Scleroderma or systemic sclerosis (SSc) is a term used to describe a connective tissue disorder that is characterized by thickening and fibrosis of the skin.
Localized scleroderma and systemic sclerosis. Localized scleroderma is skin thickening without any organ involvement and includes three subset conditions. Morphea. Linear scleroderma. Scleroderma en coup de sabre.
Systemic sclerosis is a multiorgan disease characterized by fibrosis and vasculopathy. Systemic sclerosis includes skin fibrosis along with variable severe involvement of diverse internal organs.
It is further divided into two major subgroups:
(1) Limited cutaneous SSc (lcSSc), which involves skin thickening of the face, neck, and distal to the elbows and knees; and
(2) diffuse cutaneous SSc (dcSSc), which affects the skin in a more generalized distribution including the proximal and distal extremities, face, neck, and trunk. Clinical features of the major subsets of systemic sclerosis are summarized:
Limited SSc:
Skin sclerosis, distal limbs, and face
Long preexisting RP symptoms
Lower frequency of severe lung fibrosis and renal crisis
High burden of nonlethal morbidity
Diffuse SSc:
Proximal skin sclerosis
Shorter preexisting RP history
High frequency of severe lung fibrosis
Increased risk of scleroderma renal crisis
Inflammatory skin changes and pruritus common for first 1-3 yr
Sine SSc:
Features of RP with scleroderma-associated ANA reactivity and at least one internal organ manifestation of SSc
Frequency uncertain because of likely underdiagnosis
Overlap SSc:
Cases that fulfill classification criteria for SSc and that are diagnosed as SSc but also show features of another autoimmune rheumatic disease
Most often myositis
Other cases of lupus, arthritis, or vasculitis
Comprise up to 20% of SSc cohorts
The following points are some therapeutic options that may apply to ***. Therapeutic decision making directed under care of rheumatology or dermatology and pulmonary.
The management of systemic sclerosis (SSc) is complex, evolving, and requires a multidisciplinary approach and the lead physicians in her case are rheumatology or dermatology, pulmonary medicine and GI specialist.
Numerous targeted therapeutic options for SSc, including skin fibrosis, are emerging and include B-cell
depletion, anti-interleukin 6, Janus kinase, and transforming growth factor b inhibition.
For Skin fibrosis: HSCT, MTX, Corticosteroids, Cyclophosphamide, or Rituximab.
For Skin and lung fibrosis in Ssc: anti-CTLA4 (abatacept), anti-IL-13/4 (romilkimab), transforming
growth factor-b antibody (fresolimumab), cannabinoid receptor analogs (lenabasum), and Janus kinase inhibitors (tofacitinib).
For Raynaud's phenomenon: CCB, IV iloprost, PDE type 5, and Angiotensin II receptor antagonists
For Digital ulcers: CCB, PDE type 5, IV iloprost, Bosentan.
For SSc-ILD:
• Mycophenolate mofetil and/or cyclophosphamide are considered first line treatment options for SSc-ILD.
• Autologous HSCT and lung transplantation are reserved for severe or progressive cases.
• Nintedanib, a small molecule tyrosine kinase inhibitor, has recently been FDA approved for SSc-ILD,
• Riociguat (soluble guanylate cyclase (sGC) stimulators) and PDE type 5 for pulmonary arterial hypertension.
Cardiac:
• Standard treatments for ischemic heart disease, valvular disease, arrhythmias, diastolic and/or systolic dysfunction is indicated, which includes ACE inhibitors, diuretics, and implantable defibrillators.
• Myocarditis and pericarditis may respond to immunosuppression with MMF or corticosteroids, NSAIDs
and/or colchicine are additional options for the latter condition.
• Interventions such as pericardiocentesis for pericardial effusion and/or creation of a pericardial window
in cases of tamponade may be indicated.
Gastrointestinal:
• GERD - Proton-pump inhibitors, H2 blockers, and antacids are used for GERD.
• GI dysmotility - Prokinetic agents; eg, metoclopramide and domperidone
• Esophageal strictures: Patients may require endoscopic dilatation.
• Small intestinal bowel bacterial overgrowth (SIBO): Can be treated with various protocols of rotating antibiotics, such as ciprofloxacin, norflaxacin, amoxicillin and metronidazole.
• GAVE (gastric antral vascular ectasia): Management involves correction of anemia, iron supplementation, and, in some cases, endoscopic treatment with argon plasma photo-coagulation or with radiofrequency ablation. There are case reports of intravenous cyclophosphamide in refractory GAVE
ACE inhibitors have a role in management of cardiac dysfunction as well as Scleroderma Renal
Crisis (SRC).
Ultimately, a multidisciplinary approach is vital, and for each organ system involved, specialist care is indicated.