- Pierce Geoghegan, senior house officer,
- Donal J Sexton, specialist registrar in nephrology,
- Louise Giblin, consultant nephrologist,
- Anthony O’Regan, consultant respiratory physician
- 1Galway University Hospital, County Galway, Republic of Ireland
- Correspondance to: P Geoghegan
A 45 year old man with a history of ulcerative colitis presented to his general practitioner because of Raynaud’s phenomenon and itchy tight skin that had affected his hands and elbows bilaterally over the preceding few months. He was referred to a rheumatologist, who performed a series of immunological tests. The results included a positive antinuclear antibody staining pattern on immunoflourescence, with anti-RNA polymerase III antibodies identified on further testing. Tests for anti-centromere and anti-topoisomerase I (anti-Scl 70) antibodies were negative. The rheumatologist diagnosed a systemic connective tissue disorder.
When the skin symptoms worsened over the next few weeks the patient started to use over the counter ibuprofen to ease the pain associated with his tight skin. He subsequently presented to the emergency department with fatigue. His blood pressure was found to be 210/120 mm Hg. In addition to the skin abnormalities, bibasal fine inspiratory crepitations were detected on physical examination. Routine laboratory testing showed haemoglobin 75 g/L (reference range 130-180), creatinine 475 µmol/L (80 µmol/L one month previously (80-110), and lactate dehydrogenase 357 U/L (70-250). Electrocardiography showed T wave inversion in the lateral leads. He was excreting 1.15 g of protein in his urine per 24 hours and haematuria was detected on dipstick urinalysis.
1 What is the diagnosis?
2 What further test might help to confirm the diagnosis?
3 What factors predispose to this condition?
4 How is the condition treated?
5 What is the natural course of the condition?
What is the diagnosis?
Scleroderma renal crisis, which results in a hypertensive emergency.1
Our patient had diffuse cutaneous scleroderma. Figures 1⇓ and 2⇓ show taut shiny skin on his forearm and hands, with areas of hypopigmentation on the extensor surfaces. The skin was so tight that he developed contractures at the elbows (fig 1 shows the position of maximal …