October 01, 2020 Updated: September 23, 2021 5 min read 1 Comment
In this article, we go into detail on systemic sclerosis (scleroderma) which is an autoimmune disease translating to “hardened skin.” Though this disease is uncommon, it frequently shows up on Medical-Surgical Nursing exams and you may see patients with it, so it is important to learn. We cover the pathophysiology, limited vs. diffuse scleroderma, risk factors, signs and symptoms, diagnosis and lab values, treatment with medications, and nursing care and patient teaching.
This disease is covered in our Medical-Surgical flashcards (Immune system), and Cathy’s video follows along with the cards.
Scleroderma is an autoimmune disorder marked by chronic inflammation of the body’s connective tissue, resulting in damage and occlusion to the blood vessels (vasculitis). Scleroderma also causes overproduction of collagen, which causes tissues to become inflamed, fibrotic and sclerotic (hard).
Approximately 20% of the patients who have scleroderma also have lupus. Unfortunately, patients can have more than one autoimmune disease, which is called polyautoimmunity.
There are two main types of scleroderma — limited and diffuse. Limited scleroderma is when the skin thickening is limited to the extremities, like finger, hands and toes. With diffuse scleroderma, skin thickens over most of the body and some of the internal organs, like the heart, lungs and kidneys.
There are several risk factors associated with scleroderma. Women are more likely than men to get scleroderma, and the age of onset is often between 30 and 50 years of age.
The signs and symptoms of scleroderma include arthralgia (joint pain); Raynaud’s syndrome, which is characterized by periods of vasospasming causing limited blood flow to the fingers and toes which causes those extremities to be pale, cold and numb; pitting edema in the hands marked by taut, shiny skin.
If scleroderma reaches the esophagus, the patient may have reflux or dysphagia (difficulty swallowing).
If scleroderma reaches the heart the patient may develop arrhythmias or dyspnea (difficulty breathing). Scleroderma in the lungs and the resulting fibrosis of the lung tissue can also cause dyspnea.
If the patient develops sclerosis of the kidneys, the blood pressure may become severely elevated, so you may see malignant hypertension in a patient with scleroderma. Malignant hypertension is hypertension that develops rapidly, causes organ damage, and is usually above 180/120.
The diagnosis and lab values associated with scleroderma are similar to that of lupus. In a patient with scleroderma, they will have a positive antinuclear antibody (ANA) titer. You may also see fast erythrocyte sedimentation rate (ESR) indicating inflammation.
Normal levels of ESR are covered in our Lab Values flashcards for nursing students!
There is no cure for scleroderma, so treatment is usually supportive. Immunosuppressants like prednisone or methotrexate can be given to a patient with scleroderma to calm the immune response and prevent escalation of symptoms. The patient can also be given ACE inhibitors like captopril.
Prednisone, methotrexate and captopril are all covered in our Pharmacology Flashcards for nursing students, including their modes of action, side effects, and contraindications; whether you need to study them for Med-Surg, Pharm, or you just need a refresher!
Patients with scleroderma should be encouraged to moisturize their skin as this can prevent it from drying out and becoming more hardened. They should take frequent rest periods. They should avoid stress and cold. To avoid cold, they should wear socks and really focus on keeping themselves warm, because if they get cold, the Raynaud’s syndrome side effect will worsen.
Cathy’s teaching on the immune system is intended to help prepare you for Medical-Surgical nursing exams. The Medical-Surgical Nursing video series is intended to help RN and PN nursing students study for nursing school exams, including the ATI, HESI and NCLEX.
Alright! In this video, I'm going to talk about systemic sclerosis or scleroderma.
If you're following with cards, I'm on card 14.
Scleroderma is a chronic inflammatory connective tissue disorder. It is estimated that 20% of the patients who have scleroderma also have lupus, which we just talked about in my previous video.
So scleroderma is an autoimmune disorder, and it results in damage and occlusion to the blood vessels, so vasculitis. And it also causes overproduction of collagen, which causes tissues to become inflamed, fibrotic, and then sclerotic, so they become hard.
There are two types of sclerosis.
We have limited. So this is where we have skin thickening that is limited to the extremities.
But then we also have diffuse scleroderma which involved skin thickening over most of the body as well as organ involvement.
In terms of risk factors associated with this disorder, women are more likely to get this just like what we saw with lupus, and the age of onset is typically between 30 and 50 years of age.
The symptoms of scleroderma include arthralgia, which is a fancy name for joint pain as well as Raynaud's. So this is where we have vasospasming that limits the blood flow to the extremities, to the fingers or the toes, so those fingers become pale, cold, and numb.
In addition, we'll have pitting edema in the hands with taut, shiny skin over the hands.
With organ involvement, we may see things such as GI dysfunction. So the patient may have reflux or difficulty swallowing, so that's dysphagia. They may have arrhythmias.
They may have difficulty breathing, so dyspnea, due to fibrosis of the lung tissue and the heart tissue.
And then, with renal involvement, so if we have sclerosis of the kidneys, that can send the blood pressure sky-high, so the patient may have malignant hypertension associated with scleroderma.
In terms of the labs and diagnosis of this condition, just like with lupus, we will have a positive ANA titer, so that's anti-nuclear antibody, and that titer will be positive. In addition, we'll have elevated ESR indicating the presence of inflammation in the body.
In terms of treatment, there is no cure for scleroderma, so treatment is really supportive. We can provide immunosuppressants such as prednisone or methotrexate. We can also give ACE inhibitors such as captopril.
In terms of teaching, we definitely want to encourage our patient to moisturize their skin.
They should take frequent rest periods.
And then, they should avoid stress and cold. So they should wear socks and really keep themselves warm because if they get cold, then that Reynaud's side effect will definitely get worse.
So that's it with scleroderma. When I come back, we will talk about HIV. Thanks for hanging in there with me, and I'll see you on another video soon!
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