for Kawasaki disease?

A

Fever for at least 5 days and at least 4 of the following 5 findings: Bilateral conjunctival injection without exudate; rash; changes in the lips or oral cavity; changes in the peripheral extremities; and cervical lymphadenopathy (>1.5cm).

41
Q

What is the typical appearance of the rash associated with Kawasaki disease?

A

It is typically macular and polymorphous in character with no vesicles, scaling, or crusting. It is found on the trunk and is frequently more prominent in the perineal area later in the course. Desquamation of the affected area occurs later in the disease course.

42
Q

What are some (3) typical oral abnormalities in patients with Kawasaki disease?

A

Red pharynx, dry fissured lips, and/or an injected strawberry tongue.

43
Q

What are the most common peripheral extremity changes seen in patients with Kawasaki disease?

A

Redness and swelling of the hands/feet, with subsequent desquamation of the fingers/toes.

44
Q

When might one be able to make a diagnosis of Kawasaki disease without at least 4 of the 5 diagnostic findings?

A

If a patient has fever and coronary aneurysm, a diagnosis of Kawasaki may be made even if the patient doesn’t meet full criteria otherwise.

45
Q

What gallbladder abnormality is seen in Kawasaki disease?

A

Hydrops of the gallbladder

46
Q

What are the cardiac manifestations of Kawasaki disease?

A

Nearly 1/3 of patients have pericardial effusions, and myocarditis is also common. Coronary artery abnormalities (aneurysms) can occur as early as day 3 of disease, but are more typically seen from 10 days to 4 weeks after onset.

47
Q

List 6 risk factors which increase risk for coronary aneurysm development in patients with Kawasaki disease.

A

Age < 1 year, male gender, fever >16 days, cardiomegaly, arrhythmias, and fever recurrence after 48 hours of being afebrile.

48
Q

When would you be most likely to see thrombocytosis in a patient with Kawasaki disease?

A

Thrombocytosis most commonly occurs after day 7 of illness.

49
Q

What is the treatment for Kawasaki disease?

A

IVIG at a dose of 2 g/kg as a single infusion over 12-14 hours as well as aspirin. Aspirin is initially dosed at 80-100 mg/kg/day until IVIG response is achieved and fever resolves, then decreases to low-dose therapy (3-5 mg/kg/day), which is continued until cardiac involvement is ruled out.

50
Q

What are potential therapies available for Kawasaki disease that is refractory to IVIG?

A

Infliximab and cyclosporine.

51
Q

When should ECHO be performed in patients with Kawasaki disease?

A

Every patient should have an ECHO at time of diagnosis and another ECHO 6-8 weeks later. ECHO may be performed more frequently if cardiac abnormalities are identified.

52
Q

What is polyarteritis nodosa (PAN)?

A

PAN causes inflammation of medium-sized arteries and results in a focal segmental necrotizing vasculitis.

53
Q

When is polyarteritis nodosa most likely to occur in children?

A

PAN is rare in children, but occurs at a mean age of about 9 years.

54
Q

What is the male:female ratio in polyarteritis nodosa (PAN)?

A

Males are more frequently affected, with a male:female ratio of 2:1.

55
Q

How does polyarteritis nodosa typically present in children?

A

PAN typically presents with constitutional symptoms (fever, fatigue, anorexia), musculoskeletal findings, and renal disease.

56
Q

Which organ system is typically spared in polyarteritis nodosa?

A

There is an absence of lung involvement in patients with PAN.

57
Q

When would you be most likely to see orchitis in a patient diagnosed with polyarteritis nodosa?

A

Orchitis occurs most commonly in those with concomitant Hepatitis B infection.

58
Q

List 8 criteria which may be helpful in making a diagnosis of polyarteritis nodosa.

A

Diagnosis of PAN is difficult, but is usually based on the following criteria: skin lesions (purpura, livedo reticularis), testicular pain/orchitis, mononeuritis multiplex (presenting as a foot drop), renal involvement, HTN, evidence of Hepatitis B, weight loss, and biopsy or angiographic findings.

59
Q

How is polyarteritis nodosa typically treated?

A

Treat with steroids and immunosuppressive agents. Prognosis is poor without aggressive treatment.

60
Q

Differentiate between the renal involvement characteristic of polyarteritis nodosa vs that of microscopic polyangiitis.

A

PAN is a medium vessel vasculitis that causes aneurysms and stenosis, resulting in hematuria and renovascular HTN. PAN does not cause glomerulonephritis, whereas microscopic polyangiitis ( a small-vessel disease) does.

61
Q

What is Takayasu arteritis?

A

It is a granulomatous vasculitis of large vessels that leads to arteritis of the aorta and its major branches, resulting in weak or absent pulses in the upper extremities. It is very rare in children in the U.S., but is the 3rd most common childhood vasculitis in Japan, after HSP and Kawasaki.

62
Q

How is Takayasu arteritis typically treated?

A

Glucocorticoids and cyclophosphamide are the mainstay of therapy, and some studies have shown benefit from TNF or IL-6 blockade in patients refractory to initial management.

63
Q

How is Takayasu arteritis diagnosed?

A

Angiographic abnormalities of the aorta or main brainches (as demonstrated by CT or MRA) + at least one of the following: decreased peripheral pulses and/or claudication of the extremities; blood pressure difference of >10mmHg between arms; audible bruits over aorta and/or major branches; and HTN.

64
Q

What is the classic triad of Behcet disease?

A

Oral ulcers (painful, recurrent), genital ulcers (painful, recurrent), and inflammatory eye disease.

65
Q

How does Behcet disease differ from most other vasculitides?

A

Behcet disease is unlike any other vasculitis in that it can involve blood vessels of any size and type (including arteries or veins).

66
Q

Behcet disease is found more commonly in people of what descent?

A

It is much more common in children of Mediterranean or Far Eastern descent.

67
Q

Which skin lesions are commonly seen in Behcet disease?

A

The key finding in Behcet is the presence of recurrent buccal aphthous ulcers, which are found in nearly 100% of patients.

68
Q

How does one make the diagnosis of Behcet disease?

A

The diagnosis is clinical and requires observation of recurrent oral ulceration at least 3x over a 1 year period + at least 2 of the following: recurrent genital ulceration, eye lesions, skin lesions, or positive pathergy test.

69
Q

What is the pathergy test?

A

You prick the skin with a needle, and after 48 hours you see a pustule or papule surrounded by redness for a positive result.

70
Q

How is Behcet disease usually treated?

A

Treat initially with corticosteroids. Some patients with ulcerative manifestations benefit from colchicine and pentoxifylline. Azathioprine can be used to treat severe vasculitis with CNS or eye involvement. Infliximab helps treat colitis.

71
Q

What is CNS vasculitis?

A

An inflammatory disease affecting the blood vessels of the brain. It may be primary or secondary.

72
Q

What is childhood primary angiitis of the CNS (cPACNS)?

A

It is a primary form of CNS vasculitis which can present with arterial stroke in children.

73
Q

How do patients with small-vessel cPACNS usually present?

A

Seizures, psychiatric manifestations, and/or diffuse neurological deficits.

74
Q

How do patients with medium/large-vessel cPACNS usually present?

A

HA, focal deficits, movement disorders, cranial neuropathies, and arterial ischemic strokes.

PBR: Rheumatology (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions