Refractory adult dermatomyositis with pneumatosis cystoides intestinalis treated with infliximab

A. Selva-O'Callaghan, X. Martínez-Costa1, R. Solans-Laque, M. Mauri1, J. A. Capdevila1 and M. Vilardell-Tarrés

Department of Internal Medicine, Vall d’Hebron General Hospital and 1 Consorci Sanitari Del Maresme, Mataro Hospital, Barcelona, Spain

Correspondence to: A. Selva-O’Callaghan, C/Siracusa No. 12 bis A, Barcelona, 08012, Spain. E-mail: aselva{at}hg.vhebron.es

SIR, Idiopathic inflammatory myopathies are a group of acquired conditions characterized by inflammation of skeletal muscles [1, 2]. Although corticostercoids remain the mainstay of treatment, many patients require additional immunosuppressive agents to achieve complete remission or to control the disease [3]. Here we report a patient with refractory adult dermatomyositis who presented with tetraparesis and subsequently developed pneumoperitoneum due to pneumatosis cystoides intestinalis. Treatment with infliximab effectively achieved complete remission of the weakness and pneumoperitoneum.

A 58-yr-old woman presented in March 2002 with progressive, proximal, symmetrical muscle weakness of the limb girdles and the neck. The patient also complained of dysphagia, arthralgia and Raynaud's phenomenon. Gottron papules were observed over the skin of the hands and the elbows, and periorbital oedema with heliotrope rash of the eyelids was also noted. Investigation at this stage demonstrated high creatine kinase values (>2000 UI/l, normal value <195 UI/l). Antinuclear antibodies were positive (1/1280) and anti-Mi-2 antibodies were detected by immunoprecipitation analysis. Myopathic changes were found on electromyography, and muscle biopsy of the deltoids showed characteristic findings of inflammatory myositis. Screening for cancer including upper and lower abdominal ultrasound and mammography, and the chest-X-ray was normal. Treatment with corticosteroids (1 mg/kg/day) and weekly oral methotrexate (7.5 mg) was instituted with mild improvement of muscle weakness, but during the following month the clinical situation worsened with the development of tetraparesis and severe dysphagia, necessitating enteral nutrition through a nasogastric tube. Aggressive therapy with boluses of intravenous methylprednisolone (1 g/day, 3 days), monthly intravenous immunoglobulins (1 g/kg/day, 2 days), and a series of six monthly cyclophosphamide boluses (0.7 g/m2) were administered in addition to weekly oral methotrexate and cyclosporin, without response. She was transferred to our centre for therapeutic evaluation in November 2002, when she presented with a fever of 39°C. Blood and urine cultures were done and she received broad-spectrum antibiotic treatment with piperacillin and tazobactam. CT scanning of the chest was normal while abdominal CT scanning showed intramural gas in the right colon and pneumoperitoneum. Surgical intervention was not deemed appropriate without signs of peritonitis and a conservative approach was followed. Blood and urine cultures were negative, and after 3 weeks of antibiotic treatment the possibility of pneumatosis cystoides intestinalis associated with dermatomyositis was considered. In view of these developments and after discussion with the patient and her family, treatment was initiated with TNF-{alpha} inhibitor (infusions of infliximab 5 mg/kg), given at 0, 2 and 6 weeks and then bimonthly in addition to prednisone (15 mg/day), cyclosporin (5 mg/kg/day) and weekly oral methotrexate (7.5 mg). Physical therapy was also recommended. The patient had a very good response and 6 months later was able to walk and swallow, and enteral feeding was stopped. A follow-up abdominal CT scan was normal, with no evidence of intramural or free abdominal air. One year later the patient is doing well, with normal creatine kinase and EMG.

Treatment of inflammatory myopathies remains a challenge. Corticosteroids are the agents of choice for the initial treatment; nevertheless many patients require additional immunosuppressive drugs. Refractory myositis is defined as persistent activity of the disease despite administration of full doses of corticosteroid and other additional immunosuppressive agents. Only one randomized crossover study exists, and the results suggest that administration of weekly oral methotrexate and azathioprine may benefit patients with resistant myositis [4]. In the case presented here there was no response to different immunosuppressive treatments, and anti-TNF-{alpha} infliximab was tried. In patients with dermatomyositis elevation of soluble tumour necrosis factor has been observed, and this suggests a role for biological therapies directed against TNF-{alpha} [5] Although not all patients seem to respond well to the therapy [6], in the case presented here the response to the administration of infliximab was excellent, allowing the patient to walk, swallow and become fully self-caring.

Pneumatosis cystoides intestinalis has been described rarely in adult patients with dermatomyositis [7], and several mechanisms have been proposed for its development, including breaks in the intestinal mucosa, infection, and ischaemia due to vasculitis. Pneumatosis cystoides intestinalis is often asymptomatic and may occur with pneumoperitoneum, which is sterile and must be distinguished from a perforated viscus [8]. The clinical course and the abdominal CT scan in our case uphold the diagnosis of pneumatosis cystoides intestinalis. This rare manifestation of dermatomyositis also improved after therapy with TNF inhibitor. Treatment with infliximab, a TNF inhibitor, could be an alternative in patients with refractory myositis.

The authors have declared no conflicts of interest.

References

  1. Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344–7.[ISI][Medline]
  2. Dalakas MC. Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med 1991;325:1487–98.[ISI][Medline]
  3. Choy EHS, Isenberg DA. Treatment of dermatomyositis and polymyositis. Rheumatology 2002;41:7–13.[Free Full Text]
  4. Villalba L, Hicks JE, Adams EM et al. Treatment of refractory myositis. A randomized crossover study of two new cytotoxic regimens. Arthritis Rheum 1998;41:392–9.[CrossRef][ISI][Medline]
  5. Shimizu T, Tomita Y, Son K, Nishinarita S, Sawada S, Horie T. Elevation of serum soluble tumour necrosis factor receptors in polymyositis and dermatomyositis. Clin Rheumatol 2000;19:352–9.[CrossRef][ISI][Medline]
  6. Roddy E, Courtney PA, Morris A. Non-Hodgkin's lymphoma in a patient with refractory dermatomyositis which had been treated with infliximab. Rheumatology 2002;41:1194–214.[Free Full Text]
  7. Pasquier E, Wattiaux M-J, Peigney N. First case of pneumatosis cystoides intestinalis in adult dermatomyositis. J Rheumatol 1993;20:499–503.[ISI][Medline]
  8. Heng Y, Schuffer MD, Haggitt RC, Rohrmann CA. Pneumatosis intestinalis: a review. Am J Gastroenterol 1995;90:1747–58.[ISI][Medline]
Accepted 28 May 2004