Antibodies associated with severe GI Scleroderma

1
Antibodies associated with severe GI Scleroderma
Chinelo I Azih, MD Justin Peng, MD Virginia
Steen, MD
Department of Rheumatology, Georgetown University
Hospital, Washington, DC Georgetown University
Medical Center, Washington, DC
Georgetown University
Results (continued)
Purpose
Gastrointestinal (GI) dysfunction is common in
Systemic Sclerosis (SSc) with a 90 prevalence
rate.¹ Severe gastrointestinal manifestations
often involve the small bowel, are associated
with high morbidity and mortality and can lead to
life threatening complications.¹,² We evaluated a
series of patients with Systemic Sclerosis and
severe small bowel dysfunction to further
characterize this association and to identify
factors and antibodies that may place patients
at risk for severe small bowel involvement .
Table 1. Comparison of variables in patients
with mild GI symptoms (Group 1) and severe GI
symptoms (Group 2)
Introduction
There were more patients with nucleolar antibody
in the severe group (Group 2) than in the mild
group (Group1)? 36.3 vs 20. There were more
black patients in the severe group compared to
the mild group? 59 vs 20 and more non-black pts
in the mild group (80) compared to the severe
group (41).
Systemic sclerosis is a chronic autoimmune
disease characterized by proliferative vascular
lesions with resultant fibrosis of skin and
multiple organs including the gastrointestinal
(GI) tract.¹ GI dysmotility is prevalent in 90
of SSc patients and can increase morbidity and
mortality.² Up to 60 of patients with GI
dysfunction are asymptomatic leading to
under-diagnosis or delayed diagnosis.³ GI
involvement can affect any area from the
esophagus to the anus.¹ The most commonly
affected site is the esophagus which is involved
in about 80 of patients. Small intestine
dysfunction occurs in about 40 of patients and
large intestine dysfunction occurs in about
10-50 of patients.² Severe GI involvement in
Scleroderma often involves the small bowel and is
associated with increased morbidity and
mortality.

• 90 of pts with nucleolar antibody were black
• 90 of pts with nucleolar antibody had diffuse
  disease.
• The average duration of onset of GI disease from
  first scleroderma symptom was shorter in the
  nucleolar group (2.4 years) than the
  non-nucleolar group (8.2 years).
• The average age of GI onset was also slightly
  younger in the nucleolar group compared to the
  non-nucleolar group.
• 80 of patients with nucleolar antibody had
  severe pseudo-obstruction where as only 50 in
  the non-nucleolar group had severe
  pseudo-obstruction.
• 60 of patients with nucleolar antibody had
  refractory disease where as only 5 of patients
  in the non-nucleolar group had refractory
  disease.
• 90 of patients in the nucleolar group required
  hospital admissions for their GI manifestations
  compared to 73 in the non-nucleolar group.
• GI disease was the cause of death in 50 of the
  patients in the nucleolar group compared to 0
  of the patients in non-nucleolar group.

Patterns of small bowel involvement in Systemic
Sclerosis
Table 2. Comparison of variables in nucleolar and
non-nucleolar antibody groups
Conclusions
Nucleolar antibody appears to be associated with
earlier onset of GI symptoms, increase in
severity and refractory nature of GI symptoms,
increased frequency of hospitalizations for GI
symptoms and more fatal GI symptoms in SSc
patients. Black patients tended to have more
nucleolar antibody and more severe symptoms
compared to non- black SSc patients. More
definitive conclusions can be drawn after
statistical analysis.
Methods
Thirty two patients with small bowel
manifestations of SSc were identified from
Georgetown University Hospital. A retrospective
chart review was conducted to obtain the
following data demographics, types of GI
symptoms, antibody status, timing of diagnosis,
other SSc organ involvement, treatment,
co-morbities, and survival outcome.
Results
25 patients were female and 7 were male. 14
patients were white and 15 were black. 18
patients were characterized as diffuse and 14
were limited. 10 patients had a nucleolar
antibody pattern. 20 patients had
pseudo-obstruction, 10 had mild
pseudo-obstruction and 1 had malabsorption.
Patterns of small bowel involvement identified
included bloating, pseudobstruction, and
malabsorption. Patients were divided into two
groups according to GI disease severity. Group 1
with mild symptoms i.e. mild pseudo-obstruction
and Group 2 with severe symptoms i.e. Severe
pseudo-obstruction and malabsorption. We
identified a likely association with nucleolar
antibody so we also divided the patients into two
groups based on nucleolar antibody status.
(Nucleolar and non-nucleolar antibody groups)
References

• Sallam H, Mcnearney TA, Chen JDZ. Systemic
  review pathophysiology and management of
  gastrointestinal dysmotility in systemic
  sclerosis. Aliment Pharmacol Ther 2006 23
  691-712
• 2. Nishimagi, E et al. Characteristics of
  Patients with Early Systemic Sclerosis and Severe
  Gastrointestinal Tract Involvement. Journal of
  Rheumatology 2007 34(10) 2050-5
• 3. Sjogren RW. Gastrointestinal features of
  scleroderma. Curr Opin Rheumatol 1996 8 569-75
• 4. UptoDate

Acknowledgments
Georgetown University Hospital Department of
Rheumatology