**9. Result**

*Vascular Biology - Selection of Mechanisms and Clinical Applications*

Pulmonary arterial hypertension

Skin and lung disease

*systemic sclerosis; RP, Raynaud's phenomenon.*

*Treats and reduces the formation of new ulcers.*

**Organ involvement Recommendation Strength of** 

• PDE-5 inhibitors A • Prostanoids A • Fluoxetine C

• PDE-5 inhibitors A • Bosentan\* A

• ERA, PDE-5 inhibitors, riociguat B

• Intravenous epoprostenol A

• HSCT A

Prokinetic drugs C Antibiotics L

• Other prostacyclin analogs (iloprost,

• \*\*Glucocorticoids are associated with a

*ERA, endothelin receptor antagonists; HSCT, hematopoietic stem cell transplantation; PAH, pulmonary arterial hypertension; PDE-5, phosphodiesterase type 5; PPI, proton pump inhibitor; SRC, scleroderma renal crisis; SSc,* 

Raynaud's phenomenon • Calcium antagonists A

Digital ulcers • Intravenous iloprost A

treprostinil)

Skin • Methotrexate A Lung disease • Cyclophosphamide A

SRC • ACE inhibitors **C**

higher risk of SRC

Gastrointestinal disease PPI B

**recommendation**

B

**C**

of symptoms [33]. If cardiac tamponade develops due to pericarditis in cardiac involvement, treatment becomes more difficult. The patient may not respond to systemic corticosteroid therapy, and drainage treatment may become necessary [47]. Some SSc patients may develop microvascular ischemia due to vasospasm of small coronary arteries and arterioles, also called cardiac Raynaud's phenomenon.

*\*\*Blood pressure and renal function should be carefully monitored in SSc patients treated with glucocorticoids.*

SSc patients with GI involvement of dysphagia, pyrosis, esophageal reflux, esophagitis, distention, abdominal pain, and diarrhea can be treated with proton pump inhibitors (PPI), prokinetic drugs, and intermittent antibiotics (gg). Patients that developed gastric antral vascular ectasia may have severe upper GI bleeding. Here, supportive treatment and endoscopic treatment methods can be used. Surgical antrectomy becomes necessary as the last resort in resistant cases [49]. The first choice for patients with scleroderma renal crisis is a high dose of angiotensin receptor antagonists (ACE-I). It was determined that this treatment significantly decreased mortality [31]. In cases with insufficient response, angiotensin receptor blockers (ARB) and calcium channel blockers may be combined with ACE-I treatment of nitrates [31]. Beta-blockers are not recommended due to

Nifedipine treatment is quite effective in such patients [48].

*The updated EULAR recommendations for treatment of systemic sclerosis.*

**128**

*\**

**Table 2.**

As a result, we should not forget that systemic sclerosis is a chronic disease that can cause serious morbidity and mortality and that placing a diagnosis can be difficult from time to time. The disease does not have a specific treatment. The patient should be evaluated according to involved systems and clinical table. In addition, early diagnosis and early initiation of treatment increase the chances of efficacy. A better understanding of the pathogenesis may lead to the development of more successful and specific treatment methods.
