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Scleroderma

Scleroderma, an autoimmune disease, primarily targets the skin and internal organs. With its name derived from 'hard skin', scleroderma initially affects the skin before spreading to organs like the kidneys, lungs, heart, and gastrointestinal tract. It manifests in two forms: diffuse and limited, varying in the degree of skin involvement.

 

 

Scleroderma's cause remains elusive, though genetic predisposition may heighten susceptibility. Environmental factors like industrial solvents could contribute. 


 

Skin: Thickening and darkening. Blood vessels: Raynaud's phenomenon, ulceration risk. 

Gastrointestinal tract: Difficulty swallowing, acid reflux, diarrhea, constipation. 

Lungs: Fibrosis leading to coughing, breathlessness, potential fatality. 

Heart: Pulmonary artery hypertension, left ventricular failure. 

Kidney: Rarely, renal failure. Early intervention crucial."


 

Scleroderma diagnosis relies on thorough clinical examination by a skilled clinician. Subsequent tests assess organ involvement, such as pulmonary function tests (PFT), CT scans, and cardiac scans, ensuring comprehensive evaluation and tailored management.

Testing for scleroderma includes ANA (antinuclear antibody), Scl-70, and anticentromere antibody tests. Additional tests may involve skin biopsies and imaging studies. Early diagnosis and monitoring by a rheumatologist are crucial for effective management. Consult a rheumatology specialist for comprehensive care and personalized treatment options.

Early detection of scleroderma allows for prompt treatment, slowing disease progression and improving quality of life. It enables better management of symptoms, reduces complications, and enhances the effectiveness of interventions. Regular monitoring by a rheumatologist is essential for optimal outcomes in scleroderma care.

Scleroderma patients benefit from tailored drug regimens addressing individual organ involvement:

Raynaud’s phenomenon:

  • Avoid smoking and cold exposure.
  • Dress warmly and use prescribed circulation-improving drugs (e.g., calcium channel blockers).

Skin issues:

  • Moisturize frequently, use warm water, gentle soaps, and avoid harsh chemicals.
  • Treat ulcers with nitroglycerine paste or antibiotic cream.

Gastrointestinal problems:

  • Opt for small, early dinners and remain upright after eating.
  • Avoid citrus fruits at night and consider proton pump inhibitors for heartburn.

Lung disease:

  • Immunosuppressants like cyclophosphamide and Mycophenolate Mofetil, plus low-dose steroids, treat pulmonary fibrosis.

Heart disease:

  • Vasodilators like prostacyclin, Bosentan, and sildenafil alleviate right heart pressure."


 

If scleroderma is diagnosed and treated early it can be controlled very well. This is because in scleroderma most of the damage occurs early in the disease and if not controlled early the damage which happens is irreversible.

Scleroderma Scoring Tests: Diagnostic assessments aid in quantifying disease severity and organ involvement:

  1. Modified Rodnan Skin Score: Measures skin thickening at various body sites.
  2. Pulmonary Function Tests (PFTs): Assess lung function and detect fibrosis.
  3. Echocardiogram: Evaluates heart function and detects pulmonary hypertension.
  4. Esophageal Manometry: Determines esophageal involvement.
  5. Renal Function Tests: Assess kidney function and detect renal involvement.
  6. Autoantibody Testing: Identifies specific antibodies associated with scleroderma subtypes.
  7. Nailfold Capillaroscopy: Examines microvascular changes in fingers. These tests enable accurate diagnosis, monitoring disease progression, and guiding treatment decisions."