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Scleroderma Nursing Guide

The content was created by Tameka N. Warren, MSN, RN, CLC.

Scleroderma Overview

This course is intended as a Quick Reference for scleroderma and will provide an overview as well as nursing considerations utilizing the nursing process. 

Scleroderma Etiology and Epidemiology 

Scleroderma is a rare disease that affects the connective tissues of the body including the skin, tendons, and cartilage (National Institute of Arthritis and Musculoskeletal and Skin Diseases, 2020). Scleroderma is a chronic and progressive disorder that may present with mild to severe symptoms or present in a fulminant onset that can be life threatening. 

Scleroderma is classified as an autoimmune rheumatic and connective tissue disease that causes inflammation and abnormalities in the affected body systems and tissues (Mecoli, 2020). The effects of inflammation lead to overproduction of collagen that leads to thickening, hardening, tightening, and indurations of skin and underlying structures and/or fibrosis of internal organs (Scleroderma Foundation, 2021). 

The cause of scleroderma is unknown; it is not contracted or inherited, though a genetic predisposition to develop it can be identified. Disease manifestations are attributed to several factors, including (Jimenez, 2020a): 

  • Overproduction and excessive accumulation of collagen and other macromolecules in skin and other internal organs. 
  • Fibroproliferative alterations in microvasculature. 
  • Humoral and cellular immunologic abnormalities. 

The causative triggering of these manifestations is unclear, but may be due to environmental factors including, but not limited to (Jimenez, 2020a): 

  • Exposure to: 
    • Radiation 
    • Benzene 
    • Epoxy resin 
    • Carbon tetrachloride 
    • Silica 
    • Trichloroethylene 
    • Vinyl chloride 
  • Radiotherapy 
  • Pembrolizumab (Keytruda®) 

Symptoms of scleroderma may manifest in two main disease types including (John Hopkins Medicine, 2021): 

  • Localized scleroderma: The skin and underlying structures are affected. 
    • Linear 
    • Morphea (circumscribed and generalized) 
  • Systemic scleroderma: Referred to as systemic sclerosis, multiple systems and/or organs of the body may be affected, which can be further classified into three subtypes
  • Limited: (Most common type of scleroderma) CREST acronym for symptoms 
    • Calcinosis 
    • Raynaud’s phenomenon 
    • Esophageal dysmotility 
    • Sclerodactyly 
    • Telangiectasias 
  • Diffuse: Large areas of skin affected and/or body systems 
    • Dryness, itching, and pain 
    • Acute severe hypertension 
    • Possible life-threatening outcomes if lungs and heart are severely damaged 
  • Sine: Absence of cutaneous skin thickening but internal symptoms and complications of diffuse or limited scleroderma are present. 

Scleroderma can affect anyone, however (American College of Rheumatology [ACR], 2020): 

  • Women are more frequently affected than men. 
  • It occurs most often between the ages of 30 and 50. 
  • Choctaw Native Americans and Black individuals have an increased risk. 
  • Some individuals may have a slightly higher risk of scleroderma with a familial history of scleroderma, lupus, or other autoimmune connective tissue diseases. 

Symptoms vary due to the many body systems that may be affected by the disease process and may include (Jimenez, 2020b): 

  • Cardiac: 
    • Dyspnea 
    • Arrythmias 
    • Syncope 
    • Congestive heart failure 
    • Myocardial infarction 
  • Ears, nose, throat (ENT): 
    • Blindness 
    • Hoarseness 
    • Sicca syndrome 
    • Poor dentition/loose dentition 
    • Decreased oral aperture 
  • Gastrointestinal (GI): 
    • Gastroesophageal reflux 
    • Dyspepsia 
    • Early satiety 
    • Malnutrition 
    • Gastric reflux 
    • Peristalsis (decreased or absent) 
    • Chronic iron deficiency 
  • Genitourinary: 
    • Bladder fibrosis 
    • Vaginal: 
      • Dryness 
      • Narrowing 
      • Pain 
    • Erectile dysfunction 
  • General: 
    • Fatigue 
    • Weakness 
  • Weight loss 
  • Integumentary: 
    • Diffuse pruritus 
    • Hyper- or hypopigmentation 
    • Tightened skin 
    • Skin induration 
    • Edema 
    • Thinning hair 
    • Loss of skin creases 
  • Musculoskeletal: 
    • Joint flexion contractures 
    • Tendon friction rub 
    • Weakness of muscles 
    • Decrease in joint range of motion 
    • Carpal tunnel syndrome 
  • Neurologic: 
    • Headache 
    • Paresthesia of the hands 
    • Facial pain 
  • Psychiatric: 
    • Anxiety 
    • Depression 
  • Renal: 
  • Respiratory: 
    • Chest pain 
    • Dyspnea 
    • Dry persistent cough 
  • Vascular: 
    • Cutaneous and mucosal telangiectasia 
    • Pitting ulcers in fingertips 
    • Raynaud’s disease 

Complications of systemic sclerosis may include (Jimenez, 2020a): 

  • Digital infarctions 
  • Renal failure
  • Pulmonary hypertension 
  • Myocardial Infarction 
  • Congestive heart failure 
  • Myositis 
  • Wound infections (and subsequent finger amputations)

Scleroderma Diagnosis 

To aid in the diagnosis, a classification criteria tool for systemic sclerosis was created by the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR), formerly the European League Against Rheumatism. Each section of the criteria is scored. A score greater than or equal to nine is classified as a definite case of systemic sclerosis (Gibson & Afzal, 2020). The sections include the following criteria (Gibson & Afzal, 2020): 

  • Skin thickening of the fingers of both hands extending proximally to the metacarpophalangeal joints = 9 
  • Skin thickening of the fingers: 
    • Puffy fingers = 2 
    • Sclerodactyly = 4 
  • Fingertip lesions: 
    • Digital tip ulcers = 2 
    • Fingertip pitting scars = 3 
  • Telangiectasia = 2 
  • Abnormal nail-fold capillaries = 2 
  • Pulmonary arterial hypertension and/or interstitial lung disease = 2 
  • Raynaud phenomenon = 3 
  • Systemic sclerosis related autoantibodies = 3 

Laboratory testing may include (Jimenez, 2020): 

  • Autoantibody assays 
  • Complete blood cell count (CBC) 
  • Serum muscle enzyme levels 
  • Serum CXCL4 level 
  • Erythrocyte sedimentation rate 
  • N-terminal pro-brain natriuretic peptide 

Imaging may include (ACR, 2020): 

Other diagnostics test to determine disease severity and affected body systems may include (Jimenez, 2020a): 

  • Electrocardiograms (ECGs) 
  • Transthoracic echocardiography 
  • Right-heart catheterization 
  • Pulmonary function studies 
  • Bronchoscopy with bronchoalveolar lavage 
  • Esophagogastroduodenoscopy with appropriate biopsies 
  • Esophageal manometry assessment 
  • Esophageal pH studies 
  • Nail-fold capillary microscopy 

Scleroderma Management 

Scleroderma is not curable, and any damage caused to body organs is irreversible. Early identification of multisystem involvement is imperative to prevent permanent damage. 

Management and treatments options are aimed towards symptom management and vary depending on the disease process specific to the individual. Management may involve pharmacological interventions, including (ACR, 2020): 

  • Anti-inflammatory drugs: Intravenous immunoglobin (IVIg) and/or immunosuppressive medications for muscle pain/weakness 
  • Calcium channel blockers for Raynaud’s phenomenon 
  • Phosphodiesterase-5 (PDE-5) inhibitors for Raynaud’s phenomenon 
  • Angiotensin converting enzyme (ACE) inhibitors for kidney disease 
  • Proton-pump inhibitors for gastro-esophageal reflux disease (GERD) 
  • Prostacyclin-like drugs and endothelin receptor antagonists for pulmonary hypertension 

Other management options may include (Mayo Clinic, 2020): 

  • Physical and/or occupational therapy to promote movement and ease stiffness 
  • Topical medications such as steroid skin creams, antibiotic ointments, and moisturizers 
  • Surgeries such as amputation and lung transplants 

Scleroderma Nursing Care Plan

Nursing Considerations 

Use the nursing process to develop a plan of care for individuals. The nursing assessment (with common findings listed), diagnoses, interventions, expected outcomes, and education for scleroderma are listed below. 

Assessment 

A thorough history should be taken including: 

  • Past and present health conditions 
  • Family health history 
  • Current symptomology 
  • Medication history 
  • Work history (determine exposure risk factors) 

Head to toe assessment findings may include: 

  • Skin: 
    • Thickening of skin 
    • Pigment changes (hyper and hypopigmentation) 
    • Telangiectasias of the face, hands, and chest
    • Tight and shiny skin 
    • Decreased sweating 
    • Lack of skin folds 
    • Edema 
    • Skin induration 
    • Calcinosis 
    • Hair loss 
  • Cardiac: 
    • Arrhythmias 
    • Cor pulmonale 
  • ENT: 
    • Xerophthalmia 
    • Tooth decay 
    • Loose teeth 
    • Xerostomia 
  • GI: 
    • Esophagitis or candida esophagitis 
    • Esophageal strictures 
    • Gastroparesis 
  • Musculoskeletal: 
    • Acroosteolysis 
    • Contractures of flexion joints 
    • Generalized arthralgias 
    • Hand and joint decreased function 
    • Myositis 
    • Decreased range of motion 
  • Respiratory: 
    • Dry rales 
    • Pulmonic secondary heart sound (P2) or right ventricular heave 

The individual may complain of the following symptoms: 

  • Dry, itchy, and tight skin 
  • Fatigue 
  • Weakness 
  • Dyspnea 
  • Palpitations 
  • Headache 
  • Dizziness 
  • Bloating 
  • Constipation 
  • Fecal incontinence 
  • Weight loss 
  • Poor appetite 
  • Hoarseness 
  • Cough 
  • Dry mouth 
  • Arthralgia 
  • Myalgia 
  • Morning stiffness 
  • Pain in the joints, chest, and/or face 
  • Vaginal dryness 
  • Menstrual irregularities 
  • Depression 
  • Anxiety 

Nursing Diagnosis/Risk For 

  • Acute pain 
  • Anxiety 
  • Deficient knowledge of disease and disease processes 
  • Infection 
  • Impaired skin integrity 
  • Impaired tissue perfusion 
  • Fatigue 
  • Stroke 
  • Altered levels of consciousness 
  • Acute confusion 
  • Activity intolerance 
  • Imbalanced fluid volume 
  • Unstable blood pressure 
  • Imbalanced nutrition 
  • Impaired gas exchange 
  • Ineffective beathing pattern 
  • Impaired swallowing 
  • Ineffective airway clearance 
  • Falls 
  • Body image disturbance 
  • Renal alteration 
  • Cardiovascular impairment 
  • Injury 
  • Depression 
  • Aspiration 

Scleroderma Interventions 

Appropriate interventions will be determined by the site and severity of the individual’s scleroderma but may include the following: 

  • Monitor: 
    • Vital signs
    • Efficacy of treatment 
    • Intake and output 
    • Neurological status 
    • Cardiovascular status 
    • Respiratory status 
    • Pulse oximetry readings 
    • Arterial blood gas (ABG) 
  • Provide: 
    • Comfort measures 
    • Oral care 
    • Wound care 
    • Adequate skin moisturizers 
  • Encourage: 
    • Activities 
    • Ambulation 
    • Exercise 

Expected Outcomes 

The individual will report and laboratory tests will confirm: 

  • Reduction of symptoms and/or little/no progression 
  • Vital signs within a normal clinical range 
  • Decrease in pain 
  • Compliance with medication regimen and ordered therapy services 
  • No report of falls and/or injuries 

Individual/Caregiver Education 

  • Disease process: Various topics needed for adequate management include: 
    • Avoid cold and/or wet environments 
    • Wear warm clothing 
    • Importance of sunscreen, oral care, and stress management 
  • Healthy diet options to aid in constipation relief and decrease heartburn 
  • Smoking cessation 
  • Self-care: 
    • Adequate sleep and rest 
    • Staying active 
    • Deep breathing 
  • Medication compliance: Importance of taking medication as prescribed to manage symptoms and irreversible changes 
  • Awareness of medication adverse reactions/side effects and how to manage them and notify the provider 
  • Referrals for support groups 

Scleroderma Course

This course reviews the pathological process, manifestations, and treatment options for people with scleroderma.

View Course

Additional Information

Content Release Date

4/1/2022

Content Expiration

12/31/2027

Course Contributor 

The content for this course was created by Tameka N. Warren, MSN, RN, CLC. 

Tameka N. Warren, MSN, RN, CLC, earned her Bachelor and Master of Science in Nursing degrees from Indiana University School of Nursing at IUPUI, and her Associate of Science in Nursing from Ivy Tech College. She has over 16 years of nursing experience in public health, regulation, home health, education, maternal child community health, and long-term care environments. Tameka served as a Public Health Nurse Surveyor (Home Health & Hospice) certified by the Centers for Medicare & Medicaid Services, or CMS, a maternal and infant mortality advocate, an ALPP Certified Lactation Counselor, and an Adjunct Clinical Professor. Tameka’s passion is to serve the most vulnerable populations affected by health disparities and to inspire a new generation of nurses to do the same successfully.

References 

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