**5. Autoantibodies**

Antinuclear antibodies (ANA) are 90–95% positive in SSc patients and are the most commonly detected autoantibody. Scleroderma-like diseases should also be considered in the case of ANA negativity. Anti-topoisomerase I (anti-scl 70) antibodies are connected to pulmonary complications, digitals ulcers, and progressive hand involvement. Anti-centromere antibodies are common in limited SSc and positively increase the risk of pulmonary fibrosis and pulmonary hypertension. Anti-RNA polymerase III antibody is associated with renal crisis. Also anti-U3RNP and anti-Th/ To antibodies can be detected positively, and the anti-U3RNP antibody increases the risk of pulmonary artery hypertension and cardiovascular complications [37].

#### **6. Histopathology**

The diagnosis of systemic sclerosis is placed clinically; therefore biopsy is not recommended routinely [38]. It can be used to rule out other diseases for differential diagnosis. Histologically, excessive collagen accumulation, atrophy of pilosebaceous and eccrine glands, subcutaneous fat loss, and lymphocytic infiltrate are observed. Increased collagen can compress adnexal structures, especially eccrine glands [38].

#### **7. Differential diagnosis**

There are many diseases that may trigger dermal sclerosis. The form and character of skin involvement, history of underlying diseases, and chemical exposures are helpful factors in approaching the diagnosis of a patient with skin thickening. Some laboratory studies may be beneficial in verifying imaging and skin biopsy diagnosis [8]. Eosinophilic fasciitis, scleroderma, scleromyxedema, and nephrogenic systemic fibrosis are important in the differential diagnosis of SSc. SSc can be differentiated from other scleroses by the presence of Raynaud's phenomenon, typical distal extremity involvement, nail fold capillary findings, presence of autoantibodies, and internal organ involvement. While the groove mark, which is a recess caused by the withdrawal of subcutaneous tissues along the path of the superficial vessels, favors the eosinophilic fasciitis [39], the detection of monoclonal gammopathy directs

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native treatment [41, 45].

*Systemic Sclerosis*

**8. Treatment**

*DOI: http://dx.doi.org/10.5772/intechopen.91318*

nephrogenic systemic fibrosis [40].

agents and organ-specific drugs [41].

effective and used in selected cases [42].

amputations may be the causes for morbidity in SSc.

is one of the developing methods in the treatment of DU [43].

guanylate cyclase agonist riociguat has been involved in treatment [41].

the diagnosis towards scleroderma or scleromyxedema (bbb). Again, if underlying renal failure or gadolinium exposure is detected, the first thought should be towards

The pathogenesis of SSc is still unclear. In recent years, advances have been made in the treatment of the disease, resulting in a prominent improvement in survival rates. The efficient treatment of complications increases the chances of success. Disease duration, complications, and disease activity should be taken into consideration when making therapeutic decisions. The treatment is based on modifying

Peripheral vascular involvement frequently occurs as Raynaud's phenomenon. In addition, digital ischemia due to digital vasculopathy, digital ulcers, and associated

Patients with Raynaud's phenomenon should protect themselves from the cold.

Early treatment of patients with digital ischemia SSc reduces the risk of morbidity. Intermittent infusion of prostacyclin or analogs was found to be effective in the treatment of RF and ischemic digital ulcer [41]. Sildenafil and bosentan are recommended for the treatment of digital ulcers that developed due to unsuccessful treated systemic sclerosis with calcium channel blockers and prostanoid therapies [41]. There are also uncontrolled studies suggesting that atorvastatin, vitamin E, and intravenous N-acetylcysteine may be beneficial. Mesenchymal stem cell therapy

The first choice for the treatment of pulmonary artery hypertension is phosphodiesterase type 5 inhibitors (e.g., sildenafil or tadalafil) or endothelin receptor antagonists such as bosentan and macitentan. If those are not effective, prostanoids may be added to the treatment [44]. Prostanoids are the first choice in severe cases. If there is no response, combination treatments can be applied. Recently, the

The treatment of interstitial lung disease patients is based on immunosuppressive drugs. The first preferred agent is oral mycophenolate mofetil (MMF). One of the applied treatment regimens for nonresponsive patients is the administration of cyclophosphamide (CYC) orally at doses of 1–2 mg/kg/day or iv 600 mg/m2

month. After the disease activity has been taken under control, it is recommended to continue treatment with azathioprine at a dose of 2.5 mg/kg/day [41]. In selected patients, rituximab (RTX, anti-CD20 monoclonal antibody) can be used as an alter-

Immunosuppressants such as methotrexate (MTX), cyclophosphamide, and mycophenolate mofetil are commonly used in fibrosis of the skin [31]. If the treatment is unsuccessful or if these drugs cannot be used for whatever reason, low-dose systemic corticosteroids or rituximab may be preferred [31]. The effect of

In systemic sclerosis, exertional dyspnea, tachycardia, and chest pain may occur due to myocardial involvement. Selective beta-blockers are effective in this kind

d-penicillamine, which has been used for many years, is controversial [46].

/

They also should not smoke and should avoid vasoconstrictor agents. Calcium channel inhibitors (nifedipine, diltiazem, amlodipine) should be the first choice as treatment [41]. Iloprost and other intravenous prostanoids can be used in cases that do not respond to the above. Also, phosphodiesterase type 5 (PDE5) inhibitors may be effective in resistant cases. Selective serotonin reuptake inhibitors, pentoxifylline, prazosin, and endothelin receptor antagonists (ERA) are agents that are less

the diagnosis towards scleroderma or scleromyxedema (bbb). Again, if underlying renal failure or gadolinium exposure is detected, the first thought should be towards nephrogenic systemic fibrosis [40].
