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An unusual presentation of a common disease
  1. M von Lilienfeld-Toal1,
  2. S Merkelbach-Bruse2,
  3. F L Dumoulin1
  1. 1Department of Medicine I, University of Bonn, D-53127 Bonn, Germany
  2. 2Institute of Pathology, University of Bonn, D-53127 Bonn, Germany
  1. Correspondence to:
    Dr F L Dumoulin;
    dumoulinuni-bonn.de

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A variety of rheumatic syndromes have been associated with neoplasia. We report a case of occult metastatic breast cancer which presented with symptoms and signs of adult onset Still’s disease (AOSD).

CASE REPORT

In July 2002, a 52 year old Caucasian woman presented with a 4 week history of high, spiking fevers (up to 40°C) that were accompanied by a transient macular rash, myalgia, and arthralgia affecting most joints. Initially, there had been a sore throat and painful lymphadenopathy in the left supraclavicular fossa. A lymph node was removed for histopathological examination and the lymphadenopathy resolved spontaneously. The past medical history was unremarkable other than a family history of breast cancer which had affected two second degree relatives. The physical and gynaecological examinations were unremarkable. There was no peripheral lymphadenopathy. Blood tests showed a normochromic, normocytic anaemia (haemoglobin 100 g/l), a leucocytosis (12.3 g/l; 85% neutrophils), raised C reactive protein (CRP; 91 mg/l), and erythrocyte sedimentation rate (ESR; 94/120), an exceptionally high serum ferritin level (21 762 µg/l), abnormal liver function tests (γ-glutamyltransferase 105 U/l, aspartate aminotransferase 32 U/l), and lactate dehydrogenase (532 U/l). Testing for bacterial, viral, or fungal infection and autoantibody screens were consistently negative. Additional investigation (ultrasound, computed tomography scan, bone marrow aspirate) showed only splenomegaly and increased numbers of normal sized lymph nodes in the left axilla.

At that stage the clinical diagnosis of AOSD was made based on the presence of all the diagnostic criteria (fever, arthralgia, rash, leucocytosis as well as sore throat, lymphadenopathy, splenomegaly, liver dysfunction, and absence of rheumatoid factor and antinuclear antibody) proposed by Yamaguchi et al.1

Treatment was started with aspirin (3 g/day) and this resulted in a gradual resolution of fever. To our surprise, the histopathological examination of the lymph node removed initially disclosed a small breast cancer metastasis (fig 1). Further investigation including mammography, ultrasound, and magnetic resonance imaging of both breasts showed a 15 mm suspicious mass in the left breast. A lumpectomy and lymph node dissection was carried out. The lesion was diagnosed as a ductal adenocarcinoma and all dissected lymph nodes (n = 28) were normal. After tumour resection, fever, and all other symptoms of AOSD, including serological markers of inflammation such as ESR, CRP, and ferritin, returned to normal without further symptomatic treatment. The patient is currently receiving adjuvant chemotherapy.

Figure 1

 Histopathological finding of the axillary lymph node biopsy (magnification ×250). Note the metastatic infiltration (haematoxylin and eosin stain (A)) and the dense lymphocytic infiltrate consisting of CD8 positive T lymphocytes (immunoperoxidase stain (B)). Polymerase chain reaction analysis showed a polyclonal pattern of V gamma rearrangement (data not shown).

DISCUSSION

AOSD is an inflammatory disorder of unknown aetiology characterised by fever, arthritis, and skin rashes.1–4

Symptoms of AOSD are known to occur in patients with haematological malignancies. However, they have also rarely been described as the first symptoms in patients with breast cancer,5–7 although fever as a paraneoplastic symptom is generally uncommon in such patients.8 Interestingly, published reports describe patients with similar features: all had metastatic cancer affecting lymph nodes with a relatively small tumour mass and in all patients the AOSD-like symptoms resolved completely after successful tumour treatment. Thus metastatic breast cancer, as well as lymphoma and leukaemia may mimic AOSD. The unusual presentation (AOSD) of an all too common disease (metastatic breast cancer) should remind us that symptoms of rheumatic diseases can sometimes be clues to occult malignancies9 and emphasises the importance of a thorough search for underlying occult neoplastic disease.

REFERENCES

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Footnotes

  • Conflict of interest: No conflict of interest has been declared by the authors. The patient’s written consent was obtained according to the Declaration of Helsinki (BMJ 1996;31:1448–9).

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