Clinical exam skills: A handy sign

A 32 year old woman presented with an eight month history of being "out of sorts." Five months before she had had an elective ear, nose, and throat procedure in a district hospital. In the months after her admission she had regularly visited her general practitioner with a number of symptoms, which included tiredness, joint pains, facial pains, and a recurrent stuffy nose. At the time of admission to hospital in addition to the above she complained of sore red eyes.

On examination, there was evidence of bilateral conjunctival injection. She had tender small joints in the hands, knees, ankles, and elbows. Nail fold infarcts were also noted (fig 1).

Questions

1. Describe the findings in the hands and give differential diagnoses for their cause.

   Computed tomography imaging taken before the previous ear, nose, and throat surgery was retrieved from the district hospital (fig 2).

2. Outline any abnormalities seen in this image.

   After a consultation with an ophthalmologist, anterior uveitis was confirmed. Her initial blood results (reference range) were 80 (0-19) IU/ml antineutrophil cytoplasmic antibodies and 7.4 (0-2) IU/ml proteinase 3.

3. What diagnosis do these laboratory results suggest?

   Urinalysis also found "protein ++; blood +++."

   Two days following admission the changes were noted in her blood indices (table).

4. What complication has occurred?
5. utline the other potential features of this condition?

*24 hour urinary protein 1.2 g. ÝNormal values: urea 2.5-6.7 mmol/l, creatinine 70-150 µmol/l, 24 hour urinary protein <300m.

Answers

1. You can see a nail fold infarct on the index finger of the patient's hand. She had a number of these on both hands which occurred recently. Nail fold infarcts occur in some diseases because small vessels supplying the nail bed are compromised. The main causes include bacterial endocarditis and vasculitis.

   Vasculitis is most commonly associated with rheumatological and connective tissue diseases, such as scleroderma and rheumatoid arthritis. Other conditions in which nail fold infarcts are seen include systemic lupus erythematosus, Wegener's granulomatosis, dermatomyositis, and polymyositis. You should examine the heart and check for pyrexia to exclude endocarditis.

2. This is an axial image of the sinuses. There is evidence of bilateral soft tissue opacification within the maxillary antra. There is no evidence of bony destruction. The appearances suggest sinusitis. The opacification most likely represents mucopurulent secretions within the antra. In some cases of Wegener's granulomatosis, you may see bony destruction.
3. She has a raised concentration of cANCA (antineutrophil cytoplasmic antibodies). Also the proteinase 3 component is raised. These are antibodies which are directed against components of the cytoplasm of neutrophils and are detected by indirect immunofluorescent staining. These antibodies are thought to mediate immune injury in tissue.¹

   Two ANCAs are measured in clinical practice--cANCA (classical or cytoplasmic) and pANCA (perinuclear). The measurements of proteinase 3 (PR3) and myeloperoxidase (MPO) further distinguishes specificity. cANCA-PR3 has the best correlation with disease being found in over 90% of patients with Wegener's granulomatosis. pANCA-MPO is strongly associated (about 60%) with the condition Churg-Strauss.

   cANCA titres, unlike most other autoantibodies (with the exception of double-stranded DNA in systemic lupus erythematosus) reflect the level of disease activity. Therefore, serum measurements can be used to monitor disease and its response to treatment.

   
   Fig 1. Nail fold infarcts
   
   
   
   Axial computed tomography of sinuses
   
   
4. The most likely cause for the positive urinalysis and alterations in renal indices is glomerulonephritis. This person's normal renal indices deteriorated two days after admission. Their improvement by day four relates to the administration of intravenous hydrocortisone. The initial treatment regimen for this complication of Wegener's granulomatosis is high dose steroids and cyclophosphamide.

   In Wegener's granulomatosis renal involvement is classically focal segmental necrotising glomerulonephritis. Proteinuria and haematuria are features, as with this woman. Proteinuria was confirmed on 24 hour urinary protein collection as being substantially raised at 1.2 grams. Renal biopsy may be required to confirm the exact pathology, but often does not give a definitive diagnosis so its indication should be assessed on a case by case basis.

   Prognosis is bad without treatment. A combination of steroids and cyclophosphamide is commonly used for remission. Withdrawal of this treatment often results in relapse, which is a common occurrence.

5. Wegener's granulomatosis is an idiopathic vasculitis. It is a rare multisystem disease with its effects due to necrotising granulomatous inflammation. It involves the small and medium sized blood vessels--both arterial and venous. Classic disease involves the upper respiratory tract (sinusitis, as in this woman), lower respiratory tract and kidneys (seen in this woman). However, all body systems may be implicated.

Although clinical features and ANCA results are highly suggestive, definitive diagnosis may be made by either nasal or renal biopsy, however, a normal biopsy does not exclude disease. Disease may be patchy, and so granulomas may be missed in the chosen specimen.

Respiratory disease is associated with shortness of breath, cough, and haemoptysis. Chest radiography typically reveals one or several lesions (infiltrates or nodules) which are cavitating in nature. This may be confused with a cavitating pneumonia or a cavitating bronchial carcinoma in older patients. Effects on the upper airway may include rhinorrhoea and ulceration. In its most extreme forms, the nasal septum may collapse leading to a saddle shaped nose. A number of eye complaints, including uveitis, scleritis, episcleritis, and cranial nerve palsies are associated conditions.

Ian Bickle, senior house officer,
Elisabeth McCausland, senior house officer, general medical rotation, Royal Victoria Hospital, Belfast

Email: clonvara@yahoo.co.uk

We thank G Wright, consultant rheumatologist, and B Kelly, consultant radiologist, Royal Victoria Hospital, Belfast.

studentBMJ 2005;13:1-44 January ISSN 0966-6494

1. Scott T. Wegener's granulomatosis. www.emedicine.com/neuro/topic396.htm (accessed 7 Dec 2004).