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10/29/2013
Disclosures
Mortality, Recurrence, and Hospital Course of
Patients With Systemic Sclerosis-Related Acute
Intestinal Pseudo-Obstruction
• Chris Mecoli, Shivani Purohit, Nora Sandorfi: no disclosures
• Chris Derk: Research funding: Actelion, Gilead, Celgene
Christopher Mecoli a, Shivani Purohitb, Nora Sandorfia, and
Chris T. Derka
a Division
b
of Rheumatology, University of Pennsylvania
Division of Rheumatology, Thomas Jefferson University
Background: Pseudo-obstruction
• Decreased intestinal propulsion resulting in obstructive
symptoms in the absence of a mechanical cause
Study Objectives
• To describe the phenotype of a rare subgroup
(pseudo-obstruction) within a rare disease
(systemic sclerosis)
• Determine predictors of:
– In-Hospital Mortality
– Disease Recurrence
– Hospital Stay
Image A: Cross JM, Marvin RG. The Internet Journal of Emergency and Intensive Care Medicine 2000; 41.
Image B: Nature Clinical Practice Gastroenterology & Hepatology (2008) 5, 584-588
Methodology I
• Retrospective study
Flowchart of patient selection
1,733 total admissions in patients with SSc

103 matched search criteria

• 11.5 year period (2000-2012); inpatient medical records
of SSc admissions and intestinal obstruction using ICD-9
codes
64 admissions with acute pseudo-obstruction

• Two academic institutions in Philadelphia, Pennsylvania,
USA
37 unique patients

• Identified cases based on chart review of clinical history
and radiography
28 patients with single admit; 9 patients with >1
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Methodology II
Patient and Disease Characteristics
Demographics
N=37 patients (%)
Sex
• Data abstraction tool included
– Demographics, disease characteristics, presenting
symptoms and laboratory values, treatment
• Outcomes
– In-Hospital Mortality, Disease Recurrence, and
Hospital Stay
7 males (19)
Age
59.6 ±11.7
Race
14 African-American, 23 Caucasian
Disease Characteristics
N=64 admissions (%)
Esophageal involvement
72
Raynaud’s
63
Pulmonary arterial hypertension
41
Interstitial lung disease
27
Digital ulcers
23
Bacterial overgrowth
• Statistical evaluation between group comparisons using
Fisher’s Exact and student’s t test
Medications On Admission
Pro-motility agents
48
Opioids
16
Patient and Disease Characteristics
Demographics
N=37 patients (%)
16
N=64 admissions (%)
Patient and Disease Characteristics
Demographics
N=37 patients (%)
Sex
7 males (19)
Sex
Age
59.6 ±11.7
Age
7 males (19)
59.6 ±11.7
Race
14 African-American, 23 Caucasian
Race
14 African-American, 23 Caucasian
Disease Characteristics
N=64 admissions (%)
Disease Characteristics
N=64 admissions (%)
Esophageal involvement
72
Esophageal involvement
72
Raynaud’s
63
Raynaud’s
63
Pulmonary arterial hypertension
41
Pulmonary arterial hypertension
41
Interstitial lung disease
27
Interstitial lung disease
27
Digital ulcers
23
Digital ulcers
23
Bacterial overgrowth
16
Bacterial overgrowth
Medications On Admission
N=64 admissions (%)
Medications On Admission
16
N=64 admissions (%)
Pro-motility agents
48
Pro-motility agents
48
Opioids
16
Opioids
16
Presenting Symptoms
Symptoms
Nausea
N=64 admissions (%)
49 (77)
Treatment Modalities
Treatment modality
N=64 admissions (%)
NPO
46 (74)
Intravenous fluids
41 (66)
Abdominal pain
32 (50)
Weight loss
11 (17)
Diarrhea
9 (14)
Total parenteral nutrition
26 (42)
Emesis
4 (6)
Antibiotics
20 (32)
Melena
2 (3)
Hematochezia
2 (3)
Pro-motility agent
31 (50)
Nasogastric tube**
23/29 (37/46)
Surgery
7 (11)
**23 patients with nasogastric tube, 6 additional
patients with gastro-jejunosotomy
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Mortality
Clinical Outcomes
Demographics
Outcome
N=64 admissions (%)
Spontaneous resolution
45 (73)
Permanent TPN
16 (26)
Surgery
7 (11)
Death
6 (10)
12 ±12.5
Days hospitalized
Sex
Age
Disease Characteristics
Raynaud’s
Digital ulcers
Interstitial lung disease
Pulmonary arterial hypertension
Esophageal involvement
Bacterial overgrowth
Medications On Admission
Opioids
Pro-motility
Death (%)
(N=6)
3 male (50)
62 ± 12
(N=6)
3
2
2
2
0
0
Survive (%)
(N=31)
4 male (12)
58.5 ± 13
(N=58 admissions)
37 (65)
13 (23)
15 (26)
24 (42)
42 (74)
9 (16)
2
4
9 (16)
29 (51)
= p-value < 0.05
Mortality
Laboratory values
Hemoglobin
White blood cell count
AST
ALT
Alkaline phosphatase
Creatinine
Bicarbonate
Blood urea nitrogen
Albumin
Amylase
Death N=6
9.9 ± 0.6
11.2 ± 4.4
42 ± 48
18 ± 6
68 ± 33
1.8 ± 1.8
26 ± 4.3
18 ± 9.6
2.5 ± 0.5
71 ± 21
Mortality
Survive N=58
12 ± 2.1
10.7 ± 5.8
36 ± 26
33 ± 35
124 ± 75
1 ± 0.5
28.5 ± 10.5
24 ± 16.9
3.7 ± 0.6
110 ± 66
Treatment modality
Death N=6 (%) Survive N=58 (%)
NPO
4
42 (76)
Surgery
2
3 (5)
Nasogastric tube
6 (100)
20 (36)
Pro-motility
4
27 (49)
Antibiotics
1
19 (35)
Intravenous fluids
3
38 (69)
Total parenteral nutrition
1
21 (38)
Outcome
Surgery
3 (50)
2(4)
Permanent TPN
0
16 (29)
Death
6
0
Days hospitalized
27 ± 21
10.1 ± 9.9
= p-value < 0.05
= p-value < 0.05
Recurrence
Demographics
Hospital Stay
single episode (%)
N=28
recurrent episode (%)
N=9
Sex
Age
Disease Characteristics
Raynaud’s
Digital ulcers
7 male (25)
61.1 ± 13.9
(N=28)
20 (74)
8 (30)
0 male (0)
58.6 ± 9.9
(N=36 admissions)
20 (54)
7 (19)
Interstitial lung disease
7 (26)
10 (27)
Pulmonary arterial hypertension
Esophageal involvement
Bacterial overgrowth
Medications On Admission
Opioids
Pro-motility
8 (30)
25 (93)
3 (11)
18 (49)
21 (57)
7 (19)
8 (30)
9 (33)
3 (8)
22 (59)
= p-value < 0.05
Treatment modality
NPO
Surgery
Nasogastric tube
Pro-motility agents
Antibiotics
Intravenous fluids
Total parenteral nutrition
Outcome
Spontaneous resolution
Permanent TPN
Days hospitalized
Hosp > 7 days (%)
20 (74)
6 (22)
17 (63)
17 (63)
14 (52)
18 (67)
11 (41)
Hosp < 7 (%)
24 (80)
0 (0)
8 (27)
13 (43)
13 (43)
22 (73)
11 (37)
14 (52)
8 (30)
20.7
29 (97)
8 (27)
3.9
= p-value < 0.05
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Clinical Outcomes
• In-Hospital Mortality associated with:
– male sex, low albumin, hemoglobin, ALT, alk phos, amylase
• Disease Recurrence associated with:
– female sex (note half of males died)
– less esophageal involvement
– unclear effect of opioids and pro-motility agents
Study Limitations
– Retrospective
– Diagnostic uncertainty
– Geographic restriction
• Hospital Stay associated with:
– surgery, decompression
Intestinal Pseudo-obstruction:
A rare but morbid condition in SSc
• Accounts for 4% of hospitalized SSc patients
• Majority resolve spontaneously, although 10%
mortality
Acknowledgements
•Dr. Chris T. Derk
•Dr. Nora A. Sandorfi
•Dr. Joan Von Feldt
•Dr. Peter Merkel
• Conservative management favored
• Recurrence in 25%
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