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20 Mesenteric Panniculitis 189
4. Kerdsirichairat T, Mesa H, Abraham J, et al. Sclerosing mesenteritis and IgG4-related
mesenteritis: case series and a systematic review of natural history and response to treatments.
Immunogastroenterology. 2013;2:119–28.
5. Roginsky G, Alexoff A, Ehrenpreis ED. Initial findings of an open-label trial of low-dose
naltrexone for symptomatic mesenteric panniculitis. J Clin Gastroenterol. 2015;49(9):794–5.
6. Smith ZL, Sifuentes H, Deepak P, Ecanow DB, Ehrenpreis ED. Relationship between
mesenteric abnormalities on computed tomography and malignancy: clinical findings and
outcomes of 359 patients. J Clin Gastroenterol. 2013;47(5):409–14.
7. Canyigit M, Koksal A, Akgoz A, Kara T, Sarisahin M, Akhan O. Multidetector-row
computed tomography findings of sclerosing mesenteritis with associated diseases and its
prevalence. Jpn J Radiol. 2011;29(7):495–502.
8. Vlachos K, Archontovasilis F, Falidas E, et al. Sclerosing mesenteritis: diverse clinical
presentations and dissimilar treatment options. A case series and review of the literature. Int
Arch Med. 2010;4:17. https://www.ncbi.nlm.nih.gov/pubmed/21635777
9. Viswanathan V, Murray KJ. Idiopathic sclerosing mesenteritis in paediatrics: report of a
successfully treated case and a review of literature. Pediatr Rheumatol Online J. 2010;8:5.
https://www.ncbi.nlm.nih.gov/pubmed/20205836
10. Kasporitakis AN, Rizos CD, Delikoukos, etal. Retractile mesenteritis presenting with
malabsorption syndrome. Successful treatment with oral pentoxifylline. J Gastrointestin Live
Dis. 2008;17:91–94. https://www.ncbi.nlm.nih.gov/pubmed/18392253
11. Akram S, Pardi DS, Schaffner JA, Smyrk TC. Sclerosing mesenteritis: clinical features,
treatment, and outcome in ninety-two patients. Clin Gastroenterol Hepatol. 2007;5:589–596.
https://download.journals.elsevierhealth.com/pdfs/journals/1542-3565/
PIIS1542356507002248.pdf
12. Ginsburg PM, Ehrenpreis ED. A pilot study of thalidomide for patients with symptomatic
mesenteric panniculitis. Aliment Pharmacol Ther. 2002;16:2115–22.
13. Ferrari TC, Couto CM, Vilaça TS, Xavier MA, Faria LC. An unusual presentation of
mesenteric panniculitis. Clinics (Sao Paulo). 2008;63(6):843–4.
14. National Organization for Rare Disorders. Mesenteric Panniculitis. https://rarediseases.org/
rare-diseases/mesenteric-panniculitis/. Accessed 26 Feb 2019.
PPP Syndrome: Pancreatitis, Panniculitis, Polyarthritis
Eli D. Ehrenpreis
Definition
PPP Syndrome is a rare occurrence of extra-pancreatic complications of acute
pancreatitis., Panniculitis usually involves the fatty layers of the skin of the lower
extremities, but can be involve fatty tissue in any location in the body, including the
mesentery. Symmetric polyarthritis generally of the lower extremities is a characteristic occurrence.
Epidemiology
Pancreatic panniculitis is purported to affect about 2–3% of patients with pancreatic
disease. About 40 cases of PPP Syndrome have been reported in the medical
literature. Only a few cases with mesenteric involvement have been described in the
medical literature.
21
Patients at Risk
This syndrome is generally described in male patients with a history of alcohol
abuse between the ages of 40 and 70 years old. It occurs in the setting of acute or
chronic pancreatitis. It has also been described in association with cancer of the
pancreas.
E. D. Ehrenpreis (&)
Department of Medicine, Advocate Lutheran General Hospital, 1775 Dempster St, 6 South,
Park Ridge, IL 60068, USA
e-mail: e2bioconsultants@gmail.com
© Springer Nature Switzerland AG 2021
E. D. Ehrenpreis et al. (eds.), The Mesenteric Organ in Health and Disease,
https://doi.org/10.1007/978-3-030-71963-0_21
191
192 E. D. Ehrenpreis
Pathophysiology
Although the exact cause of this condition is unknown, it appears to be due to entry
of pancreatic enzymes into the systemic circulation. This in turn leads to an
inflammatory destruction of fat-containing organs including subcutaneous tissues,
bone marrow and the mesentery. Destruction of fatty tissue, primarily by pancreatic
lipase results in the development of fat saponification and necrosis. It is presumed
that joint inflammation occurs due to circulating inflammatory cytokines, although
some have proposed that deposition of antigen–antibody complexes contribute to
inflammatory lesions of the disease. Arthritis may also develop as a result of joint
infiltration by free fatty acids. Fistulation between a pancreatic pseudocyst and a
portion of the mesenteric venous system may be an important factor in the
development of the condition.
Signs and Symptoms
The clinical presentation of this condition is variable and dependent on the severity
of pancreatic disease, and the extent of involvement of other affected areas. Typically, patients will have abdominal pain related to acute and chronic pancreatitis.
The severity of abdominal symptoms may worsen with the subsequent development
of mesenteric fat necrosis and the development of an inflammatory mass of the
mesentery. Tender, deep erythematous nodular lesions of the skin are present.
These may open and extrude an oily brown or creamy material. An inflammatory
polyarthritis and interosseous fat necrosis are additional characteristics of this
condition, resulting in joint and bone pain. The consequ ences of severe pancreatic
disease is associated with a 25% mortality rate. Untreated disease can result in bone
marrow necrosis, scarring of the skin, septic arthritis, chronic debilitating arthritis,
and worsening chronic pancreatitis.
Diagnosis
The diagnosis of PPP syndrome is made on the basis of the triad of pancreatitis,
panniculitis and polyarthritis occurring simultaneously in an affected patient.
Typical physical findings, elevated serum lipase level and characteristic radiographic and histologic findings confirm the diagnosis .
21 PPP Syndrome: Pancreatitis, Panniculitis, Polyarthritis 193
Physical Findings
The presentation of this condition can be variable in severity. In general, patients
will have painful nodular lesions of the skin joint pain with effusions and physical
findings consistent with acute or chronic pancreatitis. A symmetrical inflammatory
polyarthritis may occur. The abdominal examination may reveal a tender palpable
mesenteric mass.
Laboratory Testing
Characteristic laboratory findings associated with acute and chronic pancreatitis
occur in this condition. However, cases described in the literature demonstrate
markedly elevated levels of serum lipase and other pancreatic enzymes.
Imaging
CT scan of the abdomen may reveal acute pancreatitis, pancreatic pseudocysts,
chronic pancreatitis and/or pancreatic cancer. When mesenteric involvement occurs,
the presence of a mass in the mesenteric fat will be detected. Joint disruption and
effusions as well as lytic lesions of the bone may be detect ed with radiography.
Bone marrow abnormalities including necrosis are detected with MRI.
Histologic Findings
Fat necrosis is seen in skin lesions and in biopsies of the mesenteric mass when
present. Loss of nucleation and partial destruction of the cell membranes of adipocytes produces the characteristic “ghost cells” seen on biopsy. Analysis of fluid
removed from affected joints reveals the presence of inflammation and or sepsis.
Treatments
Medical
Treatment of pancreatic disease appears to result in improvement of the
extra-pancreatic complications associated with this condition. Chronic arthritis and
chronic pancreatitis may ensue. Nonsteroidal anti-inflammatory drugs and corticosteroids have been used to reduce associated inflammation and may be effective
194 E. D. Ehrenpreis
in controlling skin and joint manifestations of this condition. Use of octreotide and
plasmapheresis have been described in the literature.
Surgical
One case report described the stenting of a disrupted pancreatic duct and drainage
of a necrotic pancreatic cyst using endoscopic ultrasound and endoscopic retrograde
cholangiopancreatography. Partial pancreatoduodenectomy and resection of a
mesenteric fistula has also been described. Surgical drainage of pancreatic
pseudo-cysts may be beneficial. Total pancreatectomy has been described as
curative treatment.
Additional Comments
PPP syndrome is one of several complications of acute pancreatitis that may affect
the mesentery. Acute pancreatitis may also be associated with thrombosis in the
mesenteric veins or arteries.
Suggested Reading
1. Dong E, Attam R, Wu BU. Board review vignette: PPP syndrome: pancreatitis, panniculitis,
polyarthritis. Am J Gastroenterol. 2017;112:1215–6.
2. Narvaez J, Bianchi MM, Santo P, et al. Pancreatitis, panniculitis, and polyarthritis. Semin
Arthritis Rheum. 2010;39:417–23.
3. Dieker W, Derer J, Henzler T, et al. Pancreatitis, panniculitis and polyarthritis (PPP-) syndrome
caused by post-pancreatitis pseudocyst with mesenteric fistula: diagnosis and successful
surgical treatment: Case report and review of literature. Int J Surg Case Rep. 2017;31:170–175.
4. Laureano A, Mestre T, Ricardo L, et al. Pancreatic panniculitis-a cutaneous manifestation of
acute pancreatitis. J Dermatol Case Rep. 2014;8:35–7.
5. Ferri V, Ielpo B, Duran H, et al. Pancreatic disease, panniculitis, polyarthrtitis syndrome
successfully treated with total pancreatectomy: Case report and literature review. Int J Surg
Case Rep. 2016;28:223–6.
6. Kang DJ, Lee SJ, Choo HJ, et al. Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome:
MRI features of intraosseous fat necrosis involving the feet and knees. Skeletal Radiol.
2017;46:279.
Mesenteric Adenitis
Eli D. Ehrenpreis
Definition
Mesenteric adenitis (or mesenteric lymphadenitis) is a benign inflammatory condition involving the lymphatic system located in the right lower quadrant of the
abdomen. It is an important entity in the differential diagnosis of patients presenting
with right lower quadrant pain. Mesenteric adenitis is defined by the presence of a
group of three or more lymph nodes in the right lower quadrant mesentery that are
5 mm or greater when other conditions are ruled out. Mild terminal ileum
inflammation may be present. Primary mesenteric adenitis is acute in onset and is
likely an infectious condition of origin. Secondary mesenteric adenitis occurs as a
complication of a variety of infectious and inflammatory disorders and can occur in
patients infected with human immunodeficiency virus (HIV).
22
Epidemiology
The complaint of abdominal pain makes up about seven percent of all emergency
room visits in the United States. Next to acute appendicitis, mesenteric adenitis is
the second most common cause of right lower quadrant of the abdomen pain. In the
past, mesenteric adenitis was diagnosed in patients undergoing surgery for suspected appendicitis. With the addition of modern imaging techniques, mesenteric
adenitis is diagnosed in 2–16% of patients initially suspected of having acute
appendicitis.
E. D. Ehrenpreis (&)
Department of Medicine, Advocate Lutheran General Hospital, 1775 Dempster St, 6 South,
Park Ridge, IL 60068, USA
e-mail: e2bioconsultants@gmail.com
© Springer Nature Switzerland AG 2021
E. D. Ehrenpreis et al. (eds.), The Mesenteric Organ in Health and Disease,
https://doi.org/10.1007/978-3-030-71963-0_22
195
196 E. D. Ehrenpreis
Patients At Risk
Primary mesenteric adenitis is most commonly seen in adolescent and young adult
patients with an average age of 25 years (range 5–44) years. Secondary mesenteric
adenitis is seen in patients belonging to high risk groups. These include patients
with celiac disease, diverticulitis, cholecystitis, Crohn’s disease, ulcerative colitis,
mesenteric panniculitis, pancreatitis, systemic lupus erythematosus and other connective tissue. Patients infected with HIV may develop secondary mesenteric
adenitis from mycobacterium avium complex infection. In addition, HIV infected
patients are at risk for the immune reconstitution inflammatory syndrome (IRIS).
This occurs 3–12 months after initiation of treatment with highly active antiretroviral therapy (HAART). Due to an overall improvement of the immune system in
HIV-infected patients treated with HAART therapy, an increase in circulating
CD4 T cells results in the development of an inflammatory response to ongoing
infections with viruses (such as cytomegalovirus or herpes simplex), mycobacteria,
or fungal infections (such as Cryptococcus neoformans or Pneumocystis jerovecii).
IRIS may manifest as abdominal pain and abdominal lymphadenopathy.
Pathophysiology
It is hypothesized that mesenteric adenitis occurs when infectious organisms gain
entry to intestinal lymph nodes after invasion of intestinal lymphatics. A subsequent
inflammatory process is triggered within the lymph nodes, and the intensity of this
response varies according to the virulence of the invasive organism. Suppurative
lymph node inflammation and/or associated systemic involvement may occur.
Cultures of mesenteric lymph nodes and blood have demonstrated a number of
infectious causes of the condition (Table 22.1).
Symptoms
The classic presentation of primary mesenteric adenitis is the acute onset of fever
and right lower quadrant abdominal pain. Although the majority of patients in the
appropriate age group with these symptoms will have acute appendicitis, the
additional presence of diarrhea is more consistent with the diagnosis of mesenteric
adenitis. Signs and symptoms of an upper respiratory tract infection, other systemic
infectious illnesses and peripheral lymph node enlargement may also occur.
22 Mesenteric Adenitis 197
Table 22.1 Organisms
associated with mesenteric
adenitis
Bacteria
Yersinia species (most common causative organisms)
Beta-hemolytic streptococcus
Staphylococcus species
Escherichia coli
Streptococcus viridans
Mycobacterium tuberculosis
Non-Salmonella typhoid
Bartonella henselae (Cat Scratch Disease)
Parasites
Giardia lamblia
Viruses
Coxsackieviruses (A and B)
Rubeola virus
Adenovirus (serotypes 1, 2, 3, 5, and 7)
Epstein-Barr virus (EBV)
Acute human immunodeficiency virus (HIV)
Alan S. Putrus and Michael H. Piper. “Mesenteric Lymphadenitis”. https://
emedicine.medscape.com/article/181162.
Physical Findings
Fever and abdominal tenderness (particularly in the right lower quadrant) are
physical signs that occur in patients with either mesenteric adenitis or acute
appendicitis. Rebound tenderness and may be present in both mesenteric adenitis
and acute appendicitis. Rectal tenderness on the right or middle portion of the
digital rectal exam may occur, but does not help to differentiate between the two
conditions. Peripheral lymphadenopathy may be present in mesenteric adenitis. The
presence of other physical findings may identify causes of secondary mesenteric
adenitis.
Laboratory Testing
Leukocytosis is presen t in both mesenteric adenitis and acute appendicitis and
cannot be reliably used to differentiate the two conditions. A white blood cell count
(WBCs) of greater than 10,000/µL in present in more than of 50% of with
mesenteric adenitis.
198 E. D. Ehrenpreis
Imaging
The initial purpose of performing imaging studies in the appropriate clinical setting
is to confirm or rule out the diagnosis of acute appendicitis and other causes of
acute abdominal pain. Abdominal ultrasound (US) and computerized scan of the
abdomen (CT) are complementary tests for identification of acute appendicitis. The
range of sensitivities of abdominal US for diagnosing acute appendicitis is between
71 and 94% and a specificity ranges 81 and 98%. Ultrasound criteria for the
diagnosis of appendicitis include an appendiceal diameter of > 6 mm, thickening of
the appendiceal wall and non-compressibility of the appendix. Appendiceal
inflammation can also be confirmed using Doppler imaging. Due to high sensitivity
and specificity, US is the initial imaging procedure of choice to rule out acute
appendicitis.
Abdominal CT may identify the presence of multiple lymph nodes in the right
lower quadrant that are characteristic of mesenteric adenitis (Figs. 22.1, 22.2, and
22.3). CT is more effective in diagnosing acute appendicitis and has a range of
sensitivity between 76–100% and a specificity range from 83–100%. CT criteria for
appendicitis include appendiceal enlargement (the range for normal diameter is
generally agreed to be between 6–9 mm), thickening of the appendiceal wall and
inflammation of the mesenteric fat that is adjacent to the appendix. It should be
noted that right sided abdominal lymph node enlargement may be present in
patients with acute appendicitis. CT imaging criteria for mesenteric adenitis
includes the presence of three or more lymph nodes with a maximal diameter of
5 mm or more in the right lower quadrant of the abdomen. Mild thickening of the
wall of the terminal ileum ( 5 mm) may also be present.
CT is also beneficial to identify etiologies for the occurrence of secondary
mesenteric adenitis. Imaging with abdominal CT is highly effective in identifying
other causes of acute abdominal pain in patients presenting with symptoms that are
initially suggestive of acute appendicitis such as cholecystitis, diverticulitis, ovarian
torsion and a perforated viscous. CT can also be used to identify other causes of
right lower quadrant pain that are in the differential diagnosis for mesenteric
adenitis including epiploic appendagitis, inflammatory bowel disease, Meckel’s
diverticulum, neutropenic colitis, right-sided diverticulitis, and omental infarction.
Treatment
Medical
Stabilization of the patient with adequate hydrat ion is a mainstay of therapy for
mesenteric adenitis. Pain control may be required on a short-term basis. Use of
empiric antibiotics, with coverage for Yersinia enterocolitica is considered.
Ongoing observatio n with serial abdominal examinations by the medical and surgical team may be needed if acute appendicitis or other causes of abdominal pain
22 Mesenteric Adenitis 199
Fig. 22.1 Coronal CT image of a patient with mesenteric adenitis demonstrating enlarged
mesenteric lymph nodes. These are most prominent in the ileocolic distribution Associated
ileocecal thickening is also visualized (circle)
Fig. 22.2 Axial image from the same patient showing enlarged lymph nodes (arrows) anterior to
the right psoas muscle with thickening of the cecal wall