SOI V - Módulo 15: Reumatologia

Prof. Lays Gama - ED 04: Vasculites

Roteiro:
1. Arterite temporal: Giant Cell Arteritis
2. Granulomatose de Wegener: Granulomatosis with polyangiitis (GPA)
3. Sindrome de Churg Strauss: Eosinophilic granulomatosis with polyangiitis (EGPA;
Churg-Strauss)
4. Poliarterite nodosa: Polyarteritis nodosa (PAN)
5. Arterite de Takayasu: Takayasu’s Arteritis
6. Doença de Kawasaki: Kawasaki’s Disease

in the past GCA [Giant Cell Arteritis] was commonly referred to as temporal arteritis. However,
replacement of the term temporal arteritis with GCA was motivated by an increased recognition
that GCA can affect many other extracranial large arteries, including the aorta and its branches

Wegener’s granulomatosis was replaced by granulomatosis with polyangiitis

1907 a 1976
Imprimir:
● 1907 a 1915 (9)
● 1918 a 1938 (21) = 30
● 1945 a 1958 (14) = 44
● 1967 a 1976 (10) = 54

Arterite temporal:
● Firestein: 1918 a 1932?

Arterite de Takayasu
● Firestein: 1932 a 1938

Granulomatose de Wegener
● Firestein: 1948 a

Introdução à definição, classificação e diagnóstico das arterites

1. Vasculite (definição)
Vasculitis is inflammation of blood vessel walls

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

2. Quais as 2 principais referências de sistemas de nomenclatura das vasculites?

1ª e 2ª International Chapel Hill Consensus Conference (CHCC) on the Nomenclature of
Systemic Vasculitides

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

3. O que faz, em essência, um sistema de nomenclatura?

A nomenclature system gives the name that should be used to define a specific disease
process

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

4. Qual a função de um critério de classificação?

Essencialmente, a pesquisa: Classification criteria are a set of standardized observations that
are intended to create a relatively homogeneous cohort of patients for clinical research

5. Diferencie critério de classificação do critério diagnóstico

- Classification criteria aim to have high specificity while a lower priority is placed on sensitivity.
more important in the study of groups of patients, allowing comparison of results across studies
and ensuring appropriate enrollment into clinical trials

- Diagnostic criteria are a set of symptoms, signs, and tests that guide the care of individual
patients and must be high in both sensitivity and specificity

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

6. Jannette JC et al, em 2013, exemplificou com "necrotizing granulomatous pulmonary

inflammation" a diferença entre definição e critério diagnóstico. Faça a mesma diferenciação
In the appropriate clinical context, cavitary lung lesions documented by imaging studies can be
a sufficient surrogate criterion to conclude that a patient has necrotizing granulomatous
pulmonary inflammation even if tissue has not been examined histologically
Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

7. Na nomenclatura da CHCC 2012, o que são "Large Vessels"?

Large-vessel refers to the aorta and its main branches and the corresponding veins

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

8. Na nomenclatura da CHCC 2012, o que são "Medium Vessels"?

Main visceral arteries and veins such as renal, mesenteric, and coronary arteries

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

9. Na nomenclatura da CHCC 2012, quais são as "Small Vessels"?

Intraparenchymal arteries, arterioles, capillaries, venules, and veins

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

10. O que caracteriza uma vasculite em cada uma das classificações de tamanho?
The vessel-size category is based on the predominantly affected vessel size, but vasculitides in
all the major categories can involve any sized artery

11. Cite as duas Large vessel vasculitis (LVV)

Takayasu arteritis (TAK)
Giant cell arteritis (GCA)

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

12. Diferencie as duas Large vessel vasculitis (LVV)

The histopathologic features of Takayasu arteritis and giant cell arteritis are indistinguishable
(Some have suggested that they are the same disease)

Takayasu arteritis: disease predominantly of younger individuals

Giant cell arteritis: to be a disease predominantly of older individuals
Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

13. Defina: Takayasu arteritis (TAK)

TAK is arteritis, often granulomatous, predominantly affecting the aorta and/or its major
branches. Onset usually occurs before the age of 50 years

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

14. Defina: Giant cell arteritis (GCA)

GCA is arteritis, often granulomatous and usually affecting the aorta and/or its major branches,
with a predilection for the branches of the carotid and vertebral arteries. Giant cells are
frequently but not always observed in biopsy specimens from patients with active GCA.
Onset usually occurs after age 50

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

15. Cite as duas maiores variantes de Medium vessel arteritis

Polyarteritis nodosa and Kawasaki disease are the major variants

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

16. Defina: Polyarteritis nodosa (PAN)

Necrotizing arteritis of medium or small arteries without glomerulonephritis or vasculitis in
arterioles, capillaries, or venules, and not associated with
antineutrophil cytoplasmic antibodies (ANCAs).

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

17. Kawasaki disease (KD)

Arteritis associated with the mucocutaneous lymph node syndrome and predominantly affecting
medium and small arteries. Coronary arteries are often involved. Aorta and large arteries may
be involved. Usually occurs in infants and young children.
Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

18. Cloze: The 2 categories of SVV [Small vessel vasculitis] are characterized by [[paucity of
vessel wall immunoglobulin in one, and a prominence of vessel wall immunoglobulin in the
other.]]

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

19. Deferencie ANCA-associated vasculitis (AAV) de Immune complex SVV

The small number or lack of immune deposits in vessel walls that is characteristic of AAV differs
from the moderate to marked vessel wall immune deposition that is characteristic of immune
complex SVV

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

20. Defina: Granulomatosis with polyangiitis (Wegener’s) (GPA)

Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract,
and necrotizing vasculitis affecting predominantly small to medium vessels (e.g., capillaries,
venules, arterioles, arteries and veins). Necrotizing glomerulonephritis is common.

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

21. Defina: Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)

Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract,
and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with
asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism
Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)

Giant cell arteritis: the patient’s perspective

Giant cell arteritis: The perspective of a patient

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

01. American College of Rheumatology Classification Criteria for Giant Cell Arteritis
[Table 93.1]

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

02. Quais as artérias mais afetadas na Giant Cell Arteritis em pacientes 50+?
The disease affects primarily the extracranial branches of the carotid artery in patients older
than 50 years
Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

03. Cloze: [Polymyalgia rheumatica], a term suggested by Barber, is a syndrome characterized

by [aching in the proximal portions of the extremities and torso]

04. Polymyagia rheumatica is defined by its clinical features. Why?

No specific diagnostic tests or pathologic findings exist
05. The features included in most definitions of PMR are:

(1) aching and morning stiffness lasting half an hour or longer in the shoulder, hip girdle, neck,
or a combination;
(2) duration of these symptoms for 1 month or longer;
(3) patient age older than 50 years; and
(4) laboratory evidence of systemic inflammation such as an elevated erythrocyte sedimentation
rate (ESR)

06. Além das características clínicas incluídas na maioria das definições de PMR, algumas
definições incluem:

Some definitions also include a rapid response to small doses of glucocorticoids, such as
prednisone 10 mg/day

07. Sigla: PMR = Polymyalgia Rheumatica

08. Sigla: GCA = Giant Cell Arteritis

MANIFESTAÇÕES CLÍNICAS

09. Quais as manifestações clínicas mais comuns de GCA?

The most common manifestations of GCA are constitutional symptoms, headache, visual
symptoms, jaw claudication, and PMR

10. Sinônimos: Arterite temporal: Giant Cell Arteritis

11. Quase todos os pacientes de GCA apresentam um ou mais sintomas constitucionais,

incluindo:
Fatigue, weight loss, malaise, and fever.

12. Cloze: [In GCA,] Abnormalities of the temporal artery includes:

Enlargement, nodular swelling, tenderness, or loss of pulse
13. Cloze: Visual symptoms are common in GCA, especially [loss of vision and diplopia]

14. Qual é, geralmente, o mecanismo fisiopatológico da perda da visão?

Loss of vision often reflects an anterior ischemic optic neuropathy caused by occlusive arteritis
of the posterior ciliary artery, the chief blood supply to the head of the optic nerve.
 Lâmina 81A. Colocar no baralho de anatomia

15. Qual a aparência em fundoscopia, no contexto da cegueira causada por anterior ischemic
optic neuropathy?
“The early funduscopic appearance [...] is that of ischemic optic neuritis: slight pallor and edema
of the optic disc, with scattered cotton-wool patches and small hemorrhages.”
 The optic disc is pale and swollen, the retinal veins are dilated, and several flame-shaped
hemorrhages and a cotton-wool spot (retinal infarct) are visible

16. Anki de anatomia/semiologia do fundo do olho e fundoscopia

17. Cloze: In GCA, “intermittent claudication may occur in [the muscles of mastication
(jaw claudication), the extremities, and, occasionally, the muscles of the tongue or those
involved in swallowing]”

18. Explique: “the chewing gum test”

Detection of jaw claudication
Jaw discomfort is provoked by the patient chewing gum at a rate of one chew per second for 2-3
minutes

19. Em GCA, qual a porcentagem, aproximadamente, de pacientes com manifestações clínicas

consideradas atípicas?
“Approximately 40% of patients are seen with disease manifestations that are considered
atypical”

20. O que seria um paciente com manifestações atípicas de GCA?

In these patients, headache, jaw claudication, visual symptoms, and PMR do not occur or are
less prominent.

21. Entre as manifestações atípicas de GCA, cite as manifestações neurológicas

Peripheral neuropathy
Transient ischemic attack, stroke
Dementia
Delirium

22. Quais os problemas neurológicos mais comuns em pacientes com GCA?

Neuropathies and transient ischaemic attacks or strokes

23. Em GCA, qual a causa da hemiparesia ou brain stem events?

Result of narrowing or occlusion of the carotid or vertebrobasilar artery

24. [Anki de anatomia com a artéria vertebrobasilar]

25. Cloze: Among the vasculitides, GCA has a nearly unique propensity for involving the [C5
nerve root], resulting in [loss of shoulder abduction]

26. [Cloze] GCA: Prominent respiratory tract symptoms occur in approximately [10%] of patients

27. [Cloze] GCA: Prominent respiratory tract symptoms include cough with or without sputum,
sore throat, and hoarseness.

28. Por qual mecanismo a vasculite pode causar sintomas do trato respiratório?
Ischemia or hyperirritability of the affected tissues

29. [Cloze]: Otolaryngeal manifestations of GCA include [throat pain, dental pain, tongue pain,
glossitis, and ulceration or infarction of the tongue.]

30. GCA clinicamente evidente pode ser dividida em quais estágios?

Early (within a year of diagnosis) and late (years after diagnosis) stages

31. GCA: Early disease consists chiefly of large artery stenosis resulting in [upper extremity
claudication; bruits over the carotid, subclavian, axillary, and brachial arteries; absent or
decreased pulses in the neck or arms; and Raynaud’s phenomenon] (Manifestações clínicas)
32. GCA: Late disease most frequently involves aneurysm in which region?
Thoracic aortic aneurysm

33. GCA, prognóstico: risk of thoracic aortic aneurism

Thoracic aortic aneurysm is 17 times more likely to develop in patients with GCA than in people
without this disease

Obs.: To place this risk in context, thoracic aortic aneurysms are twice as likely to complicate
GCA as lung cancer is to result from smoking

34. GCA, prognóstico: risk of abdominal aortic aneurysm

Abdominal aortic aneurysm is also 2.4 times more common in patients with GCA.

35. [Cloze] In women, GCA can uncommonly present as [a breast or ovarian mass]

36. Além dos sintomas constitucionais, qual o sintoma mais comum de GCA?
Cefaléia

36. Diferencie a febre da GCA da febre da PMR

- 15% of GCA patients may present with fever of unknown origin (FUO) in which the
temperature spikes are high, dominating the clinical picture
- High, spiking fevers are uncommon in PMR in the absence of GCA

37. “The diagnosis of PMR is clinical and depends on eliciting the symptoms and findings noted
earlier. Two sets of criteria for the diagnosis have been proposed”. Cite os 2 critérios existentes
Diagnostic Criteria of Chuang and Colleagues (1982)
Diagnostic Criteria of Healey (1984)

38. Para PMR, qual o critério diagnóstico de Chuang e colaboradores?

- Age 50 yr or older
- Bilateral aching and stiffness for 1 month or more and involving two of the following areas:
neck or torso, shoulders or proximal regions of the arms, and hips or proximal aspects of the
thighs
- ESR >40 mm/hr
- Exclusion of all other diagnoses except giant cell arteritis

39. Para PMR, qual o critério diagnóstico de Healey?

● Pain persisting for at least 1 month and involving two of the following areas: neck,
shoulders, and pelvic girdle
● Morning stiffness lasting >1 hr
● Rapid response to prednisone (≤20 mg/day)
● Absence of other diseases capable of causing the musculoskeletal symptoms
 ● Age older than 50 yr
● ESR >40 mm/hr

DIAGNOSTIC WORK-UP

40. Em que pacientes suspeitar de GCA?

- The diagnosis of GCA should be considered in any patient older than 50 years who
experiences loss of vision, diplopia, new form of headache, jaw claudication, PMR, FUO,
unexplained constitutional symptoms, anemia, and a high ESR.
- GCA can cause so many forms of cranial discomfort (e.g., headache, scalp tenderness, jaw
claudication, pain of the throat, gums, and tongue) that the disease should also be considered in
any patient older than 50 years in whom new, unexplained “above-the-neck” pain
develops

41. Cite 4 sintomas que aumentam o likelihood ratio de GCA

Jaw claudication
Diplopia
Weight loss
Any headache

42. Cite 4 sinais que aumentam o likelihood ratio de GCA

Beaded temporal artery
Tender temporal artery
Any temporal artery abnormality
Scalp tenderness

43. Qual resultado laboratorial aumenta o likelihood ratio de GCA?

ESR > 50 mm/hr

44. Sigla: ESR = erythrocyte sedimentation rate

● Anki explicando o que é isso

45. Cloze: The presence of [both jaw claudication and double vision] had a 100% positive
predictive value for a diagnostic temporal artery biopsy.

46. A presença/ausência de quais achados diminuem o likelihood de GCA?

Absence of headache or temporal artery abnormalities on physical examination
Presence of synovitis
Normal ESR
47. Jaw claudication is not pathognomonic for GCA. Cite duas exceções à regra
Anti-neutrophil cytoplasmic antibody (ANCA)–associated granulomatous vasculitis (AGV)
Systemic amyloidosis

48. Qual o padrão-ouro para diagnóstico de GCA?

Temporal artery biopsy is the “gold standard” for diagnosing GCA

49. Você suspeita de GCA. O que fazer para continuar a investigação?

Biópsia de artéria temporal
Se não houver contra-indicação: início do tratamento com glicocorticóide

50. Paciente com suspeita de GCA retorna com biópsia unilateral negativa. É possível
descartar GCA? Como você continuaria a investigação?
- Se jaw claudication ou diplopia → repetir exame
- Se sintoma principal é cefaléia occipital → solicitar biópsia de artéria occipital
- Se arm claudication, unequal arm blood pressures e sopros axilares ou supraclaviculares →
solicitar angiograma, MRA (magnetic resonance angiography) ou CT (sugestivo de doença
subclávia/axilar)
- Se nenhuma das situações acima → não repetir biópsia. GCA provavelmente descartado

51. Paciente com manifestações clínicas que sugerem GCA, mas apresentou biópsias
negativas e imagens não sugestivas (muito raro). O que fazer?
Pessoalmente, encaminho pra reumato continuar investigação com condições que se
assemelha a GCA

52. Qual o principal objetivo do tratamento de GCA?

Previnir perda da visão

53. Most authorities recommend [starting glucocorticoid therapy] as soon as the diagnosis of
GCA is strongly suspected

- Obs.: Anki de farmaco - Qual a classe do tocilizumabe?

Inibidor de IL-6

54. Paciente sem perda aguda de visão tem suspeita de GCA. Nega alergias, outras
comorbidades e uso de outras medicações. Realize a prescrição medicamentosa apropriada.
Prednisona 60mg/dia (ou glicocorticóide equivalente)

55. Paciente com perda aguda de visão tem suspeita de GCA. Nega alergias, outras
comorbidades e uso de outras medicações. Realize a prescrição medicamentosa apropriada.
Solumedrol, IV, 1000 mg/d por 3 dias
 Giant Cell Arteritis: Breaking Down Barriers to Optimal Management

Tenho que ter a categoria “traduza” no baralho de termos desconhecidos

Fonte: (2013) Jannette JC et al. 2012 Revised International Chapel Hill Consensus Conference
Nomenclature of Vasculitides. Arthritis & Rheumatism

Fonte: (2021) Firestein GS et al. Firestein & Kelley’s Textbook of Rheumatology, 11ª ed. Elsevier

Termos desconhecidos

- Insuficiência renal