Lumps,bumps and vasculitis

Abstract

Summary

We report an unusual case of primary vasculitis presenting with unilateral submandibular gland enlargement and lymphadenopathy resulting in stridor.

Case history

A 57-year-old Caucasian lady presented with a 9-week history of progressive painless swelling in the left submandibular region associated with mild symptoms of dysphagia with solid foods. There was no history of weight loss, fevers or night sweats. Three years previously she had undergone a subtotal thyroidectomy for a multinodular goitre for which she required thyroxine replacement. There was a past history of hypertension and type II diabetes mellitus. Examination revealed an enlarged mobile left submandibular salivary gland with associated lymphadenopathy. There was no evidence of oral pathology and an orthopantomogram did not identify salivary calculi. The full blood count, renal and liver function were normal (WBC 11.1×10⁹/L, Hb12.9 gd/L, Plts 328 × 10⁹/L, Ur 4.7 mmol/L, Cr 87 μmol/L, ALT 21 U/L, ALP 99 U/L, bilirubin 11 μmol/L). Urinalysis was unremarkable. An MRI scan confirmed the clinical findings and noted normalparotid glands and oropharynx. Fine needle aspiration demonstrated benign salivary acinar cells with evidence of chronic inflammation.

Two months later the patient was admitted as an emergency with stridor, having noticed a significant increase in the left submandibular swelling over the preceding seven days. Blood tests showed a mild leucocytosis (WBC 14.1×10⁹/L) and a raised C-reactive protein (CRP 29 mg/L). Renal function and liver function tests were normal (Ur 4.6 mmol/L, Cr 104 μmol/L, ALT 18 U/L, ALP 68 U/L, bilirubin 5 μmol/L). Urinalysis was unremarkable. An urgent CT scan of the neck revealed a left-sided soft tissue mass at the level of the thyroid extending superiorly to the cricoid and inferiorly to the thoracic inlet compressing the subglottic larynx and proximal trachea (Figure 1). An emergency tracheostomy and neck exploration was performed revealing an oedematous trachea, no obvious mass but an enlarged left submandibular gland and multiple enlarged lymph nodes. Biopsies of the submandibular gland and adjacent lymph nodes demonstrated a florid vasculitis focused around medium-sized arteries associated with fibrinoid necrosis (Figure 2) and periarterial concentric fibrosis (Figure 3).

Subsequent investigations revealed a negative autoantibody screen including negative anti-neutrophil cytoplasmic antibody (ANCA), normal immunoglobulins and complement and no evidence of connective tissue disease. A QuantiFERON®-TB gold test was negative and serum ACE levels were normal. CT imaging of the thorax, abdomen and pelvis were unremarkable.

Figure 1

(a and b) CT scans of the neck taken during the emergency presentation with upper airways obstruction. The enlarged left submandibular gland and lymphadenopathy can be seen associated with significant extrinsic compression of the trachea

Figure 2

Excised left submandibular lymph node stained with haematoxylin and eosin demonstrating fibrinoid necrosis (N) and lymphocytic infiltration (L) around a medium-sized artery

Renal function was unchanged from baseline (serum creatinine 104 μmol/L) and urinalysis was unremarkable. Immunosupression with azathioprine (2 mg/kg/day) and a tapering regime of prednisolone (1 mg/kg/day) was commenced. Eight months following presentation the left submandibular gland swelling and lymphadenopathy had resolved and she continued on maintenance immunosuppression.

Comment

Localized salivary gland enlargement and lymphadenopathy are rare presentations of systemic vasculitis. Kawasaki disease, a medium vessel vasculitis most commonly seen in childhood, may cause lymphadenopathy (hence the synonym mucocutaneous lymph node syndrome) but is also associated with a rash, fever and in severe cases coronary artery vasculitis. 1 There have been a small number of case reports of Wegener's granulomatosis presenting with submandibular and

Figure 3

High power magnification of an excised left submandibular lymph node stained with haematoxylin and eosin showing periarterial concentric fibrosis (onion skinning)

parotid swelling. 2, 3, 4, 5 These cases are invariably ANCA positive and associated with nasal involvement, ear pathology or lung lesions. This case is remarkable for the absence of upper respiratory tract involvement and systemic spread and the lack of association with a serum ANCA.

The lack of a diagnosis on the initial fine needle aspirate is perhaps not surprising as one would not expect to sample blood vessels directly using this technique. The absence of other findings suggestive of multisystem disease, infection or malignancy would support a ‘watchful waiting’ approach with regular outpatient review. In this case new symptoms developed rapidly necessitating urgent treatment and a definitive excision biopsy which provided the histological diagnosis.

Prompt recognition of the more unusual presentations of systemic vasculitis is essential toensure prompt treatment with immunosuppressive agents. Untreated systemic vasculitis is a potentially fatal disease. This case highlights the need to always consider vasculitis within a differential diagnosis even when dealing with ‘lumps, bumps’ and lymphadenopathy.

Footnotes

DECLARATIONS

Competing interests None declared

Funding None

Ethical approval Not applicable

Guarantor JB

Contributorship All authors contributed equally