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.

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