**4.2 Joint involvement**

Arthralgia or arthritis is present in 2/3 of cases of patients; joint complaints are uncommon as the first symptom, but it is the appearing complaint in approximately 25% of children [21]. The articular manifestations are seen more often in adults than in children; typically involve the hips, knees, and ankles (less commonly the upper extremities); and are symmetrical (as skin lesions) in distribution [4]. The joint involvement is usually transient or migratory, typically oligoarticular and nondeforming. A prominent periarticular swelling and tenderness are usual, without joint effusion, erythema, or warmth. The most frequent symptom is arthralgia, while arthritis is very rare; joint effusions are exceptional [20].

### **4.3 Renal involvement**

Renal involvement is found in 40–85% of patients (more prevalent in older children and adults), ranging from microscopic hematuria to progressive glomerulonephritis, and is the most serious complication of Ig A vasculitis [17, 20]. The most common presentation is hematuria (with/without red blood casts) and mild proteinuria; proteinuria without hematuria is very rare. Renal manifestations usually develop within 4 weeks and never precede the onset of skin lesions. The risk of renal failure is rare in children [22] but may be present in about 30% of adult cases, especially in patients with nephrotic and nephritic syndromes [4]. Nephrotic-range proteinuria, elevated serum creatinine, hypertension, and the coexistence of hematuria and proteinuria are associated with an increased risk of renal failure; 2–5% of patients with renal involvement come to an end-stage renal failure [12, 17].

#### **4.4 Gastrointestinal manifestations**

Gastrointestinal involvement occurs in 50–75% of adult patients—abdominal pain and bloody diarrhea may precede the typical purpuric rash [15, 23]. The digestive symptoms are often present in patients with IgA vasculitis, and although less commonly found in adults, they are more severe and often atypical. The gastrointestinal symptoms vary in intensity, and prudent laparotomy is required to exclude

**39**

*Gastrointestinal Manifestations of IgA Vasculitis-Henoch-Schönlein Purpura*

toms may appear with no cutaneous purpura at any time [25, 26].

an acute abdominal condition. Gastrointestinal manifestations range from mild (nausea, vomiting, abdominal pain, paralytic ileus) to severe findings (gastrointestinal bleeding, intussusception, bowel ischemia with secondary necrosis, bowel

Gastrointestinal symptoms are often the most debilitating manifestations of the disease, and they are much more common in younger patients than in elders

The gastrointestinal manifestations may precede the onset of IgA vasculitis or may develop later in the course of the disease. Typically, gastrointestinal symptoms develop within 8 days from the rash appearance, although longer periods (weeks, even months) have been described. In 10–15% of cases, the gastrointestinal symptoms occur before cutaneous manifestation, making the differential diagnosis of other causes of acute abdomen more difficult [24]. Rarely, gastrointestinal symp-

*Abdominal pain* is the most common gastrointestinal symptom, manifested by colicky pain worsened by food (similar to bowel angina), localized to the epigastric and periumbilical regions. The pain is attributed to the involvement of the splanchnic circulation (mesenteric vasculitis) with bowel ischemia and edema of the gastrointestinal tract [4, 23, 27]. Abdominal pain associated with IgAV is caused by

Abdominal pain is often associated with diarrhea with gross or occult blood, nausea, vomiting, constipation, and gastrointestinal bleeding (hematemesis or

In most cases, on clinical examination, the abdomen is tender and slightly distended; rarely, it may resemble and be confused with an acute abdomen requiring surgery evaluation (suspicion of perforation, intussusception). Some of these cases, suggesting an acute abdomen, are leading to emergency computed tomography (CT) or unnecessary surgery [4, 23]. Sometimes, particularly in young patients, intense abdominal pain may suggest acute appendicitis, especially when the skin rash is absent. Even in the presence of rash (suggestive in a clinical context for Ig A vasculitis) in front of intense abdominal pain, the differential diagnosis of abdominal pain and the exclusion of acute surgical abdomen are mandatory; more, the IgA vasculitis rash may be a nonspecific erythematous or urticarial exanthem or limited

*Gastrointestinal bleeding* is another symptom, with an incidence varying between

Other rare gastrointestinal manifestations, such as *esophageal ulcer*, *pancreatitis*, *pseudomembranous colitis*, *gall bladder involvement*, *intestinal perforation*, and

Intussusception is the most common gastrointestinal complication of IgA vasculitis. Edema and hemorrhage can act as a pathological lead point, contributing to the development of intussusception. Intussusception is limited to the small bowel in approximately 60% of cases, in contrast to idiopathic intussusception, which is typically ileocolic. The overall incidence of this complication of IgA vasculitis varies between 2.3 and 3.5%, although some retrospective series reported an incidence of only 0.4 to 0.6% [9, 17]. Children with severe gastrointestinal pain and/or requiring

to lesions on the buttocks or lower extremities early in the disease course.

17.6 and 51% [23]. In most cases, the bleeding is occult (detected as positive stool occult blood); when manifested, the main symptom is melena rather than hematemesis or hematochezia, because the intestinal lesions are usually localized in the small bowel and colon. Gastrointestinal bleeding is usually mild; in rare cases it may be severe, requiring blood transfusion or surgical treatment, even leading to

*DOI: http://dx.doi.org/10.5772/intechopen.86966*

submucosal hemorrhage and edema.

perforation).

[23, 24].

melena).

death [4, 23].

*ischemic vasculitis* may occur [23].

hospitalization are at greater risk.

*Digestive System - Recent Advances*

**4.2 Joint involvement**

*Palpable purpura on both ankles.*

**Figure 1.**

**4.3 Renal involvement**

**4.4 Gastrointestinal manifestations**

The typical rash is symmetrically distributed and located primarily in gravity-/ pressure-dependent areas, such as the feet, ankles, and lower legs in adults; in the case of children, the buttocks, face, trunk, and upper extremities are more affected [19]. In child patients purpura gradually disappears (it can recur and become chronic), but in adults, it may be necrotic or hemorrhagic in 1/3 of cases, and

Arthralgia or arthritis is present in 2/3 of cases of patients; joint complaints are uncommon as the first symptom, but it is the appearing complaint in approximately 25% of children [21]. The articular manifestations are seen more often in adults than in children; typically involve the hips, knees, and ankles (less commonly the upper extremities); and are symmetrical (as skin lesions) in distribution [4]. The joint involvement is usually transient or migratory, typically oligoarticular and nondeforming. A prominent periarticular swelling and tenderness are usual, without joint effusion, erythema, or warmth. The most frequent symptom is arthralgia,

Renal involvement is found in 40–85% of patients (more prevalent in older children and adults), ranging from microscopic hematuria to progressive glomerulonephritis, and is the most serious complication of Ig A vasculitis [17, 20]. The most common presentation is hematuria (with/without red blood casts) and mild proteinuria; proteinuria without hematuria is very rare. Renal manifestations usually develop within 4 weeks and never precede the onset of skin lesions. The risk of renal failure is rare in children [22] but may be present in about 30% of adult cases, especially in patients with nephrotic and nephritic syndromes [4]. Nephrotic-range proteinuria, elevated serum creatinine, hypertension, and the coexistence of hematuria and proteinuria are associated with an increased risk of renal failure; 2–5% of

patients with renal involvement come to an end-stage renal failure [12, 17].

Gastrointestinal involvement occurs in 50–75% of adult patients—abdominal pain and bloody diarrhea may precede the typical purpuric rash [15, 23]. The digestive symptoms are often present in patients with IgA vasculitis, and although less commonly found in adults, they are more severe and often atypical. The gastrointestinal symptoms vary in intensity, and prudent laparotomy is required to exclude

cutaneous exacerbations may be seen for 6 months or longer [20, 21].

while arthritis is very rare; joint effusions are exceptional [20].

**38**

an acute abdominal condition. Gastrointestinal manifestations range from mild (nausea, vomiting, abdominal pain, paralytic ileus) to severe findings (gastrointestinal bleeding, intussusception, bowel ischemia with secondary necrosis, bowel perforation).

Gastrointestinal symptoms are often the most debilitating manifestations of the disease, and they are much more common in younger patients than in elders [23, 24].

The gastrointestinal manifestations may precede the onset of IgA vasculitis or may develop later in the course of the disease. Typically, gastrointestinal symptoms develop within 8 days from the rash appearance, although longer periods (weeks, even months) have been described. In 10–15% of cases, the gastrointestinal symptoms occur before cutaneous manifestation, making the differential diagnosis of other causes of acute abdomen more difficult [24]. Rarely, gastrointestinal symptoms may appear with no cutaneous purpura at any time [25, 26].

*Abdominal pain* is the most common gastrointestinal symptom, manifested by colicky pain worsened by food (similar to bowel angina), localized to the epigastric and periumbilical regions. The pain is attributed to the involvement of the splanchnic circulation (mesenteric vasculitis) with bowel ischemia and edema of the gastrointestinal tract [4, 23, 27]. Abdominal pain associated with IgAV is caused by submucosal hemorrhage and edema.

Abdominal pain is often associated with diarrhea with gross or occult blood, nausea, vomiting, constipation, and gastrointestinal bleeding (hematemesis or melena).

In most cases, on clinical examination, the abdomen is tender and slightly distended; rarely, it may resemble and be confused with an acute abdomen requiring surgery evaluation (suspicion of perforation, intussusception). Some of these cases, suggesting an acute abdomen, are leading to emergency computed tomography (CT) or unnecessary surgery [4, 23]. Sometimes, particularly in young patients, intense abdominal pain may suggest acute appendicitis, especially when the skin rash is absent. Even in the presence of rash (suggestive in a clinical context for Ig A vasculitis) in front of intense abdominal pain, the differential diagnosis of abdominal pain and the exclusion of acute surgical abdomen are mandatory; more, the IgA vasculitis rash may be a nonspecific erythematous or urticarial exanthem or limited to lesions on the buttocks or lower extremities early in the disease course.

*Gastrointestinal bleeding* is another symptom, with an incidence varying between 17.6 and 51% [23]. In most cases, the bleeding is occult (detected as positive stool occult blood); when manifested, the main symptom is melena rather than hematemesis or hematochezia, because the intestinal lesions are usually localized in the small bowel and colon. Gastrointestinal bleeding is usually mild; in rare cases it may be severe, requiring blood transfusion or surgical treatment, even leading to death [4, 23].

Other rare gastrointestinal manifestations, such as *esophageal ulcer*, *pancreatitis*, *pseudomembranous colitis*, *gall bladder involvement*, *intestinal perforation*, and *ischemic vasculitis* may occur [23].

Intussusception is the most common gastrointestinal complication of IgA vasculitis. Edema and hemorrhage can act as a pathological lead point, contributing to the development of intussusception. Intussusception is limited to the small bowel in approximately 60% of cases, in contrast to idiopathic intussusception, which is typically ileocolic. The overall incidence of this complication of IgA vasculitis varies between 2.3 and 3.5%, although some retrospective series reported an incidence of only 0.4 to 0.6% [9, 17]. Children with severe gastrointestinal pain and/or requiring hospitalization are at greater risk.

#### *4.4.1 Imaging evaluation of gastrointestinal manifestation*

*Upper gastrointestinal endoscopy* (UGD) is mandatory in patients with gastrointestinal bleeding. UGD is helpful in the diagnosis of IgA vasculitis, especially when gastrointestinal symptoms develop prior to the cutaneous lesions.

The most important part of upper gastrointestinal tract is involved in the second part of the duodenum with endoscopic features including diffuse mucosal redness, petechiae, severe erosive duodenitis, hemorrhagic lesion, and ulcers [28] (**Figure 2**). Purpuric lesions may be seen on an endoscopy, commonly in the descending duodenum, stomach, and colon.

The spectrum of upper endoscopic findings is based upon the severity of the vasculitis; usually, irregular, ulcerating, nodular lesions or hematoma-like protrusions are characteristic of IgA vasculitis in the duodenum. The stomach and colon are often involved as well, but the duodenal bulb is rarely affected; the absence of bulbar lesions is important to exclude the cause of the peptic disease hemorrhage [29]. The biopsies of gastrointestinal lesions are commonly performed in patients with or without suspected IgA vasculitis in order to rule out infection, inflammatory bowel disease, and less commonly, vasculitis. In general, vasculitis is not commonly observed in GI biopsies, and the spectrum of findings includes neutrophilic infiltrate within the small bowel and colon, with the duodenum most commonly affected. While the clinical and histologic findings may mimic early inflammatory bowel disease, the presence of predominant small bowel involvement, especially erosive duodenitis, should raise suspicion for IgA vasculitis. Biopsies should be obtained before steroid therapy is initiated, if possible [30].

Resolution of duodenal lesions is spectacular, in accordance with the remission of inflammatory disorders [31] (**Figure 3**).

*Colonoscopy*: erythema of the mucosa, petechiae, and ulcers are the most common findings [32].

*Computed tomography (CT) imaging:* the hallmarks of IgA vasculitis are multifocal symmetric, circumferential, regular wall thickening and engorgement of mesenteric vessels. Associated findings include free intraperitoneal fluid, ileus of the affected loop, vascular engorgement in the adjoining mesentery, and nonspecific lymphadenopathy [24]. The target sign is not specific; it can be seen in many other conditions such as ischemic bowel disease, inflammatory bowel disease, infectious enterocolitis, radiation enteritis, etc. [19].

In some selected cases, CT angiography can be used to visualize the site of the arterial or venous occlusion; however, a normal angiogram does not rule out the possibility of mesenteric ischemia [3]. Mesenteric vascular engorgement and skip areas are also seen in Crohn's disease, but terminal ileal involvement, stricture, fistula, and abscess would favor Crohn's disease over other conditions [12, 33].

**41**

*Gastrointestinal Manifestations of IgA Vasculitis-Henoch-Schönlein Purpura*

The diagnosis of IgA vasculitis (HSP) is usually based upon clinical manifestations of the disease, and in patients with an incomplete/unusual presentation, biopsy of the affected organ (e.g., skin or kidney) demonstrating predominantly IgA deposition supports the diagnosis. Although gastrointestinal involvement is frequent, the diagnosis of IgA vasculitis may be difficult when gastrointestinal

manifestations occur alone or precede the characteristic skin purpura.

Camelia Cojocariu1,2\*, Ana Maria Singeap1,2, Stefan Chiriac1,2, Catalin Sfarti1,2,

2 "St. Spiridon" Emergency Hospital, Institute of Gastroenterology and Hepatology,

© 2019 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

Irina Girleanu1,2, Oana Petrea1,2, Anca Trifan1,2 and Carol Stanciu2

1 University of Medicine and Pharmacy "Gr. T. Popa", Iasi, Romania

\*Address all correspondence to: cameliacojocariu@yahoo.com

provided the original work is properly cited.

*DOI: http://dx.doi.org/10.5772/intechopen.86966*

*Endoscopic appearance of the second part of duodenum remission.*

**5. Conclusion**

**Author details**

Iasi, Romania

**Figure 3.**

#### **Figure 2.**

*Endoscopic appearance of the second part of duodenum: multiple erosions, diffuse redness, submucosal hemorrhage, and small ulcerations.*

*Gastrointestinal Manifestations of IgA Vasculitis-Henoch-Schönlein Purpura DOI: http://dx.doi.org/10.5772/intechopen.86966*

**Figure 3.** *Endoscopic appearance of the second part of duodenum remission.*
