Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly affecting medium arteries. It can be idiopathic or associated with hepatitis B virus (HBV) infection. The document discusses the epidemiology, pathogenesis, clinical presentation, diagnostic criteria and tests, imaging findings, and prognosis of PAN. It highlights that PAN affects medium-sized arteries and is differentiated from other vasculitides by the absence of glomerulonephritis, ANCA positivity, or involvement of arterioles/capillaries. HBV vaccination and certain drugs have been associated with PAN in some cases.
By Dr. Usama Ragab, Zagazig Faculty of Medicine
PSC incidence ranges from 0.5 to 1.25 cases/100 000.
The prevalence of the disease ranges between six and 20 cases/100 000.
Men are more likely to be affected (70%).
Prevalence of PSC may be increased in first degree relatives of PSC patients
This was a review of different guidelines on lupus nephritis from ACR, EULAR, and KDIGO. Goal is appreciate similarities and differences between the different guidelines.
By Dr. Usama Ragab, Zagazig Faculty of Medicine
PSC incidence ranges from 0.5 to 1.25 cases/100 000.
The prevalence of the disease ranges between six and 20 cases/100 000.
Men are more likely to be affected (70%).
Prevalence of PSC may be increased in first degree relatives of PSC patients
This was a review of different guidelines on lupus nephritis from ACR, EULAR, and KDIGO. Goal is appreciate similarities and differences between the different guidelines.
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic Dilemmpateldrona
The incidence of renal cell carcinoma (RCC) in patients with polycystic kidney disease (PCKD) has shown to be higher compared to the general population [1, 4]. However the incidence of RCC in young individuals (<40 years old) is extremely rare compared to older patients...
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic Dilemmclinicsoncology
The incidence of renal cell carcinoma (RCC) in patients with polycystic kidney disease (PCKD) has shown to be higher compared to the general population [1, 4]. However the incidence of RCC in young individuals (<40 years old) is extremely rare compared to older patients...
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic DilemmAnonIshanvi
The incidence of renal cell carcinoma (RCC) in patients with polycystic kidney disease (PCKD) has shown to be higher compared to the general population [1, 4].
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic DilemmSarkarRenon
The incidence of renal cell carcinoma (RCC) in patients with polycystic kidney disease (PCKD) has shown to be higher compared to the general population [1, 4]. However the incidence of RCC in young individuals (<40 years old) is extremely rare compared to older patients...
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic DilemmAnnalsofClinicalandM
The incidence of renal cell carcinoma (RCC) in patients with polycystic kidney disease (PCKD) has shown to be higher compared to the general population
Abnormal Renal Mass in Adult Polycystic Kidney Disease: A Diagnostic Dilemmkomalicarol
A 24-year-oldAfrican American man with history of polycystic
kidney disease, autism and schizophrenia was admitted to our
hospital for nausea, vomiting and one episode of mild hematuria
for 1 week. He was afebrile at presentation. Urinalysis was negative.
There was no leukocytosis on admission. His renal function tests
were within normal limits. CT scan (with contrast) of the abdomen
was acquired showing innumerable bilateral renal cysts (Figure 1).
One of which measured 4.5 x 5.2 cm as a heterogeneous mass with
epicenter in the superior pole of the left kidney showing extension
into renal hilum. This was most concerning for RCC.
Abdominal Pain as Initial Presentation of Lung Adenocarcinomaasclepiuspdfs
Isolated celiac lymph node metastasis (ICLNM), in general, is not common with a reported incidence of 5–10% for lung adenocarcinoma. Lung adenocarcinoma rarely metastasizes to the celiac lymph node leading to abdominal pain. It is not typical for ICLNM to be the initial presentation of lung adenocarcinoma as well. In this case, a 56-year-old man presented with a 4-week history of persistent periumbilical dull pain. Workup was remarkable for celiac lymph node mass which turned out to be adenocarcinoma with unknown primary cancer. Whole body position emission tomography scan and biopsy of the mass with immunohistochemistry could identify lung adenocarcinoma as the primary cancer. After a well-informed discussion of options for chemotherapy drugs with the patient, the decision was made to pursue bevacizumab combined with chemotherapeutics. He was charged home with abdominal pain relief and outpatient follow-up after short-course of chemotherapy.
Predictive Value of Biomarkers Fibrinogen Like Protein-2 and A-Fetoprotein fo...JohnJulie1
Data concerning the utility of biomarkers for accurate early HCC detection in cirrhotic patients are lacking. 1.2. Methods: We evaluated 112 consecutive Caucasian cirrhotic patients with (n=28) or without (n=84) concomitant HCC at baseline for serum AFP and plasma fibrinogen like protein-2 (FGL-2) levels. Patients without confirmed HCC at baseline were further followed up every six months with ultrasound and serum AFP levels, according to HCC surveillance program. Imaging as well as histological confirmation of HCC was established in patients with new lesions.
Predictive Value of Biomarkers Fibrinogen Like Protein-2 and A-Fetoprotein fo...NainaAnon
Data concerning the utility of biomarkers for accurate early HCC detection in cirrhotic patients are lacking. 1.2. Methods: We evaluated 112 consecutive Caucasian cirrhotic patients with (n=28) or without (n=84) concomitant HCC at baseline for serum AFP and plasma fibrinogen like protein-2 (FGL-2) levels. Patients without confirmed HCC at baseline were further followed up every six months with ultrasound and serum AFP levels, according to HCC surveillance program. Imaging as well as histological confirmation of HCC was established in patients with new lesions. Du
Predictive Value of Biomarkers Fibrinogen Like Protein-2 and A-Fetoprotein fo...EditorSara
Data concerning the utility of biomarkers for accurate early HCC detection in cirrhotic patients are lacking. 1.2. Methods: We evaluated 112 consecutive Caucasian cirrhotic patients with (n=28) or without (n=84) concomitant HCC at baseline for serum AFP and plasma fibrinogen like protein-2 (FGL-2) levels. Patients without confirmed HCC at baseline were further followed up every six months with ultrasound and serum AFP levels, according to HCC surveillance program. Imaging as well as histological confirmation of HCC was established in patients with new lesions. Du
Predictive Value of Biomarkers Fibrinogen Like Protein-2 and A-Fetoprotein fo...EditorSara
Data concerning the utility of biomarkers for accurate early HCC detection in cirrhotic patients are lacking. 1.2. Methods: We evaluated 112 consecutive Caucasian cirrhotic patients with (n=28) or without (n=84) concomitant HCC at baseline for serum AFP and plasma fibrinogen like protein-2 (FGL-2) levels. Patients without confirmed HCC at baseline were further followed up every six months with ultrasound and serum AFP levels, according to HCC surveillance program. Imaging as well as histological confirmation of HCC was established in patients with new lesions
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
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Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
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Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
4. PAN
1866 “periarteritis nodosa” used to describe any form of systemic
vasculitis.
Vasculitis predominantly affecting medium arteries defined as the
main visceral arteries and their branches.
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).”
5. Epidemiology
Incidence: 2 - 9 per million in Europe and the US (ACR criteria)
16 per million (by CHCC definition) in Kuwait
77 per million described in an Alaskan population endemic for HBV (based on only
13 actual cases of HBV PAN – study before ACR criteria or CHCC definitions)
Changed since vaccination for HBV: incidence of HBV-related PAN follows HBV
infection rates: 7% to 38.5% of patients dg with PAN
PAN develops in 1% to 5% of patients with HBV infection, confers an approximately
1000-fold increase in risk compared with the background population
Watts RA et al. Epidemiology of vasculitis. In Bridges L, Ball G, editors: Vasculitis, Oxford, UK, 2008, Oxford University Press, pp 7–22.; el-Reshaid
K et al: The spectrum of renal disease associated with microscopic polyangiitis and classic polyarteri- tis nodosa in Kuwait, Nephrol Dial Transplant
12:1874–1882, 1997. McMahon BJ et al. Hepatitis B-associated polyarteritis nodosa in Alaskan Eskimos: clinical and epidemiologic features and
long-term follow-up, Hepatology 9:97–101, 1989.
6. Pathogenesis in HBV- PAN
Direct vessel injury by replicating virus or deposition of immune
complexes leads to activation of the complement cascade,
resulting in an inflammatory response and subsequent
endothelial damage.
The vasculitis typically occurs within the first few months
following HBV infection and may be the first presenting feature
Mechanism supported by treatment strategy to eradicate HBV
with antiviral therapy and removal of immune complexes by
plasmapheresis without the need for long-term
immunosuppression.
7. Pathogenesis for idiopathic PAN
PAN responds to immunosuppressive therapy suggests an immunologic
mechanism.
Evidence of endothelial dysfunction, an increase in inflammatory
cytokines, and an increase in expression of adhesion molecules.
Propensity for the inflammatory lesions to occur at the sites of
bifurcation in vessels, where there is most likely to be turbulent flow.
Following the inciting event, there is focal and segmental necrotizing
inflammation of medium- and small-sized arteries. This leads to
intimal proliferation with subsequent thrombosis, resulting in
ischemia or infarction of the organ or tissue supplied by these arteries.
11. Cutaneous PAN
Implies a separate entity from PAN limited to the skin, but there is
some uncertainty as to whether these are actually just early cases of
PAN and whether progression to PAN will occur.
Pathologically the findings on skin biopsy are indistinguishable
In a retrospective review of cutaneous PAN patients from a Japanese
group, 22 patients with histologically proven cutaneous vasculitis were
followed: 32% had a peripheral neuropathy, and 27% had myalgia,
suggesting a need to revise the current criteria to differentiate
between the two entities of cutaneous PAN and PAN
HCV infection has been associated with cutaneous PAN-31/161 (19%) dg
with PAN
Furukawa F. Cutaneous PAN: an update. Ann Vasc Dis. 2012;5 (3) 282-8
Saadon D et al. Hepatitis C ssociated PAN. Arthritis Care Res (Hoboken). 2011 Mar;63(3):427-35
12. Labs that support the diagnosis of
PAN
TEST Support diagnosis
Elevated ESR/ CRP +
Elevated Creatinine (w/out
hematuria, proteinuria, cell casts)
+/- (suggest renal ischemia)
Abnormal liver test + (suggest HBV/ liver ischemia)
Anemia + (chronic inflammation/ blood
loss)
CPK + (normal/slightly elevated despite
muscle involvement )
13. Labs that are against PAN
TEST Against the diagnosis
ANCA + (GPA/ MPA)
Blood Cultures + (endocarditis/ infectious vasculitis)
HCV + (HCV associated cryoglobulinemia)
RF, CCP + RF
ANA, dsDNA + SLE/CTD
HIV +
14. Imaging
Angiogram = imaging of choice shows multiple small aneurysms, vessel
ectasia, and focal occlusive lesions in medium-sized vessels, typically
in the renal and mesenteric arteries.
Sensitivity -89% and specificity 90%
MR/ CT angiography are less invasive but are much less sensitive in
demonstrating microaneurysms.
Doppler ultrasound can identify renal and hepatic aneurysms
CXR –excludes other vasculitides that may affect the lungs and also to
exclude infection
Hekali P, et al: Diagnostic significance of angiographically observed visceral aneurysms with regard to polyarteritis nodosa, Acta Radiol
32:143–148, 1991. Ozaki K, et al: Renal involvement of polyarteritis nodosa: CT and MR findings, Abdom Imaging 34:265–270, 2009.
Ozcakar ZB, et al: Polyarteritis nodosa: successful diagnostic imaging utilizing pulsed and color Doppler ultrasonography and computed
tomography angiography, Eur J Pediatr 165:120–123, 2006.
15. Coronary
angiogram
25-year-old F dg with PAN 3 years
earlier and was receiving
prednisolone maintenance therapy
when she presented with cardiac
arrest. Although severe cardiac
involvement in polyarteritis
nodosa is unusual, it can result in
myocardial infarction and confers
a poorer prognosis. Despite
treatment, the patient died a few
months later.
http://www.nejm.org/image-
challenge?ci=08092012
16. Renal angiogram
47-year-old man with abdominal
pain, weight loss, and an elevated
ESR
Right renal arteriogram reveals
multiple microaneurysms within the
upper pole of the kidney on this
selective right renal artery injection
(upper pole branch)
17. ACR criteria 1990
3/10 criteria
Sensitivity 82%; Specificity 86%
Did not differentiate PAN from MPA or GPA
18. PAN and drugs
Minocycline (many case reports)
Levamisole (antihelmintic, immunomodulator –withdrawn
from US market in 2003 ) –SE agranulocytosis and
erythematous painful papules on the skin; Thiabendazole
Abatacept-cutaneous PAN to one arm
Gemcytabine
Hep B vaccination (child)
Interferon tx for HepC
19. PAN & drugs - references
Minocycline-induced polyarteritis nodosa-like vasculitis presenting as brainstem stroke. Klaas JP, Matzke T, Makol A, Fulgham JR. J
Clin Neurosci. 2015 May;22(5):904-7. doi: 10.1016/j.jocn.2014.12.003. Epub 2015 Mar 14.
Minocycline-induced polyarteritis nodosa-like vasculitis. Agur T, Levy Y, Plotkin E, Benchetrit S.Isr Med Assoc J. 2014 May;16(5):322-
3. No abstract available.
Eur J Dermatol. 2013 Sep-Oct;23(5):738-9. doi: 10.1684/ejd.2013.2160.Cutaneous polyarteritis nodosa localized to the arm
receiving an infusion of abatacept Shibata S1, Asano Y, Sato S.
Levamisole-induced vasculitis with ecchymosis and necrosis syndrome from contaminated cocaine. Desai N, Patel M, Desai S, Cerceo
E. BMJ Case Rep. 2012 Nov 21;2012. pii: bcr2012007319. doi: 10.1136/bcr-2012-007319. No abstract available.
Thiabendazole-induced acute liver failure requiring transplantation and subsequent diagnosis of polyarteritis nodosa. Groh M,
Blanche P, Calmus Y, Guillevin L. Clin Exp Rheumatol. 2012 Jan-Feb;30(1 Suppl 70):S107-9. Epub 2012 May 11.
Eur J Dermatol. 2009 Jul-Aug;19(4):400-1. doi: 10.1684/ejd.2009.0695. Epub 2009 May 25. Cutaneous polyarteritis nodosa in a child
following hepatitis B vaccination. Ventura F, Antunes H, Brito C, Pardal F, Pereira T, Vieira AP.
20. PAN and Hairy cell leukemia
(HCL)
There is a well-recognized association between HCL and PAN [Fortin,
1996].
Direct evidence linking the two conditions with histopathology
showing direct invasion of the vessel wall by leukemic cells [Hasler et
al. 1995].
In a literature review of 42 cases of vasculitis associated with HCL, 21
cases were consistent with PAN ( 4 cases showed vessel wall
infiltration by the leukemic cells).
PAN often occurred after the diagnosis of HCL and splenectomy.
Fortin P.R. (1996) Vasculitides associated with malignancy. Curr Opin Rheumatol 8: 30–33
Gabriel S.E., Conn D.L., Phyliky R.L., Pittelkow M.R., Scott R.E. (1986) Vasculitis in hairy cell leukemia: Review of literature and
consideration of possible pathogenic mechanisms. J Rheumatol 13: 1167–1172
Hasler P., Kistler H., Gerber H. (1995) Vasculitides in hairy cell leukemia. Semin Arthritis Rheum 25: 134–142
21. PAN and the risk of cancers
In a series of 115 patients with HBV-PAN, 3 patients (2.6%) died from
cancer over a mean follow-up of 69 months ( 2 lung, 1 prostate cancer)
[Guillevin et al. 2005].
In a prospective, randomized, open-label treatment study that included
58 patients with non-HBV PAN, three PAN patients (5.2%) developed
cancer over follow-up [Ribi et al. 2010] (to CS vs azathioprine alone) -- 1
Hodgkin's lymphoma and 2 colon cancer ( The cancers occurred 19 mo, 10
mo and 4 years after inclusion in the study)
348 patients with PAN with mean follow up of 68.3 months, there were 5
cancer-related deaths (1.4%): 1 lung, 1 liver, 2 prostate cancers, and 1
myelodysplasia occurring 4—19 years after PAN diagnosis [Pagnoux et al.
2010].
Pagnoux C.et al. (2010) Clinical features and outcomes in 348 patients with polyarteritis nodosa: A systematic retrospective study
of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum 62:
616–626
Ribi C., et al. (2010) Treatment of polyarteritis nodosa and microscopic polyangiitis without poor-prognosis factors: A prospective
randomized study of one hundred twenty-four patients. Arthritis Rheum 62: 1186–1197
Guillevin L. et al. (2005) Hepatitis B virus-associated polyarteritis nodosa: Clinical characteristics, outcome, and impact of
22. Prognostic
prognostic tool designed for use to
distinguish patients with higher or lower
risk of poor outcome.
Allocating 1 point per item, there is a
reduced survival for patients with higher
scores (86% survival for 0 points, 69%
survival for 1 point, 47% survival for 2 or
more points) at onset when followed up for
6 years.
Older age at diagnosis has been shown to
be an important adverse predictor of
survival in the first year and after 5 years
of follow-up
Bourgarit A, Le Toumelin P, Pagnoux C, et al: Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic
polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients, Medicine
(Baltimore) 84:323–330, 2005. Guillevin L, Lhote F, Gayraud M, et al: Prognostic factors in polyar- teritis nodosa and Churg-Strauss syndrome: a
prospective study in 342 patients, Medicine (Baltimore) 75:17–28, 1996.
Five Factor Score
Proteinuria (<1 g/day),
Elevated creatinine
(>1.58mg/dL)
Gastrointestinal involvement
CNS involvement
Cardiomyopathy
23. Treatment
Uncontrolled vasculitis accounts for 58% -73% of deaths
occurring within the first year
Management of non-HBV PAN should be appropriate to
the severity of the disease as defined by the five-factor
score
Five factor score > 1 aggressive immunosuppression
with corticosteroids and cyclophosphamide
Gayraud M, Guillevin L, le Toumelin P, et al: Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and
Churg-Strauss syndrome: analysis of four prospective trials including 278 patients, Arthritis Rheum 44:666–675, 2001.
25. HBV related PAN
High-dose corticosteroids followed by combination
plasma exchange and antiviral agents
Reduce early mortality from vasculitis and late
mortality from the consequences of chronic
hepatitis
Guillevin L, Mahr A, Callard P, et al: Hepatitis B virus-associated polyarteritis nodosa: clinical characteristics, outcome, and impact of treatment in 115 patients, Medicine (Baltimore)
84:313–322, 2005.
26. Outcome
Earlier diagnosis and initiation of treatment has improved outcomes.
Non-HBV PAN the 7-year survival rate is 79%, compared with a 5-year
survival rate of 72.5% in HBV-related PAN ( similar to ANCA –vasculitis)
The relapse rate in HBV-related PAN is low (<10%) compared with non–
HBV-related PAN (19.4% to 57% relapse rates).
Delayed diagnosis (>3 months) increases the risk of relapse but does
not affect mortality risk.
Gayraud M, Guillevin L, le Toumelin P, et al: Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and
Churg-Strauss syndrome: analysis of four prospective trials including 278 patients, Arthritis Rheum 44:666–675, 2001
Phillip R, Luqmani R: Mortality in systemic vasculitis: a systematic review, Clin Exp Rheum 26:S51, S94–104, 2008.
27. Long term outcome in patients with
FFS=0
PAN or MPA without Five-Factor Score (FFS =0) (2005-2012) and data were recorded prospectively –
mean follow up 98.2±41.9months.
Long-term survival, disease-free survival (DFS), relapses, therapeutic responses and sequelae were
analyzed.
After having initially received glucocorticoids (GC) alone, according to the study protocol, 82%
(97/118) patients achieved remission but 18% (21/118) required ≥1 immunosuppressant(s) (IS) before
19/21 achieved remission. Two patients died before entering remission. After remission, 53% (61/116)
patients relapsed 25.6±27.9months after starting treatment.
The 5- and 8-year overall survival rates were 93% and 86%, respectively, with no difference between
PAN and MPA, and between relapsers and nonrelapsers. DFS was shorter for MPA than PAN patients
(P=0.02). Throughout follow-up, 47% of patients required ≥1 IS. At the last follow-up visit, 44% were
still taking GC and 15% IS. The mean vasculitis damage index score was 1.9±1.9; the most frequent
sequelae were peripheral neuropathy, hypertension and osteoporosis.
PAN or MPA without poor-prognosis factors at diagnosis and treated initially with GC alone, long-term
survival was excellent. However, relapses remained frequent, requiring IS introduction for nearly half
of the patients.Autoimmun Rev. 2014 Feb;13(2):197-205. doi: 10.1016/j.autrev.2013.10.001. Epub 2013 Oct 23. Samson M et al. French
Vasculitis Study Group (FVSG) Long-term follow-up of a randomized trial on 118 patients with polyarteritis nodosa or microscopic
polyangiitis without poor-prognosis factors.