The patient with Wegener's granulomatosis and an intrasplenic mass of unknown origin

(Section Editor: K. Kühn)

Syrus Hafezi-Rachti, Regine Rieß, Sven Weidner, Andrea Wonka and Harald D. Rupprecht

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 1Go). 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, 30–40 leukocytes and granular casts and dysmorphic erythrocytes.



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Fig. 1. The chest X-ray on admission shows pulmonary infiltrates, a right-sided pleural effusion and large pulmonary nodules and cavities.

 
No abnormalities of blood clotting could be detected. Serological tests for antinuclear antibodies, antibodies against glomerular basement membrane and antibodies against phospholipids were negative and complement was within the normal range. The test for antineutrophil antibodies by indirect immunfluorescence was positive with a cytoplasmatic pattern and enzyme-linked immunosorbent assay (ELISA) showed antibodies specific to proteinase 3. Abdominal ultrasonography showed enlarged kidneys and an intrasplenic inhomogeneous area of unknown origin (4 cm in diameter). In the following days the appearance of the mass changed; it increased in size and became less echogenic. A biopsy was taken from the left kidney. Five out of 6 glomeruli showed an active necrotizing crescentic glomerulonephritis (Figure 2Go). In addition a biopsy of bronchial mucosa was conducted, which disclosed heavy ulcerations and scarring and in outlines granulomas. Treatment was started with 500 mg methylprednisolone per day for three consecutive days plus cyclophosphamide in a dose of 2 mg/kg body weight per day orally. After 3 days prednisolone was continued at 125 mg/d. Serum creatinine continued to rise to a maximum of 6 mg/dl at day three after initiation of therapy. Renal and pulmonary function recovered, haemodialysis was not required and serum creatinine was 1.3 mg/dl at discharge!



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Fig. 2. Focal segmental necrotizing glomerulonephritis: a glomerulus is shown with segmental fibrin exsudation and necrosis and beginning crescent formation (PAS stain, magnification: x400).

 
In the differential diagnosis of the hypoechogenic intrasplenic mass we considered abscess, secondary cyst after infarction and haematoma. A duplex ultrasound examination of splenic blood vessels revealed a stop of blood flow in the A. lienalis and no parenchymal perfusion. CT-scan with contrast medium confirmed the diagnosis of complete splenic infarction and revealed a remnant capsular blood supply via extra-splenic vessels (Figure 3Go). Because there were no signs of an imminent rupture we decided against surgical intervention. The patient received vaccination against pneumococci. Today, two and a half years after the initial presentation the patient is in stable remission. A recently performed CT-scan shows a considerable volume reduction and scarring of the spleen (Figure 4Go).



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Fig. 3. CT-scan with contrast medium demonstrates no enhancement of the spleen except for the capsule, suggesting a nearly complete splenic infarction with a remnant capsular blood supply.

 


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Fig. 4. Two and a half years after the initial presentation the CT-scan shows a considerable volume reduction and scarring of the spleen.

 

Discussion

There are few reports on splenic involvement on postmortem in patients with Wegener's granulomatosis. This manifestation appears to be very rare. Since—surprisingly—it 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 Back

Reference

  1. Kettriz R, Anders S, Kettriz U, Schneider W, Göbel U, Luft F. Spontanous splenic hemorrhage in a patient with Wegener's granulomatosis. Am J Kidney Dis1998: 31; 860–862[ISI][Medline]