Clinical Notes in Vasculitic Diseases

CLINICAL NOTES

IN VASCULITIC

DISEASES

Dr. Faisael Albalwi

And

Dr. Ibrahim Alhomood

Dr. Fatemah Binladen

Pharmacist Fahad Albalawi

© Copyright 2022 Dr. Faisael Albalwi.

All rights reserved. No part of this publication may be reproduced, stored in a retrieval

system, or transmitted, in any form or by any means, electronic, mechanical, photocopying,

recording, or otherwise, without the written prior permission of the author.

ISBN:

978-1-6987-1232-1 (sc)

ISBN:

978-1-6987-1234-5 (hc)

ISBN:

978-1-6987-1233-8 (e)

Library of Congress Control Number: 2022913426

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this book may have changed since publication and may no longer be valid. The views

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Trafford rev.  11/12/2022

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CONTENTS

Dedication

Preface

(1) The First Chapter

Systemic Approach To The Patients With

Suspected Vasculitic Diseases:

(2) The Second Chapter

An Overview Of Vasculitic Diseases:

(3) The Third Chapter

Therapeutic Agents In Management Of Vasculitis

(4) The Fourth Chapter

Test Yourself (Short Real Cases)

References

DEDICATION

To my parents, the greatest teachers in the world

To my wife and my son Turki, the light of my life

To all Rheumatologists who have the interest in Vasculitis

PREFACE

This is a simplified book that concentrates on different vasculitic

diseases seen in the clinical practice. Hopefully, the book will guide

rheumatology trainees for better understanding of vasculitis.

(1) THE FIRST CHAPTER

SYSTEMIC APPROACH TO THE PATIENTS WITH

SUSPECTED VASCULITIC DISEASES:

**Systemic Vasculitic Disorders:

*Introduction:

–Vasculitis: systemic inflammatory process affecting the walls of blood vessels and causing wide spectrum of systemic manifestations.

–The manifestations of these vasculitic inflammatory disorders arise from one or both of the following mechanisms:

*Points should be considered when you are thinking about vasculitic diseases in ill patients:

1–Is it a primary vasculitic disorder or a mimicker condition?

2–If it is a primary vasculitis, what is the most likely subtype?

3–What is the extent of this vasculitic disease?

4–What are the tests that should be requested to confirm the diagnosis?

**Names for vasculitides adopted by the 2012 International Chapel Hill Consensus Conference on the Nomenclature of Vasculitides:

======================================

Large vessel vasculitis (LVV)

Takayasu arteritis (TAK)

Giant cell arteritis (GCA)

Medium vessel vasculitis (MVV)

Polyarteritis nodosa (PAN)

Kawasaki disease (KD)

Small vessel vasculitis (SVV)

Antineutrophil cytoplasmic antibody ANCA–associated vasculitis

(AAV):

Microscopic polyangiitis (MPA)

Granulomatosis with polyangiitis (Wegener’s) (GPA)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

(EGPA)

Immune complex SVV:

Anti–glomerular basement membrane (anti-GBM) disease

Cryoglobulinemic vasculitis (CV)

IgA vasculitis (Henoch-Schonlein Purpura) (IgAV)

Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q

vasculitis)

Variable vessel vasculitis (VVV)

Behcet’s disease (BD)

Cogan’s syndrome (CS)

Single-organ vasculitis (SOV)

Cutaneous leukocytoclastic angiitis

Cutaneous arteritis

Primary central nervous system vasculitis

Isolated aortitis

Others

Vasculitis associated with systemic disease

Lupus vasculitis

Rheumatoid vasculitis

Sarcoid vasculitis

Others

Vasculitis associated with probable etiology

Hepatitis C virus–associated cryoglobulinemic vasculitis

Hepatitis B virus–associated vasculitis

Syphilis-associated aortitis

Drug-associated immune complex vasculitis

Drug-associated ANCA-associated vasculitis

Cancer-associated vasculitis

Others

*Investigations should be ordered when you are seeing the patients suspected to have a primary vasculitic disorder:-

1–CBC with differentials: Leukocytosis or high platelets are seen among patients with active disease. Moreover, anemia of chronic disease might be noticed.

2–Renal and Hepatic profiles: to check if there is rise in serum creatinine or increment in hepatic enzymes.

3–Urinalysis and urine toxicology: to detect any abnormal urine sediment, and also twenty-four-hour urine protein collection is important.

4–ESR/CRP: These are not specific markers. However, they may rise during active disease.

5–Full septic screen: Extremely important to be done to avoid infectious process.

6–Hepatitis B or Hepatitis C serology: As they are associated with development of specific subtypes of vasculitic diseases.

7–HIV or varicella serology: May cause a clinical condition-like vasculitis (CNS vasculitis).

8–COVID-19 PCR: This is a newer viral agent that can cause different systemic condition including vasculitis mimickers.

9–Serology for syphilis: VDRL or TPHA. Patients with syphilitic disease can present with vasculitis (especially aortitis).

10–QuantiFERON-TB: It is considered an important infectious agent that can manifest with a picture of vasculitis-like phenomenon.

11–Autoimmune work-up should be done: ANA, complements, ANCA, and cryoglobulins.

12–Chest X-ray (CXR) and high-resolution CT-Chest (HRCT-chest): To recognize any lung lesions (infiltrate, nodules, cavity?).

13–Transthoracic echocardiogram: For assessment cardiac function and to rule out vasculitis mimicker like infective endocarditis.

*Confirmatory tests used to manage

primary vasculitic disorders:

(2) THE SECOND CHAPTER

AN OVERVIEW OF VASCULITIC DISEASES:

^^Large Vessels Vasculitis

Giant Cell Arteritis

*Introduction:

*Pathogenesis:

^inflammatory cells infiltrate with production of matrix metalloproteinase enzymes >>> leads to damage of tunica media and internal elastic lamina,

^vascular smooth muscle cells proliferation >>> leads to occlusion (Ischemia),

^stimulate hepatocytes for production of acute phase reactants, and

^VDGF-stimulation for neoangiogenesis >>> to help in reducing the symptoms of ischemia.

*Clinical Assessment:

–By history:

1–new onset (severe) headache\scalp pain which is located in the temporal or occipital areas (70 percent),

2–symptoms of stroke,

3–jaw, tongue claudication, or dysphagia,

4–visual symptoms like amaurosis fugax, considered the highest predictive factor for permanent vision loss (also diplopia and ptosis have been reported),

5–limbs claudication and easy fatiguability upon limb movement with involvement of aorta and its major branches (15–20 percent and could be silent!),

6–chest pain and upper back pain,

7–constitutional symptoms: weight loss, low-grade fever, chronic cough, and don’t forget to ask about any chronic comorbidities (DM, HTN),

8–don’t forget to ask about PMR symptoms (like hip or shoulder girdles stiffness), and

9–any points suggestive for malignancy.

–By Examination:

1–Examine for temporal areas tenderness, cordlike temporal artery, and reduced temporal artery pulses.

2–Look for scalp necrosis and assess for shoulder or hip girdles stiffness (not weakness).

3–Look for any difference in pulse or BP or vascular bruits.

4–Important to do: fundoscopy, or better to call an ophthalmologist to assess for retinal ischemia or papilledema (AION-arteritic).

5–Any signs for malignancy >>> lymph nodes enlargement.

*ACR 1990 diagnostic criteria for GCA (More than or equal to three) (Sen. 93.5 percent and Spe. 91.2 percent):

–age more than or equal to fifty years,

–new onset headache,

–temporal artery tenderness or decreased in pulsation,

–ESR more than or equal 50, and/or

–positive temporal artery biopsy.

*Investigations:

Basic laboratory:

1–CBC+diff. >>> Anemia of chronic disease or high platelets >>> positive phase reactant,

2–Renal profile and urine analysis >>> Any proteinuria! (Why?),

3–Hepatic profile: transaminitis or high ALP (mild) >>> should improve with treatment,

4–ESR: more than or equal 50 mm/hr in 88 percent or 10 percent of the patients with ESR < 50 (only 2–3 percent will have normal ESR!),

5–CRP is elevated in majority of the patients,

6–SPEP/UPEP rule out plasma cell dyscrasias, and

7–Hep. B, hep. C, HIV, or QuantiFERON-TB to be requested.

Basic Imaging (within one week after starting steroid)

1–Temporal artery duplex ultrasound +\- axillary artery >>> (non-compressible halo sign >>> Sen. 75 percent and Sp.83 percent),

2–High resolution cranial MRI can be used for diagnosis if ultrasound is not available, and to determine any narrowed segments in cranial arteries,

3–MRA-aorta, CTA, or PET-CT >>> used to detect aortitis in the aorta and its major branches’ involvement, and

4–transthoracic echo is important to know if there is any aortic valve dilation >>> (AR).

Pathological assessment (gold standard test):

**Temporal artery biopsy (within one week after starting steroid) is better to