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Title: Poster three by four foot poster: vertical format


1
Polyarteritis 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
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