Organs, such as the lungs, kidneys, heart, and digestive tract, may also be affected.
Reproduced with permission from DermNet New Zealandwww.dermnetnz.org2023.
Scleroderma Symptoms
There are two main types of scleroderma.
Reproduced with permission from © DermNet New Zealandwww.dermnetnz.org2023.
This article will focus mostly on systemic sclerosis (also known simply as scleroderma).
Localized Scleroderma
Localized scleroderma primarily affects only the skin and is seen mostly in children.
A subtype of limited cutaneous systemic sclerosis calledCREST syndromeis based on the characteristic symptoms that give it its name.
When scleroderma affects the kidneys, a rare but severe complication, calledscleroderma renal crisis, may develop.
This causes malignanthigh blood pressurealong with kidney failure.
Scleroderma-related heart problems may manifest asabnormal heart rhythmsorcongestive heart failure.
Lung complications are the leading causes of death in systemic sclerosis.
Experts suspect that a combination of genetics and exposure to environmental factors could be involved.
The condition usually develops between the ages of 25 to 55.
Diagnosis
No single test serves as a definitive diagnosis for scleroderma.
History and Physical Examination
You should discuss any symptoms with your healthcare provider.
Or you might be experiencing digestive problems, like acid reflux and swallowing.
Raynaud’s phenomenon is one of the earliest signs of disease in systemic sclerosis.
Raynaud’s phenomenon may also occur on its own, meaning it’s not associated withanyunderlying disease process.
Blood Tests
Various blood tests can support a diagnosis of scleroderma.
The vast majority of people with scleroderma are positive for theanti-nuclear antibody (ANA).
In other words, there is no medication that can stop or reverse the skin from hardening and thickening.
Here are some examples of how various symptoms/complications are treated in scleroderma.
Medications, such ascalcium channel blockersor phosphodiesterase key in 5 (PDE-5) inhibitors, may also be used.
For problems swallowing related to scleroderma, the drugReglan (metoclopramide)can provide some relief.
Captopril, a short-acting ACE inhibitor, is considered first-line for treating renal crisis.
And they are also treated with disease-modifying anti-rheumatic drugs (DMARDs), like hydroxychloroquine, and methotrexate.
Steroids are typically avoided because they can precipitate a renal crisis, especially in the diffuse form.
Coping
Living with a chronic, complex, disease like scleroderma is challenging.
Getting to know others who experience your same struggles can be immensely comforting and helpful.
To optimize your daily functioning and sense of well-being, it’s important to adopt healthy lifestyle habits.
American College of Rheumatology.Scleroderma.
In: StatPearls [Internet].
Treasure Island (FL): StatPearls Publishing; 2020 Jan-.
2015;44(6):680-6. doi:10.1016/j.semarthrit.2014.11.006
Adnan ZA.Diagnosis and treatment of scleroderma.Acta Med Indones.
Apr;40(2):109-12.
Hinchcliff M, Varga J.Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease.Am Fam Physician.
15;78(8):961-68.
National Institute of Health: Genetic and Rare Diseases Information Center.Systemic sclerosis.
Pattanaik D, Brown M, Postlethwaite BC, Postlethwait AE.Pathogenesis of Systemic Sclerosis.Front Immunol.2015;6:272. doi:10.3389/fimmu.2015.00272
