Scleroderma: Complications

The following are the most important diseases or complications that may be contributed to by chronic cutaneous circumscritic scleroderma:

Congenital malformations, deformities, and chromosomal abnormalities (Q00-Q99).

  • Joint contractures (stiffening of joints).

The following are the major diseases or complications that may be co-morbid with systemic scleroderma:

Respiratory system (J00-J99)

  • Alveolitis (inflammation of the air sacs).
  • Exertional dyspnea (shortness of breath under exertion).
  • Pulmonary fibrosis (connective tissue remodeling of the lungs leading to functional impairment).
  • Pneumonia (pneumonia)

Endocrine, nutritional and metabolic diseases (E00-E90).

  • Malnutrition

Skin and subcutaneous tissue (L00-L99)

  • Ulcers (ulcers) on fingers and toes.

Cardiovascular system (I00-I99)

  • Arrhythmias (cardiac arrhythmias).
  • Heart failure (cardiac insufficiency)
  • Hypertension (high blood pressure)
  • Pericardial effusion (pericardial effusion) – as an expression of inflammatory cardiac involvement or as a consequence of PAH.
  • Pulmonary arterial hypertension (PAH; pressure increase in the pulmonary arterial system).

Mouth, esophagus (food pipe), stomach, and intestines (K00-K67; K90-K93).

Musculoskeletal system and connective tissue (M00-M99).

  • Myositides (muscle inflammations).

Symptoms and abnormal clinical and laboratory findings not elsewhere classified (R00-R99)

  • Dysphagia (dysphagia).

Genitourinary system (kidneys, urinary tract – reproductive organs) (N00-N99).

  • Renal insufficiency (renal weakness/renal failure; in systemic sclerosis); occurs within the first 4 years of disease in about 20% of patients → “scleroderma renal crisis” (SRC); usually oliguric renal failure; risk factors for SRC include:
    • Extensive, rapidly progressive skin involvement.
    • joint contractures (joint stiffness)
    • Anti-RNA polymerase III antibodies
  • Renal crisis: accelerated increase in hypertension (high blood pressure) with values > 150/85 mmHg (at least 2 measurements over 24 h or diastolic blood pressure > 120 mmHg) + decrease in estimated glomerular filtration rate (GFR; filtering capacity of the kidney) by > 10% or decrease in measured GFR < 90 ml/min (approximately 5% of SSc patients).