This is a 21-year-old Caucasian male with a history of recurrent episodes of generalized joint pains (polyarthralgia) over a 2-year period before the current presentation. These recurred multiple times and resolved spontaneously with no specific rheumatologic diagnosis (he was HLA B27 negative). The only pertinent findings of note on physical examination were diffuse cervical lymphadenopathy, inflamed tonsils and splenomegaly.
The patient had been experiencing polyarthritis for the previous 2 years. These arthralgias and arthritis recurred multiple times, however would resolve spontaneously without any treatment. No specific diagnosis made under Rheumatology Care. Due to increased dysphagia and potential airway compromise, he underwent a tonsillectomy at the beginning of August 2015. At the same time, he was also noted to have diffuse lymphadenopathy as well as splenomegaly. Computed tomography (CT) scan showed bilateral tonsillar hypertrophy which was dealt with surgically. For anaemia, he had upper and lower gastrointestinal scopes which showed no lesions, a subsequent capsule endoscopy showed ulceration in terminal ileum. This was followed by a double balloon push enteroscopy for tissue diagnosis.
He lives with his parents and played competitive Ice Hockey.
He was a non-smoker, occasional alcohol user and not a recreational drug user. He worked mainly at construction sites.
Escitalopram 20 mg daily May and June 2014,
Pregabalin 50 mg three times daily from July to December 2014,
Duloxetine 30 mg daily from December 2014 to June 2015.
Patient underwent tonsillectomy for dysphagia and potential airway compromise in the community before his referral to nephrology service. The tonsillar tissue did not show any evidence of lymphoproliferative disorder on histopathology. Further workup showed a positive cANCA with PR3 positivity (a titre of 881 MFU (Normal < 120 MFU)). Chest x-ray showed vague pulmonary infiltrates, and the patient tested negative for tuberculosis (Figure 1). CT chest showed pulmonary parenchymal findings in keeping with an infectious process (Figure 1).
He had evidence of significant anaemia and underwent an endoscopy for gastrointestinal symptoms and anaemia (Haemoglobin 63 g/L (Normal 135-175 g/L)) that showed only ulceration in terminal ileum. The biopsy of the intestinal tissue did not show any evidence of vasculitis.
The kidney function was normal at presentation and remained stable (within the normal range) throughout the follow-up period (Figure 2). At 2 months of follow-up, he was noted to have new onset of microscopic haematuria and low-grade proteinuria. Urinalysis showed 1+ haemoglobin and trace protein, and urine protein: creatinine ratio of 36.9 mg/mmol (Normal < 13 mg/mmol) (Figure 2). Urine Microscopy for sediment showed dysmorphic red blood cells. A urinalysis completed 3 months prior to presentation was completely bland (we could not trace any urinalysis after this till Nephrology was consulted). Relevant serology results were all negative except for the ANCA PR3 positivity. The serology workup completed was as follows, and were all negative or within a normal range:
Antinuclear antibody (ANA)
Rheumatoid factor <10 (20 KU/L)
Anti-cyclic citrullinated peptide (anti-CCP) < 8 (0-16 U/ml)
Anti-double stranded DNA (anti-dsDNA) 14(<120 MFU)
Extractable nuclear antigens (ENA)
Hepatitis B surface antigen (HBsAG)
Hepatitis B surface antibody (HBsAb) 4.98 IU/L
Hepatitis C antibody
Parvovirus IgM
Serum complements [C3 1.1.(0.80-2.1 g/L) and C4 0.38(0 > 15-0.50 g/L)]
IgG 12.6 (6.94-16.18 g/L)
IgA 2.51 (0.70-4 g/L)
IgM 0.87 (0.60-3.00 g/L)
Immunoglobulins:
Based on the previous features of a systemic illness and new findings on urinalysis and urine microscopy, the patient underwent a kidney biopsy, and the tissue was analysed and reported by our pathology service (Figure 3). The diagnosis was in keeping with ANCA-associated vasculitis (focal proliferative and necrotizing glomerulonephritis). In summary, there were 11 glomeruli present in representative sections, and two contained segmental proliferative foci. The remaining glomeruli showed no conspicuous changes on light microscopy. In the plastic embedded material prepared for electron microscopy, the light microscopy revealed seven additional glomeruli of which one showed segmental sclerosis and another showed segmental proliferative focus with crescentic formation. There was mild interstitial edema and patchy mild interstitial fibrosis and tubular atrophy without significant interstitial inflammation. In consultation with our pathologists, the lesion was classified as focal based on Berden's classification.
We did evaluate the case using the Birmingham Vasculitis Score (BVAS), and overall score was eight.