Title: Poster three by four foot poster: vertical format
1Polyarteritis Nodosa A case presentation
Poster -- three by four foot poster vertical
format
Author Number One, MD, PhD1 Author Number Two,
MD2 Author Number Three, MD2 Author Number
Four, MD2 Author Number Five, MD2
William Bender, MPH1 Sean Whelton, MD2
1Georgetown University School of Medicine,
Washington, DC 2Department of Medicine,
Georgetown University Hospital, Washington, DC
Georgetown University
Imaging
Introduction
Polyarteritis nodosa (PAN) is a systemic
vasculitis that affects medium sized arteries.
Its prevalence is estimated to be approximately
6.3 per 100,00 and it is most commonly diagnosed
in middle aged adults. The majority of cases are
idiopathic and while the pathogenic mechanisms
are also poorly understood, it is clear that PAN
produces a segmental transmural inflammation of
muscular arteries. This process tends to produce
a weakening of arterial walls, as well as
aneurysmal dilations and localized ruptures.
Patients typically present with systemic symptoms
which can involve kidneys, joints, skin, muscles,
nerves and gastrointestinal tract. As a result,
it is not uncommon for the disease to mimic other
conditions, especially those within the
vasculitis family. There have been several
attempts, most notably by the American College of
Rheumatology (ACR) in 1990 and the Chapel Hill
Consensus Conference of 1994, to create clear
delineations between PAN and other associated
conditions, such as microscopic polyangiitis.
The ACR currently has criteria in place for the
diagnosis of PAN, but it is not uncommon for
cases to present outside of the criteria. The
purpose of this case presentation, then, is to
discuss PAN and its potential for varied
presentations and to provide guidance with
regards to both treatment and diagnosis.
Fig 3. Arteriogram demonstrating mid-splenic
artery aneurysm
Fig 2. Post-mortem section of celiac artery
demonstrating intramural hemorrhage, fibrinoid
necrosis and lymphoplasmacytic infiltrate
Fig 1. CT scan demonstrating splenic artery
aneurysm
Notable Laboratory Studies
Case
- HPI
- The patient was a 45 year old male who initially
presented to Reston Hospital Center with an acute
onset of lower abdominal pain and subsequent CT
scan of his abdomen revealed the presence of free
fluid in the pelvis as well as an incidental
finding of a right common iliac artery aneurysm. - The patient subsequently underwent an emergent
Hartmann procedure for perforated diverticulitis
and his post-operative course was complicated by
a significant amount of bloody extrusion from his
JP drains as well as episodes of hypertension. - A follow-up CT scan revealed a right common
iliac artery aneurysm now with dissection, as
well as splenic and renal artery aneurysms - Pathology from the colon demonstrated normal
blood vessels with no evidence of vasculitis. - The patient subsequently received Solumedrol 1g
IV as treatment and was transferred to Georgetown
University Hospital for further diagnostic workup
and treatment. - Past Medical History
- Significant for Hartmann Procedure, Left knee
surgery, Abdominal hernia repair as a child - Physical exam upon admission
- VS 37.3 125/75 101 22 98 RA
- Gen - AOx3, NAD
- HEENT - No malar/discoid rash noted, No oral
ulcers noted, Slightly icteric sclera - Neck - No lymphadenopathy/bruits noted
Discussion
- According to the ACR criteria of 1990, patients
with systemic vasculitis can be classified as PAN
if they present with at least three of the
following 10 criteria
- The above criteria have a sensitivity and
specificity of 82 and 87 percent respectively,
and accordingly, this case would classify as PAN.
The patient had diastolic blood pressures
greater than 99 (especially upon initial
presentation) as well as angiographic
abnormalities, and biopsy demonstrating
polymorphonuclear neutrophils. - The severity of PAN is often delineated via a
Five Factors Score (FFS), which was initially
presented by Guillevin et al in 1998 and
demonstrated significant prognostic value with
regards to factors that contributed to higher
mortality. It was found that proteinuria1gm/day,
renal insufficiency (Cr140µmol/L),
cardiomyopathy, GI manifestations and CNS
involvement all contributed significantly to
higher mortality in PAN patients. When the FFS
was zero, mortality at 5 years was 12, when the
FFS was 1, mortality was 26, and when the FFS
was 2, mortality was 46. - Applying these criteria to this patient, the FFS
score was 2, given the 24hr urine protein 1 gram
and abdominal symptoms. - Treatment for PAN has historically been based on
the FFS score, although there is ongoing debate
regarding the aggressiveness of treatment at FFS
scores (FFS0), corticosteroids alone at a dose of
1mg/kg/day, with a maximum dose between 60mg and
80mg per day is the appropriate treatment. The
controversy arises with the treatment of moderate
PAN (FFS1). Some argue that steroids alone are
sufficient, while others purport that a regimen
of both corticosteroids and oral cyclophosphamide
(1.5/2mg/kg/day) is warranted. With a FFS of 2 or
greater, corticosteroid therapy in conjunction
with cyclophosphamide has been demonstrated to
significantly prolong survival.
- Hospital Course
- The patient was admitted to the SICU for
treatment of his continued hypertension and
work-up of his multiple aneurysms. - Arteriogram confirmed previous findings and
revealed web-like stenosis of the right renal
artery as well as additional aneurysms in the
middle colic and ileocolic arteries. - The patient was started on a 5d course of
Solu-Medrol 1g IV and upon completion, began a
course of Medrol 60mg IV Qdaily. The patients
hypertension was managed with a regimen including
Hydralazine, Clonidine, Labetalol and Lasix. - The patient had an IVC filter placed secondary
to a pulmonary embolism. - The patient began to improve clinically and was
transferred to the floor with plans of continuing
the Medrol treatment and obtaining a follow-up
angiogram in 1 month - Upon transfer, the patient began to develop
hypotension into the 60s systolic, as well as
seizure activity and increased sanguineous
drainage from his JP drains - The patient received IV fluids and a Levophed
drip and was transferred back to the SICU upon
arrival the patient was noted to be increasingly
somnolent with continued seizure-like activity
and a distended and protuberant abdomen. The
patient had cessation of respiration and was
noted to have no pulse CPR was initiated and
continued for 18 minutes and after a brief
recovery, the patient was noted to have lost
pulse again subsequent resuscitation was
unsuccessful and the patient expired - Post-mortem autopsy revealed significant
hemoperitoneum and the splenic artery was unable
to be identified secondary to copious coagulated
blood, autolysis and adhesions significant
atherosclerosis, a diffusely hemorrhagic colon
and abdominal peripancreatic adherent hematoma
were also noted - The cause of death was attributed to hemorrhagic
shock secondary to vasculature rupture with
clinical features suggestive of polyarteritis
nodosa and confirmed by histology and special
stains
- This patients presentation placed him in the
category of moderate to severe PAN due to his FFS
of 2, and he received the more conservative
treatment of IV corticosteroids without
cyclophosphamide. Given the ambiguity and
controversy regarding treatment of moderate
severity PAN (FFS1) as well as the effectiveness
seen with severe PAN (FFS 2) , the question thus
arises would this patient have benefited from
adjuvant treatment with cyclophosphamide?
Retrospectively, it is impossible to tell,
however, this highlights the importance of
developing more stringent criteria to not only
diagnose, but also treat PAN, especially in cases
that initially present in the mild to moderate
category.
References
- Treatment and prognosis of polyarteritis nodosa.
Up To Date. Accessed 4/25/2008 - Clinical manifestations and diagnosis of
polyarteritis nodosa. Up To Date. Accessed
4/25/2008. - Gayraud M, Guillevin L et al. Long-term follow up
of polyarteritis nodosa, microscopic
polyangiitis, and Churg-Strauss Syndrome
analysis of four prospective trials including 278
patients. Arthritis Rheum 200144668-77. - Guillevin L, Lhote F. Treatment of polyarteritis
nodosa and microscopic polyangiitis. Arthritis
Rheum 1998412100-5. - Segelmark M, Daina S. The challenge of managing
patients with polyarteritis nodosa. Curr Opin
Rheumatol 20071933-38.
Acknowledgments Leila Kia