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A young man with a characteristic syndrome
  1. J Ray,
  2. D O'Gradaigh,
  3. R Tighe
  1. Norfolk and Norwich Hospital, Brunswick Road, Norwich, Norfolk NR1 3FR, UK

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    A 21-year-old man presented with a 12-day history of feverish symptoms with a sore throat, polyarthralgia, and left-sided pleuritic pain. On direct questioning, a recent history of nasal crusting and weight loss was elicited. His right shoulder was painful and the pain in his left hip made walking increasingly difficult. On examination, he was mildly icteric, thin, and looked unwell with a low-grade pyrexia and very tender cervical lymphadenopathy. There were petechiae and ulcerations on the palate. The left groin was also tender and a fluctuant swelling was palpable on the lateral border of the thigh 10 cm below the anterior superior iliac spine. Active and passive movement of the left hip in all directions was limited by pain, whilst a full range of movement was seen in the right shoulder.

    Investigations revealed haemoglobin 10.2 g/dl, mean corpuscular volume 90.1 fl, platelets 310 × 109/l, white blood cell count 14.9 × 109/l (neutrophils 12.56, lymphocytes 1.13, eosinophils 0.01), C-reactive protein 157 mg/l, erythrocyte sedimentation rate 67 mm, bilirubin 58 m/h μmol/l, γ-glutamyl transpeptidase 211 IU/l, alanine transaminase 114 IU/l, prothrombin time 17.3 s, XDP <10 mg/dl, cANCA weakly positive. Urine microscopy was normal, Paul Bunnel test for Epstein Barr virus was negative, and a ventilation/perfusion scan showed a low probability of pulmonary embolus. A transthoracic echo and X-ray of the left hip were normal. His chest X-ray is shown in the figure.

    Questions

    1
    How would you report this chest X-ray?
    2
    What further investigations are required?
    3
    What is the most probable diagnosis?

    Answers

    QUESTION 1

    The chest X-ray demonstrates multiple lesions. On the left side is a cavitating lesion in the region of the heart shadow near the costovertebral angle.

    QUESTION 2

    Blood cultures and aspiration of the left hip using ultrasound guidance should be performed.

    QUESTION 3

    The most probable diagnosis is Lemierre's syndrome.1 2

    Discussion

    The differential of multiple cavitating lung lesions is broad and includes infections, inflammatory conditions, and metastic disease. An infection with embolisation would be in keeping with the history and examination findings. A range of infective conditions would need consideration, for example, infective endocarditis, tuberculosis, or opportunistic infection in an immunocompromised individual.

    Ultrasound of the left hip joint demonstrated an effusion which extended laterally and this was aspirated. Subsequent culture isolatedFusobacterium necrophorum. This Gram-negative anaerobe can cause a rare septicaemia which is also known as Lemierre's syndrome. The infection was successfully treated with a 2-week course of intravenous antibiotics (1.2 g benzylpenicillin qid and 500 mg metronidazole tid, in accordance with proven microbiology sensitivities), followed by 2 weeks of oral antibiotics.

    A differential diagnosis of Wegener's granulomatosis3 was considered in view of the arthralgia, upper respiratory tract involvement and systemic upset. On immunofluorescence staining for ANCA, our patient had a weakly positive reaction with a cytoplasmic pattern. However, further clarification is important, and in this case, ELISA identified reactivity to bacterial permeability inhibitor, suggesting the antibodies were secondary to infection. A differential antigen, PR3, is strongly associated with Wegener's granulomatosis. Furthermore, the liver enzyme abnormalities and very short prodrome militate against this diagnosis, and while arthralgia can be marked, joint effusions are uncommon. Histological confirmation is essential before considering immunosuppression, and in this case, the microbiology from the hip effusion suggested the correct diagnosis, and obviating the need for further investigation. The consequences of undertaking immunosuppression in this patient, assuming a diagnosis of Wegener's granulomatosis, could have been very serious.

    Lemierre's syndrome is caused by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein, which is frequently complicated by metastatic infection. Painful cervical lymphadenopathy is usual. Systemic infection, characteristically involves the lung and joints, as occurred with our patient. Other sites of infection include liver, spleen, kidney and meninges. The early empirical use of antibiotics in the treatment of pharyngitis makes this a rarely seen condition. Lemierre's description of the clinical findings constituted, “a syndrome so characteristic that mistake is almost impossible.”4

    Final diagnosis

    Lemierre's syndrome (metastatic Fusobacterium necrophorum infection).

    References

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