Systemic Sclerosis Diagnosis Symptoms Treatment Management

 Diagnosis Systemic Sclerosis —

Scleroderma (systemic sclerosis [SSc]) is a chronic disease of unknown cause characterized by diffuse fibrosis of skin and visceral organs and vascular abnormalities

History

  • Raynaud phenomenon is frequently the initial complaint.
  • Skin thickening, “puffy hands,” and gastroesophageal reflux disease (GERD) are often noted early in the disease process.

Systemic Sclerosis Physical Exam

  • · •BP
  • · •Skin:
    • o Tightness, swelling, thickening of digits
    • o Subcutaneous calcinosis
    • o Scaling of skin
    • o Pruritus
    • o Narrowed oral aperture
    • o Hypopigmentation
    • o Hyperpigmentation
    • o Digital ulcerations
  • · •Peripheral vascular system:
    • o Raynaud phenomenon (differentiate from Raynaud disease, generally affecting younger individuals and without digital ulcers)
  • o Telangiectasia
  • · •Joints, tendons, and bones:
    • o Sclerodactyly
    • o Polyarthralgia
    • o Joint stiffness
    • o Hand swelling
    • o Friction rub on tendon movement
    • o Flexion contractures
  • · •Muscle:
    • o Proximal muscle weakness
  • o Weakness
  • · •GI tract:
    • o Xerostomia
    • o Weight loss
    • o Nausea and vomiting
    • o Malabsorptive diarrhea
    • o Esophageal reflux owing to dysmotility (most common systemic sign in diffuse disease)
    • o Dysphagia
  • · •Kidney:
    • o May develop scleroderma renal crisis: Acute renal failure
    • o Hypertension
  • · •Pulmonary:
    • o Dry crackles at lung bases
    • o Dyspnea
  • · •Nervous system:
    • o Trigeminal neuropathy
    • o Peripheral neuropathy
  • · •Cardiac (progressive disease):
    • o Secondary cor pulmonale
    • o Conduction abnormalities
    • o Cardiomyopathy

Systemic Sclerosis Clinical manifestations Symptoms

  • Pain (67 percent)
  • Sleep difficulties (66 percent)
  • Skin discoloration (47 percent)
  • Fatigue (76 percent)
  • Stiff joints (74 percent)
  • Loss of strength (68 percent)
  • 1. Raynaud’s phenomenon
    • – Precipitated by cold, vibration, emotional stress.
    • – There is pallor, followed by cyanosis, followed by redness on rewarming.
    • – There is coldness and numbness of finger with pain and tingling.
  • 2. Swelling of fingers, hands, forearms, feet, legs, face
  • 3. There is redness and thickening of skin
  • 4. Skin may become thin and atrophic
  • 5. Pigment, ulcers may be found on skin
  • 6. Polyarthritis
  • 7. Muscle weakness
  • 8. Peptic ulcer
  • 9. Dysphagia
  • 10. Malabsorption syndrome
  • 11. Dyspnoea, cough
  • 12. Interstitial fibrosis
  • 13. Pulmonary hypertension
  • 14. RVF
  • 15. Pericarditis
  • 16. Heart failure
  • 17. Heart block and arrhythmias
  • 18. Renal failure
  • 19. Hypertension, malignant hypertension
  • 20. Sjogren’s syndrome-chronic autoimmune dis­ease with xerostomia (dry mouth) and xe­rophthalmia (dry eyes)
  • 21. Hypothyroidism.

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Scleroderma (systemic sclerosis [SSc

 

Systemic Sclerosis Lab investigations

  • · ESR increased
  • · Anaemia
  • · Anti-nuclear antibodies ANA detected. ,
  • Nail fold capillary microscopy
  • CBC
  • Creatinine
  • Urinalysis
  • Antinuclear antibodies (ANAs), Scl-70 in systemic disease, and anticentromere antibodies in CREST variant
  • ECG
  • Cardiac echo
  • Pulmonary function tests (PFTs)

Imaging investigations

  • Radiographic evidence of soft tissue atrophy and acro-osteolysis
  • Can see overlap syndromes such as rheumatoid arthritis
  • Barium enema:
  • Colonic diverticula
  • Megacolon
  • Chest radiograph:
  • Diffuse reticular pattern
  • Bilateral basilar pulmonary fibrosis

Systemic Sclerosis Management

  • · Blood Pressure monitoring
  • · Blood counts
  • · Urine analysis
  • · Renal function tests
  • · Pulmonary function tests
  • · Immunosuppressive drugs-Colchicine, D-penicH­lamine, Interferon alpha, chlorambucil
  • · Minocycline
  • · Etanercept
  • · Anti platlet thera py
  • · Glucocorticoids
  • · Skin care.

Causes of Raynaud’s phenomenon

  • · Systemic sclerosis
  • · Connective tissue diseases
  • · Thoracic outlet syndromes-cervical rib
  • · Shoulder hand syndrome
  • · Trauma
  • · Vibratory machine operators
  • · Cold injury
  • · Cold agglutinins.

Management of Raynaud’s phenomenon

  • · Warm clothes
  • · Avoid amphetamine, ergotamine
  • · Avoid beta blockers
  • · Prazosin may be useful
  • · Nifedipine, Diltiazem, Amlodipine may relieve
  • symptoms
  • · Sildenafil
  • · Losartan (ARB)
  • · IV Iloprost (Prostacyclin)
  • · IV Epoprostenol
  • · Pentoxiphylline
  • · Surgical cervical sympathectomy
  • · Anti biotics
  • · Cyclophosphamide
  • · Dialysis in CRF.

Management Treatment of Systemic Sclerosis —-

There is no specific therapy. General supportive therapy is indicated. A great number of drugs including corticosteroids, vasodilators, d-penicillamine, and immunosuppressive agents have been tried

  • Alveolitis: Immunosuppressants and alkylating agents (e.g.,  cyclophosphamide)
  • Angiotensin-converting enzyme inhibitors for preservation of renal blood flow and for treatment of hypertensive renal crisis
  • Antacids, proton pump inhibitors: For gastric reflux
  • Antibiotics: For secondary infections in bowel and active skin infections
  • Avoidance of  caffeine, nicotine, and sympathomimetics may ease Raynaud symptoms.
  • Consider immunosuppressives for treatment of life-threatening or potentially crippling scleroderma or interstitial pneumonitis.
  • Corticosteroids: For disabling myositis, pulmonary alveolitis, or mixed connective tissue disease (not recommended in high doses owing to increased incidence of renal failure)
  • Dipyridamole or aspirin: Antiplatelet therapy
  • Hydrophilic skin ointments: Skin therapy
  • Intestinal dysfunction: Metoclopramide
  • Nitrates and dihydropyridine calcium channel blockers for Raynaud phenomenon
  • NSAIDs: For joint or tendon symptoms
  • PDE-5 antagonists (e.g., sildenafil), prostanoids, and endothelin-1 antagonists are changing management of pulmonary hypertension.
  • Penicillamine: To reduce skin thickening and delay the rate of new visceral involvement (use is now controversial; newer therapies such as relaxin may be better)
  • Precautions/contraindications/interactions: Refer to the manufacturer’s literature for each drug.
  • Topical clindamycin, erythromycin, or silver sulfadiazine cream may prevent recurrent infectious cutaneous ulcers.

Additional Therapies

  • Physical therapy to maintain function and promote strength
  • Heat therapy to relieve joint stiffness