VASCULITIS, AMYLOIDOSIS, IMMUNODEFICIENCY
(EXCEPT AIDS)
Ed Friedlander, M.D., Pathologist
scalpel_blade@yahoo.com

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Welcome to Ed's Pathology Notes, placed here originally for the convenience of medical students at my school. You need to check the accuracy of any information, from any source, against other credible sources. I cannot diagnose or treat over the web, I cannot comment on the health care you have already received, and these notes cannot substitute for your own doctor's care. I am good at helping people find resources and answers. If you need me, send me an E-mail at scalpel_blade@yahoo.com Your confidentiality is completely respected. No texting or chat messages, please. Ordinary e-mails are welcome.

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Pathology's modern founder, Rudolf Virchow M.D., left a legacy of realism and social conscience for the discipline. I am a mainstream Christian, a man of science, and a proponent of common sense and common kindness. I am an outspoken enemy of all the make-believe and bunk that interfere with peoples' health, reasonable freedom, and happiness. I talk and write straight, and without apology.

Throughout these notes, I am speaking only for myself, and not for any employer, organization, or associate.

Special thanks to my friend and colleague, Charles Wheeler M.D., pathologist and former Kansas City mayor. Thanks also to the real Patch Adams M.D., who wrote me encouragement when we were both beginning our unusual medical careers.

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Especially if you're looking for information on a disease with a name that you know, here are a couple of great places for you to go right now and use Medline, which will allow you to find every relevant current scientific publication. You owe it to yourself to learn to use this invaluable internet resource. Not only will you find some information immediately, but you'll have references to journal articles that you can obtain by interlibrary loan, plus the names of the world's foremost experts and their institutions.

Alternative (complementary) medicine has made real progress since my generally-unfavorable 1983 review. If you are interested in complementary medicine, then I would urge you to visit my new Alternative Medicine page. If you are looking for something on complementary medicine, please go first to the American Association of Naturopathic Physicians. And for your enjoyment... here are some of my old pathology exams for medical school undergraduates.

I cannot examine every claim that my correspondents share with me. Sometimes the independent thinkers prove to be correct, and paradigms shift as a result. You also know that extraordinary claims require extraordinary evidence. When a discovery proves to square with the observable world, scientists make reputations by confirming it, and corporations are soon making profits from it. When a decades-old claim by a "persecuted genius" finds no acceptance from mainstream science, it probably failed some basic experimental tests designed to eliminate self-deception. If you ask me about something like this, I will simply invite you to do some tests yourself, perhaps as a high-school science project. Who knows? Perhaps it'll be you who makes the next great discovery!

Our world is full of people who have found peace, fulfillment, and friendship by suspending their own reasoning and simply accepting a single authority that seems wise and good. I've learned that they leave the movements when, and only when, they discover they have been maliciously deceived. In the meantime, nothing that I can say or do will convince such people that I am a decent human being. I no longer answer my crank mail.

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More of Ed's Notes: Ed's Medical Terminology Page

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Courtesy of CancerWEB

STUDY OBJECTIVES

Describe the typical clinical pictures, gross and microscopic pathology, immunopathology, and pathophysiology of each of the following:

Explain the importance of making an accurate diagnosis of each of the diseases listed above, and tell how to go about doing it.

Define amyloid and give its composition and physical chemistry. Describe its light and electron microscopic appearances. Tell where to find it and how to recognize it. Recognize "primary amyloidosis" and "secondary amyloidosis" as archaic terms.

Given an amyloid derived from each of the following, tell its names, and describe the associated syndrome in some detail.

Describe where amyloid is deposited in each of these organs, and its effect on function:

Recognize possible amyloidosis patients. Tell how to make the diagnosis of systemic amyloidosis. Tell how to treat them.

Give the distinguishing features of each of the following hereditary immunodeficiencies:

Recognize and describe common variable immunodeficiency.

Mention the distinguishing features of the following hereditary deficiencies of the complement system:

Recognize each of the following using the microscope:

*  *  * 

Vasculitis Images
University of Washington
Pictures and comments

Immune Disease
Iowa Virtual Microscopy
Have fun

Immuno Pathology
Photos, explanations, and quiz
Indiana U.

KCUMB Students
"Big Robbins" -- Immuno
Lectures follow Textbook

POLYARTERITIS NODOSA ("periarteritis nodosa")

{16917} fibrinoid necrosis of artery (polyarteritis nodosa)
{53545} fibrinoid necrosis of artery (polyarteritis nodosa)

Polyarteritis Nodosa
From Chile
In Spanish

{16920} polyarteritis, angiogram showing aneurysms

{11987} polyarteritis nodosa, infarcts on the elbows
{11988} polyarteritis, gangrene

{46396} old polyarteritis nodosa, burned-out scarred lesion showing disrupted elastica (elastic stain, of course)
{16918} old polyarteritis nodosa, elastic stain
{16922} old polyarteritis nodosa, elastic stain (the old break in the internal elastic membrane is obvious; organized thrombus in the center stains with elastic stain also)

MICROSCOPIC POLYANGIITIS ("small vessel polyarteritis", anti-myeloperoxidase disease, p-ANCA disease, small-vessel polyarteritis)

WEGENER'S GRANULOMATOSIS ("pathergic granulomatosis"; "granulomatosis with polyangiitis"; Am. J. Med. Sci. 321: 76, 2001)

{40524} Wegener's; vasculitis lesion is in center
{40520} Wegener's; giant cells and damaged tissue
{40525} Wegener's; burned-out lesion (elastic stain, showing disrupted elastica)
{40526} Wegener's, vasculitis, note giant cell at top of picture
{40527} Wegener's; necrosis and giant cell
{11990} Wegener's, necrosis of elbow

Granuloma
?? Wegener's
KU Collection

Wegener's
Pittsburgh Pathology Cases

Wegener's
Lung pathology series
Dr. Warnock's Collection

CHURG-STRAUSS VASCULITIS

Churg-Strauss
Lung pathology series
Dr. Warnock's Collection

BEHÇET'S DISEASE (Behcet's disease; NEJM 341: 1284, 1999; Medicine 91: 18, 2012)

AMYLOIDOSIS

COMPOSITION OF AMYLOID AMYLOID NAME CLINICAL CORRELATIONS
Serum amyloid associated protein (acute phase reactant) Amyloid AA chronic inflammation (leprosy, osteomyelitis, tuberculosis, rheumatoid arthritis, etc.); New Guinea amyloid goiter
Immunoglobulin light chains Amyloid AL B-cell proliferations (plasma cell myeloma, benign hyperplasias)
Transthyretin ("prealbumin") Amyloid ATTR (AF) Substituted: Familial syndromes (Amyloid ATTR)

Non-substituted: Patchy deposits in the hearts of the elderly ("senile cardiac amyloidosis") or widespread in the elderly ("senile systemic amyloidosis" -- Arch. Int. Med. 165: 1425, 2005). Probably both are mild illnesses.

Various endocrine proteins Amyloid Esub Stromas of endocrine tumors (various ones; prolactin is APro, calcitonin is ACal, etc.)
Beta-two microglobulin Amyloid H (beta-2m) Chronic hemodialysis patients
Amyloid beta protein Amyloid beta / A4 Cores of Alzheimer senile plaques; cerebral blood vessels in Alzheimer's and some congophilic angiopathy patients
Tau microtubule protein tangles, plaques, and granules of Alzheimer's
Amylin (islet amyloid-associated peptide) Amyloid IAPP stroma of insulinomas; islands of some type II diabetics
Prolactin (An amyloid E) Old folks' pituitaries
Atrial natriuretic peptide/factor A-ANF Old folks' atria
* Semenogelin I ASgI Old guys' seminal vesicles
Keratin AKer ("Amyloid D") Some skin diseases
* Gamma trace alkaline microprotein (cystatin C) Hereditary congophilic angiopathy of Iceland
* Gelsolin AGel Familial Finnish (Am. J. Path. 126: 1223, 1990)
Prion Precursor Protein (PRPP) AScr Prion diseases
* Apolipoprotein A1 (Am. J. Path. 154: 221, 1999) AApoA1 Familial Iowa amyloid; dog amyloid
* OSMR ? Familial cutaneous amyloidosis (nodular / lichen / macular) Arch Derm 145: 695, 2009.

There are many, many other rare familial amyloidosis syndromes (Blood 90:4799, 1997).

Amyloid
Congo red stain
WebPath Photo

Amyloid
Bryan Lee

Portuguese Familial Amyloidosis
Brazil Pathology Cases
In Portuguese

Reviews: Postgrad. Med. 96: 119, 1994; Mayo Clin. Proc. 74: 490, 1999. Molecules NEJM 349: 583, 2003.

AMYLOID is a class of insoluble, homogeneous, eosinophilic ("hyaline") substances that accumulate in extracellular spaces. Their common link is that they are BETA-PLEATED SHEETS (rather than α-helices), and therefore cannot be effectively handled by the body.

AMYLOIDOSIS is a group of systemic and (?) localized diseases with varied and often bizarre clinical presentations.

Amyloid was named by Dr. Virchow, who noticed that iodine turned it orange-brown and subsequent application of sulfuric acid turned it purple-blue. Because starch undergoes the same color reactions, amyloid means "starch-like".

Most amyloids are altered forms of various proteins that exist in the healthy body.

{08129} Congo red
{08132} Congo red, birefringence

{17480} crystal violet stain, amyloid (red) in heart
{35978} crystal violet stain, rectal positive for amyloid (red)

{08159} iodine treatment has blackened amyloid in glomeruli
{08165} amyloid, histology, glomeruli
{07937} amyloid between myocardial cells, histology
{11021} amyloid in the kidney, thioflavin T (stains amyloid yellow and everything else black)
{16664} amyloid, electron micrograph
{17334} amyloid, electron micrograph

Renal Amyloidosis
Electron micrographs
VCU Pathology

CLASSIFICATION

AMYLOID DERIVED FROM IMMUNOGLOBULIN LIGHT-CHAINS (AMYLOID AL; "immunocyte-derived amyloidosis", "B-cell dyscrasia with amyloidosis", the old "primary amyloidosis" / amyloid B, etc.).

AMYLOID DERIVED FROM AAP PROTEIN (AMYLOID AA, "reactive systemic amyloidosis", the old "secondary amyloidosis"; *  "permanganate sensitive amyloidosis", "amyloid A", etc.; old review Medicine 70: 246, 1991; new review NEJM 356: 2361, 2007)

AMYLOID DERIVED FROM TRANSTHYRETIN (AMYLOID ATTR; old names have included ncluding "amyloid AF", "familial amyloid", "amyloidosis C", "old age amyloid", "senile amyloid" (Virchows Archiv. 442: 252, 2003), *  "amyloid Sc1"=senile cardiac, first type)

AMYLOID DERIVED FROM A HORMONE POLYPEPTIDE

AMYLOID COMPOSED OF BETA-2 MICROGLOBULIN ("Amyloid H"; Amyloid beta2M)

AMYLOID COMPOSED OF Aβ PROTEIN / AMYLOID BETA PROTEIN (Am. J. Path. 142: 1449, 1993)

AMYLOID COMPOSED OF INFECTIOUS PRIONS (PrP)

AMYLOID DERIVED FROM ATRIAL NATRIURETIC FACTOR ("AANF", "atriopeptin")

OTHER AMYLOIDOSIS SYNDROMES

{38620} amyloid, tongue
{22023} amyloid, cornea
{22026} amyloid, cornea, histology
{22029} amyloid, conjunctival surface, histology

Amyloidoma
Lung pathology series
Dr. Warnock's Collection

{38899} amyloidoma, histology; note foreign-body reaction

ORGAN INVOLVEMENT IN SYSTEMIC AMYLOIDOSIS SYNDROMES

{10880} renal amyloidosis, congo red
{10883} renal amyloidosis, congo red
{11021} renal amyloidosis, thioflavin T
{16785} amyloid in the glomerulus
{17171} amyloid in the glomerulus

Amyloidosis

WebPath Photo

{08111} amyloid, heart
{24519} amyloid heart, gross (looks and feels like candle wax)
{10685} congo red stain (my case)
{10688} congo red, polarized (my case again)

Amyloidosis
Heart
WebPath photo

Chronic rejection
Amyloidosis
Heart -- apple green

{03386} amyloid, coronary artery (left: Sirius stain; right: crystal violet)

{24551} amyloid in small bowel (vessels and outer layer of muscularis propria)
{10370} amyloid in gut wall (Congo red and unstained slices)

{39805} amyloid in the liver

{24607} amyloid, adrenal (looks and feels like candle wax)

Sago spleen
Urbana Atlas of Pathology

{12215} amyloid, eyelids

MAKING THE DIAGNOSIS

TREATMENT AND PROGONSIS

INTRODUCTION TO IMMUNODEFICIENCY

Immunodeficiency I
From Chile
In Spanish

Immunodeficiency II
From Chile
In Spanish

HEREDITARY IMMUNODEFICIENCIES ("primary immunodeficiencies"; reviews Ped. Clin. N.A. 47(6): Dec. 2000, whole issue; Mayo Clin. Proc. 73: 865, 1998; J. Allerg. Clin. Imm. 111(2S): S-571, 2003. molecular diagnosis of 75 of the ~100 known syndromes is now available, review Lancet 357: 1863, 2001; Nat. Imm. 5: 23, 2004; the Budapest Classification of 2005: J. Allerg. Clin. Imm. 117: 883, 2006.)

{53740} ataxia-telangiectasia, patient (all you can tell from this picture is this is some kind of nervous system disease)

COMMON VARIABLE IMMUNODEFICIENCY ("acquired hypogammaglobulinemia" -- update Mayo Clin. Proc. 80: 1187, 2005; molecules J. Allerg. Clin. Imm. 117: 740, 2006; for clinicians Lancet 372: 489, 2008) AND ITS KIN

GENETIC DEFICIENCIES OF THE COMPLEMENT SYSTEM

BIBLIOGRAPHY / FURTHER READING

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