A 70-year-old Caucasian male was diagnosed with iris deposits and elevated IOP LE. On examination, VA was 20/25 RE and 20/30 LE. IOP was 10 mm Hg RE and 16 mm Hg LE, on topical anti-glaucoma medication LE. Gonioscopy revealed ~200 translucent spherules of 200–400 microns diameter in the angle LE [Fig. and ]. Glaucomatous optic disc cupping with superior and inferior arcuate field defects were noted LE. Fine needle aspiration biopsy revealed amorphous material with mild Congo red staining, consistent with amyloid []. Systemic evaluation was normal. Medication compliance was poor with eventual glaucoma progression and refusal of surgical intervention. At 10-year follow-up, phthisis bulbi with no light perception was noted. The RE examination was normal all throughout.
A 71-year-old woman was investigated for a one-year history of deteriorating dyspnoea on a background of well-controlled asthma. Other co-morbidities included cutaneous systemic lupus erythematosus, rheumatoid arthritis and Sjogren’s syndrome. High-resolution computerised tomography (HRCT) showed multiple cysts, solid nodules and aggregate masses – the largest (2 × 1.5 cm) in the peripheral left lower lobe with fine-needle aspiration suggestive of amyloid. Serum biochemistry showed a polyclonal elevation of IgG, no detectable monoclonal band and normal serum-free light chains. Immunohistochemistry showed no specific staining for kappa or lambda antibodies. Mass spectrometry was not performed. Echocardiography excluded cardiac involvement and no visceral uptake by 123I SAP scintigraphy. Pulmonary function tests demonstrated a restrictive pattern, and a further HRCT raised suspicion of lymphocytic interstitial pneumonitis. FDG-PET/CT demonstrated similar low-grade uptake in all lung nodules (). A mini-thoracotomy was performed to obtain tissue from the largest nodule showing evidence of amyloid but too few infiltrating cells were present to confirm clonality.\nA low-grade lymphoplasmocytic lymphoma (LPL) was presumed to be driving the pulmonary amyloid, based on the assumption of low-grade B-cell clonal disorders that can occur with Sjogren’s syndrome. A trial of R-CVP (Rituximab – Cyclophoshamide, Vincristine, Prednisolone) chemotherapy was commenced for four months resulting in no progression of symptoms and stable lesions.