In our #AMreport today at @, we reviewed a case with a constellation of symptoms including: chronic diarrhea, refractory acid reflux, Raynaud’s phenomenon and sclerodactyly. This was a case of the “CREST- Calcinosis, Esophageal dysmotility, Sclerodactyly, Telangiectasias” syndrome occurring as part of the limited cutaneous systemic scleroderma (lcSS). Gastrointestinal symptoms are sometimes overlooked in cases of systemic sclerosis. Apart from the esophagus, the small bowel may have impaired motility in these patients which could lead to variations between bloating, constipation and diarrhea. Furthermore bacterial overgrowth can also develop, resulting in intermittent episodic diarrhea as is in this case.
Here is a great review of systemic scleroderma: http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/systemic-sclerosis/
- Gastrointestinal symptoms may predate skin manifestations and apart from acid reflux, other gastrointestinal manifestations such as diarrhea or bloating may herald systemic sclerosis.
- Raynaud phenomenon occurs in the majority of patients with systemic sclerosis and usually may predate eventual progression to usually limited cutaneous systemic sclerosis (lcSS).
- Antinuclear antibodies (ANA) is positive in more than 95% of patients with systemic sclerosis.
- Glucocorticoid therapy carries a significant risk for scleroderma renal crisis (SRC) and use should be avoided or used sparingly in patients with lcSS.
- Pulmonary involvement can occur in both major types of systemic sclerosis; pulmonary arterial hypertension with lcSS and alveolitis or ILD is usually associated with diffuses cutaneous systemic sclerosis (dcSS).