**4.3 Pulmonary involvement**

*Vascular Biology - Selection of Mechanisms and Clinical Applications*

**4.2 Gastrointestinal system involvement**

*Nasal sharpening and perioral vertical streaks in a patient.*

and have anorectal involvement [29].

Ninety percent of scleroderma patients show gastrointestinal involvement. Although it can involve any part of the gastrointestinal tract from the mouth to the anus, the esophagus is the most commonly involved area [27]. The most common complications of SSc in the oral cavity are microstomy and xerostomy. Symptoms of esophageal disease depend on dysmotility and reflux, and related to this, dysphagia, odynophagia, regurgitation, pyrosis, chronic cough, or hoarseness can occur. Stricture, Barrett's esophagus, aspiration pneumonia, and adenocarcinoma may develop as complications in these patients [27, 28]. The two most common symptoms of SSc in the stomach are gastroparesis and gastric antral vascular ectasia (GAVE). A typical striped watermelon appearance is present. Iron deficiency anemia due to GAVE may also be seen [28]. After bacterial growth due to small intestinal involvement, diarrhea and malabsorption or pseudo-obstruction due to hypomotility and dilatation may be observed [28]. Constipation, fecal, rectal prolapse, spontaneous perforation, and colon infarction may develop in the colon

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**Figure 1.**

**Figure 2.**

*Digital ulcer in a patient.*

Pulmonary involvement, the most important cause of mortality and morbidity in SSc, may occur in the form of interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH) [30].

The highest risk for ILD development is within the first 5 years of the disease. Progressive exercise dyspnea and nonproductive cough are the most common symptoms. Diagnosis is placed with the help of imaging methods and pulmonary function tests. High-resolution computed tomography is a sensitive method in imaging and can show the degree of fibrosis.

Pulmonary artery hypertension affects approximately 15% of scleroderma patients and leads to right heart failure, and the gold standard method for the diagnosis of PAH is right heart catheterization with high pressure in the pulmonary artery [31].
