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Use of prostacyclin (iloprost) in digital vasculitis secondary to meningococcaemia
  1. K Siddiqui1,
  2. A R R Razak1,
  3. B Kneafsey2,
  4. N Delanty3
  1. 1Department of Neurology, Beaumont Hospital and Royal College of Surgeons in Ireland, Beaumont Road, Dublin 9, Ireland
  2. 2Department of Plastic Surgery, Beaumont Hospital, Ireland
  3. 3Department of Neurology, Beaumont Hospital and Royal College of Surgeons in Ireland, Beaumont Road, Dublin 9, Ireland
  1. Correspondence to:
 Dr Norman Delanty;
 normandelantyeircom.net

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Extrameningeal complications of meningococcal septicaemia occur in about 11–19% of cases, and include myocarditis, acute renal failure, arthritis, pneumonia, skin gangrene, conjunctivitis, endocarditis, pericarditis, endophthalmitis, urethritis, Waterhouse-Friderichsen syndrome, vasculitis, and digital ischaemia.1

We describe the use of a prostacyclin analogue in the treatment of cutaneous digital ischaemia in a patient with meningococcal meningoencephalitis and meningococcaemia.

Case report

A 16 year old female developed progressive headache, photophobia and increasing neck stiffness over 3 days, with nausea and vomiting, and development of non-blanching purpuric rash over her trunk and right thigh. She became increasingly obtunded with depressed level of consciousness, resulting in cardiorespiratory embarrassment.

She was intubated, mechanically ventilated, and treated with volume expanders. Her initial investigations revealed leucocytosis, thrombocytopenia, anaemia, and coagulopathy. Computed tomography (CT) of the brain showed diffuse swelling of both cerebral hemispheres consistent with cerebral oedema. Meningococcal DNA polymerase chain reaction (PCR) was positive for group C serotype. She was treated with high doses of cefotaxime, benzylpenicillin, and steroids from the day of admission. Her condition improved after 48 hours of intensive care, and she was extubated. After 5 days, she developed pain and swelling in the thumb and middle finger of her left hand; within 24 hours these symptoms had worsened with marked swelling and pregangrenous changes of the acral regions of the involved thumb and finger, and there was severe pain and tenderness (fig 1A, B).

Figure 1

Pregangrenous changes (A, B) of thumb and middle digit of left hand, which resolved (C, D) following treatment with prostacyclin.

A diagnosis of imminent digital gangrene due to digital vasculitis and/or septic embolisation secondary to endarteritis was made. In an empirical effort to improve digital perfusion high dose steroids were recommenced and treatment with intravenous heparin, aspirin and clopidogrel was initiated; little improvement in the digital ischaemia occurred. In an attempt to save the digit she was then started on a prostacyclin (iloprost) infusion at a titrating dose of 0.5 ng/kg body weight/min with marked improvement in pain and coloration after 5 days of treatment. Four weeks following her discharge from hospital her fingers were nearly back to normal and she was pain free (fig 1C, D).

Comment

The pathophysiology of digital ischaemia in meningococcaemia is complex, and involves injury to the endothelium triggering the coagulation cascade, inhibition of the thrombomodulin protein C system, and lodgement of small emboli in digital capillaries. In this report we describe the use of a prostacyclin analogue, iloprost, in digital pregangrene due to digital ischaemia occurring as a complication of meningococcal septicaemia. Prostacyclin is a potent endogenous vasodilator that affects both the systemic and pulmonary circulations; it also inhibits platelet adhesion and aggregation and prevents smooth muscle proliferation. Iloprost, a stable synthetic prostacyclin analogue, has been used with varying success in the treatment of pulmonary hypertension, peripheral arterial occlusive disease,2,3,5 thromboangiitis obliterans,6 and digital vasculitis secondary to progressive systemic sclerosis,2 Sjögren’s syndrome and systemic lupus erythematosus.3,5,6 In these situations, iloprost has been shown to improve perfusion, healing digital ulceration and reducing pain secondary to ischaemia in digital vasculitis, when given in a dose of 0.5–2.0 ng/kg body weight/min (according to individual tolerability) with incremental increases in dose every 30 min. Side effects include headache, nausea, vomiting, and hypotension. Blood pressure and heart rate must be measured at the start of infusion and with each increase in dose.

The use of iloprost in digital ischaemia due to meningococcal septicaemia has not been reported previously. This report suggests that iloprost may be useful in preserving tissue integrity in the cutaneous manifestations of meningococcal septicaemia, and perhaps obviate the need for amputation in some patients. Although our report of a single case does not prove efficacy beyond doubt, the biological and pharmacological rationale behind the use of a prostacyclin analogue in this situation, and the apparent response to therapy in our patient, strongly support a direct therapeutic benefit. We believe that this is of sufficient importance to warrant therapeutic trials in patients with this potentially devastating condition.

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