Department of Internal Medicine and Nephrology, Friedrich-Alexander-University, Erlangen-Nürnberg, Germany
Keywords: duplex ultrasound; splenic infarction; systemic vasculitis; Wegener's granulomatosis
Introduction
Wegener's granulomatosis is a systemic vasculitis characterized by granulomatous inflammation mainly involving the upper and lower respiratory tract and the kidneys. A number of organs may be involved apart from the respiratory tract and the kidneys and it is of interest that in the first description of the syndrome in 1936 Wegener referred to splenic involvement. We had the opportunity to observe a patient with documented Wegener's granulomatosis who developed a puzzling lesion of the spleen, the nature of which could be diagnosed non-invasively and which responded to treatment of vasculitis.
Case
A 30-year-old male (body weight 110 kg, height 185 cm) was admitted to the hospital because of increasing swelling and tenderness of the left leg. Five weeks prior to admission he consulted an ENT-doctor for persistent rhinitis and otitis. In addition he complained about muscle and joint pain. The diagnosis of a deep venous thrombosis of the left leg was made by ultrasound examination. In order to localize the cranial extension of the thrombosis an additional CT-scan was performed, which revealed a thrombosis reaching up to the left iliac vein. Remarkably the only conceivable risk factor on admission for deep venous thrombosis was obesity. The patient was immobilized and received standard treatment with heparin i.v. Surgery was not performed because of a deteriorating condition of the patient with increasing dyspnea and tachypnea. A chest X-ray was performed, that revealed several pulmonary nodules (Figure 1). Rapidly rising creatinine led to referral to the renal unit. Vital signs: blood pressure 140/80, temperature 38.9°C, heart rate 120 bpm, respiratory rate 40/min. Physical examination revealed rales over the basal parts of the lung. Heart and abdominal examination were unremarkable, the skin showed no abnormalities. Lymphadenopathy, uveitis or skleritis were not present. Haemoglobin was 9.6 g/dl, white blood cell count 22 100/mm3, platelets 648 000/mm3. Serum creatinine was 5.0 mg/dl. The urinalysis showed 50 red cells, 3040 leukocytes and granular casts and dysmorphic erythrocytes.
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Discussion
There are few reports on splenic involvement on postmortem in patients with Wegener's granulomatosis. This manifestation appears to be very rare. Sincesurprisinglyit does not usually cause major symptoms, it may fequently go unrecognized. Our patient had no abdominal pain at all. In the observation of Kettriz [1] even massive enlargement of the spleen did not provoke any abdominal symptoms. These observations parallel what is observed in delayed post-traumatic splenic rupture of the spleen which is also usually completely painless.
The above case illustrates that examination with ultrasound in combination with duplex sonography of splenic blood supply permits non-invasive diagnosis of splenic infarction. The diagnosis can be confirmed by MRI an CT scan which permits to assess the extent of splenic infarction. The evolution of our case illustrates that, unless there are signs of imminent rupture or bleeding, a conservative approach is justified. In the long term, these patients may be more susceptible to pneumococcal infection because of the functionally asplenic condition. This possibility adds one more argument to diagnose this rare condition in vivo.
Teaching point
In the patient with systemic vasculitis and an asymptomatic splenic mass: consider splenic haemorrhage or splenic infarction.
A conservative approach is justified in the absence of rupture or internal haemorrhage.
Notes
Supported by an educational grant from
Correspondence and offprint requests to: Dr Syrus Hafezi-Rachti, Medizinische Klinik 4/Schwerpunkt Nephrologie, Universität Erlangen-Nürnberg, Breslauerstr. 201, D-90471 Nürnberg, Germany. E-mail: SyrusHafezirachti{at}netscape. net
Reference