Rheumatology Clinical Conference

Laura Kobashigawa, PGY-5

July 1st, 2024
 HPI

• 51yo F with PMHx ANCA vasculitis/MPA (dx 2016 +P-ANCA, MPO Ab, inflammatory
arthritis) c/b pauci-immune glomerulonephritis (diagnosed by renal biopsy 2019),
usual interstitial pneumonia (UIP) interstitial lung disease (ILD), peripheral
neuropathy
• Transferred from outside hospital for higher level of care for 2.8 x 2.5cm intracardiac
mass on tricuspid annulus
• Rheumatology consulted at LAGMC for concern for autoimmune etiology of
intracardiac mass
 HPI

• Brought to OSH after found unconscious on her bathroom floor

• Arrival OSH bradycardic, 2:1 block -> admitted ICU dopamine drip
• TTE with cardiac mass invading and encircling ostium of RCA
• Course c/b polymorphic VT and then PEA arrest, s/p ROSC
• Transvenous pacemaker placed and LHC -> 80-90% narrowing of RCA at ostium likely
from mass effect
• Transferred to LAGMC for higher level of care
 Ancillary History

PMH: ANCA vasculitis c/b pauci-immune glomerulonephritis (GN), UIP ILD, peripheral neuropathy

PSH: denies

Meds: cellcept 1500mg BID, plaquenil 300mg daily, lisinopril 2.5mg daily, ofev 150mg BID (prescribed
but was not taking)

Allergies: Rituxan (anaphylaxis)

FH: mother with leukemia. No family history of autoimmune disease

SH: denies smoking/alcohol/drugs. Lives with daughter at home in Los Angeles. Born in Mexico. Works
in housekeeping.
 Rheumatologic History

• Initial presentation 2016 (shortness of breath, joint pain)

• Diagnosed with UIP ILD in 2016
• History of flares 2/2 medication non-adherence
• MPA flare requiring hospitalization in 2019, s/p renal biopsy showing pauci-immune
glomerulonephritis
• Lost to follow-up in rheumatology clinic 8/2022-3/2024
• Medication history:
– Plaquenil 2016- 5/2024
– Prednisone- on and off since 2016
– Rituxan 2016-2017- discontinued due to anaphylaxis requiring Epinephrine
– Cellcept 2018- present
 Rheumatologic History

Previous serologies (2016)

P-ANCA 1:160 SSA/SSB negative

MPO 73.2 B2GP/LAC/Cardiolipin negative
PR3 negative RF 78 (H)
ANA negative CCP negative
Ds-DNA negative Scl-70 negative
C3 109 (nl) Centromere Ab negative
C4 19.5 (nl) Jo-1 negative
Sm negative GBM Ab negative
Sm/RNP negative ACE negative
 Review of Systems

Constitutional: +weight loss, +night sweats, dizziness

Eye: denies changes in vision, blurry vision
Respiratory: denies, +shortness of breath, sputum production
Cardiovascular: denies +chest pain
Gastrointestinal: denies abdominal pain, constipation
Genitourinary: denies dysuria, frequency, hematuria
Musculoskeletal: denies joint pain
Neurological: denies any numbness or tingling in extremities
Psychiatric: denies feeling depressed, hallucinations
Hematologic/lymphatic: denies easy bleeding or bruising
Allergy/immunologic: denies rash
 Physical Exam

Vital Signs: T 36.8 HR 84 RR 26 BP 95/60 O2 sat 99% RA Weight: 60 kg BMI 26

General: no acute distress, lying in bed

HEENT: MMM, pupils equal and reactive, EOMI, no oral ulcers, palpable small left supraclavicular lymph node
Cardio: RRR, no m/r/g, no elevated JVD
Pulmonary: tachypneic, crackles to mid lung fields bilaterally, on RA
Abdominal: Soft, nontender, nondistended, +BS, no hepatomegaly, no splenomegaly
Skin: warm, dry, intact, no rashes
Extremities: joints with no active synovitis, no lower extremity edema bilaterally
Neuro: cranial nerves grossly intact bilaterally, 5/5 strength throughout, sensation to light touch intact
bilaterally
 CBC

MCV 80.8

9.5
10.8 265
30.1
RDW 17.2%

N72.5 L19.8 M6.6 E0.5 Baso0.6

 CMP

BMP Liver Panel

135 100 18 7.5 28 0.5

147 117
4.1 22 0.89 3.2 30

Ca 8.7 Mag 2.1 Phos 4.3 PT 15.1 INR 1.22 Lactate 2.0
 Rheum Labs

P-ANCA 1:40 (1:320 ~2 months prior) IgA 514 (H)

MPO 44.8 (82.2 ~2 months prior) IgG 2,266 (H)
PR3 negative IgM 105 (nl)
ANA 1:80 nuclear, speckled
C3 113 (nl) ESR 104 (H)
C4 20 (nl) CRP 115.7 (H)
Cardiolipin Ab IgG and IgM negative
Beta 2 GP IgG and IgM negative UPCR= 0.70 (1.22 ~2 months prior)
ACE negative
 Additional Labs

Blood Cx negative Pro-BNP 25,641

Sputum Cx negative Troponin 97 -> 104 -> 102 -> 69
Procal 0.22
HIV negative
Hepatitis serologies negative
Quant gold negative
1,3 beta D glucan negative
Cocci Ab negative
Crypto Ag negative
EBV DNA negative
SPEP/UPEP negative
 Diagnostics
Transthoracic echocardiogram (TTE)
Summary:
1. Rhythm is complete heart block with right ventricular pacing.
2. Left ventricle: The cavity size is moderately increased. Wall thickness is normal. Systolic function is severely
reduced by the biplane method of disks. The estimated ejection fraction is 27%. The basal function is relatively
preserved, but the mid and apical walls are severely hypokinetic. Unable to assess diastolic function due to patient's
underlying heart rhythm.
3. Right ventricle: The cavity size is increased. Systolic function is moderately reduced. RVSP is at least 35mmHg
(assuming RAP 8).
4. Left atrium: The atrium is severely dilated.
5. Right atrium: The atrium is dilated. There is a large (~2.5x2.0cm) non-mobile, heterogenous mass abutting the
right atrial side of aortic root just above the septal leaflet of the tricuspid valve (seen best with Definity at end of
study). There is a mobile, filamentous echodensity in the right atrium that may be associated with the pacemaker vs
prominent Eustachian valve.
6. Mitral valve: There is mild to moderate regurgitation.
7. Tricuspid valve: There is mild-moderate regurgitation.
8. Pericardium, extracardiac: There is no pericardial effusion.
 Diagnostics Continued

Cardiac MRI
IMPRESSION:
1. Right atrial septal mass, which although appears immediately adjacent to the tricuspid does not
appear to affect cardiac function or valve function. As this mass is smoothly marginated with
enhancement similar to the myocardium and with diffusion restriction differential consideration include
benign neoplastic etiologies such as fibroma, myxoma, although malignant lesions cannot be excluded.
2. Normal left ventricular size and minimally reduced contractility. Calculated left ventricular ejection
fraction 36%.
3. Normal right ventricular size and contractility.
4. No delayed gadolinium enhancement.
 Diagnostics: CTPA

RV

RA LV
PA
LA Ao

RA
LV
RV
A
B

CT angiography pulmonary artery showing interatrial cardiac mass (red arrows) in axial (A) and coronal (B) views.
 Diagnostics: CTPA

CTPA 5/3/24

IMPRESSION:
1. Pulmonary emboli involving anterior and superior segmental and subsegmental branches of the left
upper lobe pulmonary artery. No evidence of right heart strain.
2. Patchy ground-glass and consolidative opacities with interlobular septal thickening and small bilateral
pleural effusions, which are new when compared to the outside hospital CT scan dated 4/29/24.
Findings are favored to reflect pulmonary edema. Superimposed infection is not excluded.
3. Basilar predominant reticular opacities with areas of architectural distortion and honeycombing in a
usual interstitial pneumonia pattern, which are slightly increased in extent when compared to the CT
scan dated 7/20/23.
4. Right atrial mass involving the tricuspid annulus, which is better characterized on echocardiography.
5. Few prominent hilar and mediastinal lymph nodes, which are nonspecific and may be reactive.
 Diagnostics: PET Scan

B C

PET scan showing increased uptake at the site of the cardiac mass (white arrow) in axial (A) and coronal (B) views.
Peribronchovascular consolidations with increased metabolic activity (white arrow) in the upper lobes (C).
 Diagnostics: PET Scan

PET Scan (5/3/24)

IMPRESSION:
1. Study is limited by extensive radiotracer extravasation in the right axilla.
2. Focus of increased FDG avidity in the region of the interatrial septum, likely corresponding to patient's
known intracardiac tumor.
3. Compared to the most recent CT chest from 4/29/24, interval development of bilateral peribronchovascular
consolidations, predominantly in the upper lobes, which demonstrate increased metabolic activity. This
finding is not specific and may reflect pneumonitis, consider correlation with bronchoscopy.
4. Multiple mildly enlarged lymph nodes in the chest with examples as described above, which demonstrate
mildly increased FDG avidity.
5. Redemonstrated cardiomegaly, with interstitial pulmonary edema superimposed on underlying fibrotic
changes.
6. Small right and trace left pleural effusions.
 Diagnostics Continued

• Biopsy of cardiac mass performed with pathology showing fragments of blood clot.
• Repeat biopsy 1 week later showing organizing thrombus.
• No evidence of myxoma, negative for amyloid, no malignant cells.
Diagnostics Continued

Cardiac biopsy #1 H&E 100: fragments of blood clot

 Diagnostics Continued

Cardiac biopsy #2 H&E 100x: organizing thrombus Cardiac biopsy #2 H&E 200x: organizing thrombus
 Hospital Course

• CTPA with subsegmental pulmonary emboli -> heparin gtt

• Cardiac biopsy #1 -> fragments of blood clot
• Developed chest pain with small pericardial effusion on bedside US -> started
on colchicine for treatment of suspected pericarditis
• Multi-disciplinary meeting with cardiology, oncology, pulmonology, and
rheumatology
• Cardiac biopsy #2 -> organizing thrombus
 Hospital Course Continued

• Patient started on empiric intravenous steroids (prednisone 1mg/kg

equivalent) given suspicion for mass due to vasculitis
• Surgery to excise the mass deferred given high risk procedure
• No further episodes of Mobitz I, II, or complete heart block -> pacemaker
placement deferred
• Discharged in stable condition on steroid taper with plan for repeat imaging
of cardiac mass as outpatient
 Post-Hospital Course

• Repeat cardiac MRI obtained 3 weeks after discharge with near resolution of
right atrial septal mass, improved ejection fraction 55%, and no delayed
gadolinium enhancement.
• Seen in Cardiology clinic 5 days ago, feeling well at that time. EKG normal
sinus rhythm with slightly prolonged PR interval -> beta blocker held and zio
patch ordered for monitoring
• Scheduled for rheumatology follow-up later today 7/1. Plan for likely
initiation of avacopan as outpatient.
 Diagnosis:

Intracardiac mass suspect 2/2 ANCA vasculitis

Cardiac Involvement in
ANCA Vasculitis
 Learning Objectives

At the conclusion of this presentation, participants will be able to:

1. Discuss the pathogenesis of ANCA-associated vasculitis (AAV)

and cardiovascular disease
2. Recognize intracardiac tumors as rare complications of AAV
3. Describe cardiac manifestations in patients with AAV
4. Discuss current guidelines for cardiac screening in patients with
AAV
 Overview

• Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a rare

autoimmune disease characterized by inflammation of the small and medium blood
vessels which leads to endothelial injury and end-organ damage
• Prevalence of approximately 400 per million people globally
• Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and
eosinophilic granulomatosis with polyangiitis (EGPA)
• Commonly involved organ systems include upper and lower respiratory tract, eyes,
lungs, kidneys and skin

Sayer M. Current Rheumatology Reports. 2024;26(1):12-23.

Overview

Sayer M. Current Rheumatology Reports. 2024;26(1):12-23.

Overview

Hellmich B. Annals of the Rheumatic Diseases. 2024;83(1):30-47.

 Overview

• A meta-analysis of over 14,000 patients with AAV reported a relative risk of 1.65 for
all cardiovascular events compared to the general population
• Higher Birmingham Vasculitis Activity Score, dyslipidemia, body-mass index (BMI)
and family history of cardiovascular (CV) events are predictive factors of CV disease
• Cardiovascular disease is the leading cause of death in AAV

Poledniczek M. SN Comprehensive Clinical Medicine. 2022;4(1).

 Risk of thrombosis

• Patients with AAV have a two-to threefold greater risk of developing venous as well
as arterial thrombotic events
• Endothelial injury is a key pathogenic event in AAV
• Antiphospholipid antibodies (APLs) have been detected in up to one third of AAV
patients -> further drives thrombosis
• Once thrombogenesis occurs, the homeostatic mechanisms resulting in clot
dissolution are further impaired in AAV due to anti-plasminogen antibodies

Misra D. Clinical Rheumatology. 202;40(12):4807-4815.

Pathogenesis

Misra D. Clinical Rheumatology. 202;40(12):4807-4815.

Pathogenesis

Sayer M. Current Rheumatology Reports. 2024;26(1):12-23.

 Cardiac Tumors

• Mass lesions are uncommon manifestations of GPA, mostly reported in the upper
respiratory tract, lungs or skin, and rarely in other organs such as the heart
• Only eight patients with cardiac masses were reported since 2010
• Patients were mostly women (75%), with a median age at the time of mass
detection of 45 years, and ANCA were positive in 75% of cases
• Cardiac mass occurred at the time of GPA onset in all cases and was associated with
systemic manifestations in all cases except one
• Cardiac masses were mostly associated with ENT involvement (85%) and less
frequently with lung and renal manifestations

Padoan R. Autoimmunity Reviews. 2022;21(3):103018.

 Cardiac Tumors

• Treatment and outcome

– Cardiac tumors usually surgically removed to make definitive diagnosis
– 4 patients underwent surgical excision followed by immunosuppressive
treatment with complete recovery
– 2 cases saw regression of intracardiac mass after therapy with cyclophosphamide
and glucocorticoid pulse
– 1 case with complete atrioventricular (AV) block due to mass located in
conduction system -> treated with immunosuppressive therapy and pacemaker
placement

Padoan R. Autoimmunity Reviews. 2022;21(3):103018.

Cardiac Tumors

Padoan R. Autoimmunity Reviews. 2022;21(3):103018.

 Cardiac Tumor: Case Report

• Case report published in Journal of the American College of Cardiology

• 75yo F referred for fatigue and weight loss
• Echocardiography: isoechogenic mass in interventricular septum
• Complete atrioventricular block -> sudden cardiac death
• Autopsy: white tumor-like mass and massive inflammatory fibrotic changes
• Systemic necrotizing vasculitis of small arteries throughout the body and elevated P-
ANCA of post-mortem blood samples
• Diagnosis: cardiac tumor secondary to AAV

Hara M. Journal of the American College of Cardiology. 2010;55(17):1882-1882.

Cardiac Tumor: Case Report

Hara M. Journal of the American College of Cardiology. 2010;55(17):1882-1882.

 Going Back to Our Patient

• Evidence of active disease on PET scan, elevated inflammatory markers,

positive P-ANCA/MPO
• Pulmonary emboli of unclear etiology (hypercoagulable 2/2 AAV? vs tumor
emboli from cardiac mass)
• Possible that initial endocardial injury occurred -> predisposed to
development of adherent thrombus -> formation of cardiac mass
• Responded to steroids (+/- anticoagulation?), resulting in regression of mass
in 1 month
 Cardiac Manifestations in AAV

• Pericarditis
• Valvular disease
• Myocardial disease
• Coronary artery disease
• Conduction abnormalities
 Pericarditis

• Retrospective review of AAV patients at a single referral institution from

January 1996 to November 2015 with diagnosis of pleuritis and/or pericarditis
• Study included patients with GPA (n=1,058), MPA (n=267), and EGPA (n=201)
• 88 of 1,058 patients (8.3%) with GPA, 27 of 267 (10.1%) with MPA, and 35 of
201 (17.4%) with EGPA had a manifestation of pleuritis and/or pericarditis
attributable to vasculitis
• Of those patients, pleuritis and/or pericarditis was a presenting feature in
81.4%
• Pericarditis frequently occurred concomitantly with general manifestations and
with pulmonary and nervous system involvement

Thompson G. Chest. 2021;160(2):572-581.

 Pericarditis

• Treatment of pericarditis included immunosuppressive therapy (52.1%), a

combination of symptom and immunosuppressive therapy (14.9%), or
symptom management only (18.1%)
• Procedural management of pericarditis occurred in 13.8% of patients

Thompson G. Chest. 2021;160(2):572-581.

Pericarditis

Thompson G. Chest. 2021;160(2):572-581.

 Valvular Disease

• Significant valvular disease is a rare manifestation of GPA

• Proposed mechanisms: accelerated degeneration, endocardial masses, and
rarely, valve perforation
• Infective endocarditis should always be excluded
• Studies have shown that up to 6% of patients with infective endocarditis were
found to be positive for both PR3-ANCAs and MPO-ANCAs

Borowiec A. Portuguese Journal of Cardiology. 2024;43(3):97-103.

 Valvular Disease

• Prospective observational study that evaluated 105 patients with GPA hospitalized
at an academic medical center in Poland between 2010 and 2020
• Aim: to assess cardiac valvular changes in patients with GPA (n=105) followed for a
mean of 6.2 years
• Collected laboratory studies, echocardiographic exams and review of all
cardiovascular (CV) adverse events during observation
• Aortic regurgitation (AR) was diagnosed in 43% of patients at baseline and was the
most common valvular lesion. AR was also found to significantly increase during the
observation period (p=0.01).
• However, only two (1.9%) of patients with AR required surgery due to significant
regurgitation

Borowiec A. Portuguese Journal of Cardiology. 2024;43(3):97-103.

Valvular Disease

Borowiec A. Portuguese Journal of Cardiology. 2024;43(3):97-103.

Valvular Disease

Borowiec A. Portuguese Journal of Cardiology. 2024;43(3):97-103.

 Arrhythmias and Conduction Abnormalities

• Atrial tachycardia, atrial fibrillation and flutter are the most common arrhythmias
that are found in patients with GPA
• Ventricular arrhythmias are usually noted in association with dilated
cardiomyopathy, cardiac ischemia or secondary to cardiac masses and are
uncommon in patients with no structural damage
• Conduction defects including varying degrees of atrioventricular (AV) heart block
may occur
 Diagnostic Tools

• Electrocardiogram (EKG)
• Echocardiography
• Cardiac magnetic resonance (CMR) imaging with late gadolinium
enhancement (LGE)- can detect focal myocardial fibrosis

Giollo A. International Journal Cardiovascular Imaging. 2021;37(3):1053-1062.

 Echocardiographic Features in AAV

• Retrospective study in Korea of 89 AAV patients evaluated with

echocardiography between 2010 and 2017 at an academic medical center
• Patients with GPA (n=17), MPA (n=49), and EGPA (n=23) included in the study
and were matched with age and gender controls
• Found that AAV patients exhibited lower mean left ventricle ejection fraction
(LVEF) (64.0 vs 69.1%, p=0.002) and higher right ventricle systolic pressure
(RVSP) (30.2 vs 23.2mmHg, p<0.001) compared to controls

Ahn S. Clinical Rheumatology. 2017;36(12):2751-2759.

Echocardiographic Features in AAV

Ahn S. Clinical Rheumatology. 2017;36(12):2751-2759.

Echocardiographic Features in AAV

Ahn S. Clinical Rheumatology. 2017;36(12):2751-2759.

 Cardiac MRI

• Single center, observational, cross sectional study in the UK that evaluated undiagnosed
cardiac involvement in patients with vasculitis between 18 and 80 years age with complete
remission of vasculitis (Birmingham Vasculitis Activity Score or BVAS <0) following induction
therapy and no prior diagnosis of cardiovascular disease (CVD) or diabetes
• Participants matched with healthy controls
• Primary objective: determine prevalence and pattern of subclinical cardiac involvement in
GPA as detected by CMR imaging
• Secondary objective: describe which GPA disease characteristics were associated with
cardiac abnormalities detected by CMR

Giollo A. International Journal Cardiovascular Imaging. 2021;37(3):1053-1062.

 Cardiac MRI

• Total of 26 patients with vasculitis underwent cardiovascular magnetic resonance (CMR) to

identify myocardial abnormalities in GPA and their correlation with disease phenotype
• Study found that late gadolinium enhancement (LGE) pattern of fibrosis was detected in 24%
of patients and no controls (p=0.010)
• LGE scar mass was higher in patients presenting with renal involvement
• Study concluded that asymptomatic GPA patients with well controlled disease may have
significant myocardial abnormalities on CMR, though clinical implications remain to be
determined
• CMR can be a useful tool for risk stratification of myocardial involvement in GPA

Giollo A. International Journal Cardiovascular Imaging. 2021;37(3):1053-1062.

Cardiac MRI

Giollo A. International Journal Cardiovascular Imaging. 2021;37(3):1053-1062.

 EULAR Guidelines

• European Alliance of Associations for

Rheumatology (EULAR) recommends
regular cardiovascular risk assessment in
patients with AAV

Hellmich B. Annals of the Rheumatic Diseases. 2024;83(1):30-47.

EULAR Guidelines

Hellmich B. Annals of the Rheumatic Diseases. 2024;83(1):30-47.

EULAR Guidelines

Hellmich B. Annals of the Rheumatic Diseases. 2024;83(1):30-47.

ACR Guidelines

Chung S. Arthritis Rheumatology. 2021;73(8):1366-1383.

 Going Back to Our Patient

• Cardiac involvement:
• AV block, polymorphic ventricular tachycardia
– Conduction abnormalities possibly 2/2 intracardiac mass? Or did arrythmias
predispose to clot formation?
• Endocardial injury -> thrombus -> intracardiac mass
• Plan for addition of avacopan as adjunct therapy to treat relapse
 Final Teaching Points

• Intracardiac tumors are a very rare complication of AAV and may respond to steroid
treatment alone +/- immunosuppressive therapy
• More common manifestations of cardiac disease in AAV patients include pericarditis,
valvular disease, and conduction abnormalities
• Cardiovascular disease is one of the leading causes of death in AAV patients
therefore timely treatment is imperative
• Current guidelines recommend screening for traditional cardiovascular risk factors
for patients with AAV in addition to baseline echocardiography for patients with
EGPA
 References
1. Ahn SS, Park ES, Jung SM, Song JJ, Park YB, Lee SW. Echocardiographic features in patients with ANCA-associated vasculitis within 3 months before and after diagnosis. Clin
Rheumatol. 2017 Dec;36(12):2751-2759. doi: 10.1007/s10067-017-3868-2. Epub 2017 Oct 8. PMID: 28988280.
2. Borowiec A, Rosinska M, Kowalik I, Rybski S, Chwyczko T, Jankowski J, Życińska K. Cardiac valvular involvement in granulomatosis with polyangiitis in long-term
observation. Rev Port Cardiol. 2024 Mar;43(3):97-103. English, Portuguese. doi: 10.1016/j.repc.2023.08.008. Epub 2023 Dec 19. PMID: 38122897.
3. Chung SA, Langford CA, Maz M, Abril A, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH,
Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA. 2021
American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis
Rheumatol. 2021 Aug;73(8):1366-1383. doi: 10.1002/art.41773. Epub 2021 Jul 8. PMID: 34235894
4. Giollo A, Dumitru RB, Swoboda PP, et al. Cardiac magnetic resonance imaging for the detection of myocardial involvement in granulomatosis with polyangiitis. Published
online 2021. doi:10.1007/s10554-020-02066-2
5. Hara M, Nishino M, Yamada Y. Cardiac Tumor-Like Mass in a Patient With Systemic Vasculitis. Journal of the American College of Cardiology. 2010;55(17):1882-1882.
doi:10.1016/j.jacc.2008.11.072
6. Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Annals of the rheumatic
diseases. 2024;83(1):30-47. doi:10.1136/ard-2022-223764
7. Misra DP, Thomas KN, Gasparyan AY, Zimba O. Mechanisms of thrombosis in ANCA-associated vasculitis. Clin Rheumatol. 2021 Dec;40(12):4807-4815. doi:
10.1007/s10067-021-05790-9. Epub 2021 Jun 9. PMID: 34109491; PMCID: PMC8189705.
8. Padoan R, Campaniello D, Gatto M, Schiavon F, Doria A. Current clinical and therapeutic approach to tumour-like mass lesions in granulomatosis with
polyangiitis. Autoimmunity reviews. 2022;21(3):103018-. doi:10.1016/j.autrev.2021.103018
9. Poledniczek MH. Coronary Artery Disease in Granulomatosis with Polyangiitis: a Review. SN comprehensive clinical medicine. 2022;4(1). doi:10.1007/s42399-022-01156-7
10. Sayer M, Chapman GB, Thomas M, Dhaun N. Cardiovascular Disease in Anti-neutrophil Cytoplasm Antibody-Associated Vasculitis. Current rheumatology reports.
2024;26(1):12-23. doi:10.1007/s11926-023-01123-8