**8. Clinical picture**

Most patients with PHG are asymptomatic, but a significant number of patients exhibit symp‐ toms related to iron deficiency anemia from chronic GI bleeding. Bleeding from PHG may be either chronic occult blood in the stool, or overt which occurs in a smaller proportion of patients. Endoscopic diagnosis of acute hemorrhage from PHG is established when there is active bleeding from gastropathy lesions, if non‐removable clots overlying these lesions is observed, or when there is PHG and no other cause of acute bleeding can be demonstrated after thorough evaluation of the gastrointestinal tract [9]. Recurrent bleeding is common in PHG after the initial episode.

Although all PHG patients with chronic bleeding develop severe chronic iron deficiency ane‐ mia, no study has evaluated the prevalence of PHG in cirrhotic patients with chronic iron deficiency anemia. In patients with portal hypertension, with or without cirrhosis and chronic iron deficiency anemia, chronic bleeding from PHG must be suspected, its diagnosis is con‐ firmed by upper endoscopy. Comprehensive study of the whole gastrointestinal tract in these patients by capsule endoscopy is mandatory to exclude similar lesion elsewhere along gastro‐ intestinal tract e.g. colonopathy [27].

#### **8.1. Diagnosis**

Also, there is no evidence that there is any increase in the prevalence or severity of PHG with the use of either nonsteroidal anti‐inflammatory drugs (NSAIDs), or COX‐(cyclooxygenase)‐2 inhibitor [24]. Another negative correlation was found between alcohol and smoking along‐ side PHG, thus there is no benefit from abstinence or quitting smoking [25]. Likewise, the

Patients with portal hypertension and chronic iron deficiency anemia who are suspected to have chronic bleeding should go for PHG screening. Chronic bleeding is diagnosed when there is either decrease in hemoglobin level below 2 g/dL within 6‐month, or if there is pres‐ ence of iron deficiency anemia with a positive fecal occult blood test. For those patients, upper

PHG may change in an individual patient over time, about 30% of the patients with cirrhosis and mild PHG, progress to severe PHG during a 10 year follow up period, considering that these patients did not receive any prophylactic treatment. Most of the patients with worsening PHG, severe lesions, or *de novo* PHG develop bleeding. There are only a few improved cases in PHG without treatment [27]. Furthermore, Patients who have PHG associated with cirrho‐ sis‐related portal hypertension have more frequently persistent and progressive PHG, which is more likely to bleed than patients with PHG related to non‐cirrhotic portal hypertension [9]. Although, as mentioned above, patients with previous endoscopic therapy (sclerotherapy or endoscopic variceal ligation) have a higher prevalence of PHG, the clinical course of PHG in this context, particularly in non cirrhotic portal hypertension, may be milder and transient [28]. PHG is a dynamic condition emphasized by the observation that 30% of patients who have endoscopic features of PHG remained unchanged throughout follow‐up period, whereas 25% of patients show either worsening or improvement of the condition during the follow up period. Thus, not only can PHG appear for the first time or progress from mild to severe condition over time, but it can also revert from severe to mild, and even disappear completely

Most patients with PHG are asymptomatic, but a significant number of patients exhibit symp‐ toms related to iron deficiency anemia from chronic GI bleeding. Bleeding from PHG may be either chronic occult blood in the stool, or overt which occurs in a smaller proportion of patients. Endoscopic diagnosis of acute hemorrhage from PHG is established when there is active bleeding from gastropathy lesions, if non‐removable clots overlying these lesions is

presence of PHG was independent of patient's age, or sex.

**6. Whom should go for screening for PHG**

endoscopy can confirm the diagnosis of PHG [26].

**7. Natural history**

64 Stomach Disorders

with treatment [23].

**8. Clinical picture**

The diagnosis of PHG is done mainly by upper endoscopy. The endoscopic findings of PHG diagnosis was classified by the New Italian Endoscopic Club according to its severity based on the presence of four elementary lesions: mosaic like pattern, red point lesions, cherry red spots, and black brown spots (**Figure 1**) [29]. Early change in PHG is called scarletina, which appears as a fine pink speckling. On the other side, severe PHG appear as cherry red spots that may become confluent and is very friable, so it can actively bleed dur‐ ing endoscopy. These lesions are present predominantly in the fundus and/or the corpus of the stomach, yet PHG–like lesions have been described in other sites in the gastrointestinal tract e.g. the rectum, colon, and small bowel in asymptomatic patients and in patients with bleeding [29].

#### **8.2. Endoscopic evaluation of PHG**

The elementary lesions of PHG according to the New Italian Endoscopic Club for the Study and Therapy of Esophageal Varices [NIEC] classification are as follows [30]:


PHG is classified by endoscopy, into mild, moderate and severe forms. PHG mucosa can be seen as snakeskin (MLP) in mild cases, while RPLs, CRSs or BBCs that is liable to bleeding, are found in severe cases, however, the presence of red or brown spots without bleeding is considered a moderate disease [35].

**Figure 1.** Endoscopic images of portal hypertensive gastropathy that show the four main findings of this condition. Mild (A) and moderate (B) mosaic‐like gastric mucosal pattern, red point lesions (C), cherry‐red spots (D), and black–brown spots, including an intramucosal hemorrhage (E) and a brown spot (F).

#### **8.3. Classification of portal hypertensive gastropathy**

PHG is classified on the basis of the condition's severity, therefore many classifications of the PHG mucosa have been proposed. McCormack's classification, NIEC's and Tanoue's classifi‐ cations are the most popular used classifications (**Table 3**). McCormack classified the mucosal changes into two main categories, mild and severe, on the other hand, the NIEC classification has three categories; mild, moderate and severe, and further subdivided the mosaic‐like pat‐ tern into three groups [28]. Finally, the McCormack classification, a two‐category classifica‐ tion system, is the recommended one. The classification comprises of:

**Mild portal hypertensive gastropathy:** Only one change in the stomach mucosa is present, that of appearance of mosaic or snakeskin pattern on it.


**Table 3.** Endoscopic finding and classification of PHG.

**Severe portal hypertensive gastropathy:** In addition to the mosaic or snakeskin pattern of the stomach mucosa; bulging, flat red or black‐brown spots are seen. There also may be active bleeding.

In the rare instance where PHG cannot be clearly diagnosed on the basis of endoscopic appearance and location alone, biopsy for histology may prove useful [28].

#### **8.4. Histologically**

**8.3. Classification of portal hypertensive gastropathy**

spots, including an intramucosal hemorrhage (E) and a brown spot (F).

66 Stomach Disorders

that of appearance of mosaic or snakeskin pattern on it.

tion system, is the recommended one. The classification comprises of:

PHG is classified on the basis of the condition's severity, therefore many classifications of the PHG mucosa have been proposed. McCormack's classification, NIEC's and Tanoue's classifi‐ cations are the most popular used classifications (**Table 3**). McCormack classified the mucosal changes into two main categories, mild and severe, on the other hand, the NIEC classification has three categories; mild, moderate and severe, and further subdivided the mosaic‐like pat‐ tern into three groups [28]. Finally, the McCormack classification, a two‐category classifica‐

**Figure 1.** Endoscopic images of portal hypertensive gastropathy that show the four main findings of this condition. Mild (A) and moderate (B) mosaic‐like gastric mucosal pattern, red point lesions (C), cherry‐red spots (D), and black–brown

**Mild portal hypertensive gastropathy:** Only one change in the stomach mucosa is present,

The unique histological features of PHG are marked dilatation of the capillaries and collect‐ ing venules in the gastric mucosa with markedly congested and tortuous submucosal venules (**Figure 2**) [31]. These vascular features are present in the absence of any inflammatory cell

**Figure 2.** Hematoxylin and eosin shows numerous dilated capillaries in the superfacial gastric mucosa. These are not entirely specific for PHG.

infiltrate or erosion of the gastric mucosa. Stromal fibrosis and edema of the lamina propria can also be seen [31].

It may be difficult to differentiate between severe PHG and gastric antral vascular ectasia (GAVE) by endoscopic examination, so biopsy is beneficial in this case, as GAVE has distinct histological features that can set it apart from severe PHG. GAVE has many fibrin thrombi in the mucosal vessels with ectasia and spindle cell proliferation (smooth muscle and myofibro‐ blast hyperplasia) in the superficial mucosa. Furthermore, fibrohyalinosis is more commonly present in GAVE. All of these findings provide additional features that help in differentiating between severe PHG and GAVE [32].

#### **8.5. Portal gastropathy score calculation**

**Table 4** shows the scoring system to calculate the severity of PHG, the calculation depends on three categories: *Microscopic picture*; score 1 considered for mild findings, and score 2 for severe findings. *Red marks*; isolated lesion is considered score 1, whereas confluent red marks is scored 2. *GAVE*; if absent scored 0, while its presence is scored 2. If the total score was equal to 3 or less, the PHG is mild, on the other hand if the score is 4 or greater, PHG is severe [33].

#### **8.6. Other diagnostic modalities**

#### *8.6.1. Non‐endoscopic methods for diagnosis of HPG*

Other non‐endoscopic methods for the diagnosis of PHG such as MRI and CT are not yet considered as routine investigation for the diagnosis of PHG. Their results must be further evaluated to warrant their role in the diagnosis of PHG. Endoscopy still remains the main diagnostic method [34].

In CT scan, enhancement of the inner layer of the gastric walls due to gastric congestion, char‐ acterizes PHG lesion. Similarly, MRI is used to measure the diameter of collateral veins e.g. the left gastric, paraesophageal, and azygos veins to confirm the diagnosis of portal hypertension. In patients with PHG, the measurement of the diameters of these veins does not differ from those


Mild portal hypertensive gastropathy ≤ 3; severe portal hypertensive gastropathy ≥ 4.

**Table 4.** Portal hypertensive Gastropathy scoring system.

in patients without PHG, therefore it is not helpful in the diagnosis. These data suggest that these imaging techniques is still in its infancy period and best reserved for experimental purposes [35].

#### **8.7. Differential diagnosis**

infiltrate or erosion of the gastric mucosa. Stromal fibrosis and edema of the lamina propria

It may be difficult to differentiate between severe PHG and gastric antral vascular ectasia (GAVE) by endoscopic examination, so biopsy is beneficial in this case, as GAVE has distinct histological features that can set it apart from severe PHG. GAVE has many fibrin thrombi in the mucosal vessels with ectasia and spindle cell proliferation (smooth muscle and myofibro‐ blast hyperplasia) in the superficial mucosa. Furthermore, fibrohyalinosis is more commonly present in GAVE. All of these findings provide additional features that help in differentiating

**Table 4** shows the scoring system to calculate the severity of PHG, the calculation depends on three categories: *Microscopic picture*; score 1 considered for mild findings, and score 2 for severe findings. *Red marks*; isolated lesion is considered score 1, whereas confluent red marks is scored 2. *GAVE*; if absent scored 0, while its presence is scored 2. If the total score was equal to 3 or less, the PHG is mild, on the other hand if the score is 4 or greater, PHG is

Other non‐endoscopic methods for the diagnosis of PHG such as MRI and CT are not yet considered as routine investigation for the diagnosis of PHG. Their results must be further evaluated to warrant their role in the diagnosis of PHG. Endoscopy still remains the main

In CT scan, enhancement of the inner layer of the gastric walls due to gastric congestion, char‐ acterizes PHG lesion. Similarly, MRI is used to measure the diameter of collateral veins e.g. the left gastric, paraesophageal, and azygos veins to confirm the diagnosis of portal hypertension. In patients with PHG, the measurement of the diameters of these veins does not differ from those

Severe 2

Confluent 2

Absence 1 Presence 2

can also be seen [31].

68 Stomach Disorders

severe [33].

between severe PHG and GAVE [32].

**8.6. Other diagnostic modalities**

diagnostic method [34].

**Gastric antral vascular ectasia** 

**(GAVE)**

*8.6.1. Non‐endoscopic methods for diagnosis of HPG*

**Endoscopic findings Parameter Score Mucosal mosaic pattern** Mild 1

**Red markings** Isolated 1

Mild portal hypertensive gastropathy ≤ 3; severe portal hypertensive gastropathy ≥ 4.

**Table 4.** Portal hypertensive Gastropathy scoring system.

**8.5. Portal gastropathy score calculation**

The endoscopic findings of the gastric mucosa include many differential diagnoses of several disorders. When red spots are seen in the stomach by endoscopy, important diagnostic con‐ siderations are evoked. GAVE or watermelon stomach which is a common differential diag‐ nosis of PHG, has characteristic endoscopic findings which are linear red stripes, separated by normal mucosa, these findings give the appearance of a watermelon, that is seen in the gastric antrum or proximal stomach. GAVE and PHG are distinct entities, however both are encountered in cirrhotic patients. While PHG routinely affects the gastric body and fundus, GAVE almost exclusively inhabits the antrum [32].

These findings have a diagnostic accuracy of 85% for GAVE and help distinguish it from PHG.

**Table 5** further shows the differences between GAVE and PHG, however, there may be an overlap between GAVE and PHG in cirrhosis with portal hypertension [6].

Other conditions that increase the dilemma of PHG diagnosis include simple acute gastritis caused by non‐steroidal anti–inflammatory drug (NSAIDS) or *H. pylori*, which may have a mosaic like pattern endoscopically but, the main histopathological feature in gastritis is inflam‐ matory cell infiltration with minor vascular dilation, and it is also localized to the mucosa (superficial lesion). Lastly, endoscopic lesions similar to PHG may be also seen in some uncom‐ mon diseases e.g. polycythemia, gastric purpura, and Osler‐Weber‐Rendu disease [36].


**Table 5.** Comparison between portal hypertensive gastropathy and gastric antral vascular ectasia.
