A 78-year-old Japanese woman was found to have anaemia and reticular shadows on her chest X-ray during a health examination, for which she came to our hospital. She did not have any symptoms, including fever, cough, and purulent sputum, at that time. Her dietary habits were normal and she had not experienced any weight loss. Her haemogram and serum biochemistry revealed the following: haemoglobin: 7.8 (11.3-15.2) g/dL, MCV: 78.5 (79-100) fL, MCHC: 29.5 (30.7-36.6)%, serum iron: 62 (43-172) mug/dL, ferritin: 67.2 (4.6-204.0) ng/dL, unsaturated iron-binding capacity: 188 (137-327) mug/dL, vitamin B12: 470 (180-914) pg/mL, folic acid: 3.8 (3.1-9.7) ng/mL, blood urea nitrogen: 20.9 (8-22) mg/dL, creatinine: 1.13 (0.4-0.7) mg/dL, sodium: 145 (138-146) mEq/L, potassium: 3.5 (3.6-4.9) mEq/L, and chlorides: 109 (99-109) mEq/L. Her faecal occult blood test was negative. We diagnosed the cause of anaemia as defective iron utilization, and we followed up her laboratory data and chest X-ray findings to clarify the cause of defective iron utilization.
Three months later, she was admitted to the hospital with renal failure, chest radiograph abnormality, and severe anaemia. She had a past medical history of hypertension but had never smoked and had no history of tuberculosis (TB). She had not been exposed to any fine particles. She did not have fever or any respiratory symptoms, such as breathlessness, cough, haemoptysis, dyspnoea, and chest pain.
Physical examination showed a body temperature of 36.3 C and no crackles were audible on auscultation. Her chest X-ray showed persistence of the reticular shadows in the right middle and lower lung fields. In addition, a calcified lesion was observed in the left middle part (Figure 1(a)). Chest computed tomography (CT) showed a ground glass shadow and consolidation in the right upper and lower lobes with left sided pleural thickening and calcification (Figures 1(b)-1(e)). Her laboratory findings at this time were haemoglobin 6.9 g/dL, white blood cell count 6,600 (3,500-9,000)/muL, blood urea nitrogen 61.5 mg/dL, creatinine 2.56 mg/dL, C-reactive protein (CRP) 0.0 (<0.3) mg/dL, erythrocyte sedimentation rate (ESR) 90 (2-15) mm/h, myeloperoxidase (MPO)-ANCA 54 (<10) EU, proteinase 3 (PR3)-ANCA <10 (<10) EU, and anti-glomerular basement membrane (GBM) antibody <10 (<10) EU. More than 100 counts per high power field of red blood cell (RBC) casts were found on urinalysis.
Renal biopsy was performed and pathological evaluation of the specimen revealed cellular crescent formations and lobulation in more than half the glomeruli (Figure 2). Evaluation of bronchoalveolar lavage fluid (BALF) revealed increased RBC counts, and cytology indicated that 90% of the detected macrophages were haemosiderin-laden. These findings indicated the presence of alveolar haemorrhage and, hence, she was diagnosed as MPA. She did not consent to undergo surgical lung biopsy at this time. We initially planned to treat her with corticosteroids and intravenous cyclophosphamide (IVCY), and, therefore, we first started treatment with corticosteroid therapy (prednisolone 50 mg/day) (Figure 3). After prednisolone therapy at this dose for 14 days, we started tapering it by 10 mg every 2 weeks, and once the dose of 20 mg was reached we tapered it by 5 mg every 4 weeks. This resulted in improvement in the renal failure, anaemia and lung abnormalities and, hence, IVCY was not required. Once the corticosteroid dose was reduced to 5 mg, it was combined with azathioprine. Currently, the patient is on maintenance therapy with corticosteroids (prednisolone 3 mg/day) and azathioprine (5 mg/day) without relapse of lung and renal lesions. She has been carefully followed up for development of TB by examination of chest X-rays once a month and sputum culture every three months, but with no TB prophylaxis.