A 69-year-old male patient presented as an outpatient with increased creatinine levels (281 mumol/L) associated with proteinuria (2.5 g/24 h) and hematuria (110 g/L). His history was not unusual and he denied any abuse of alcohol, drugs, or over-the-counter stimulants. Review of his history was negative for any recent fever, rash, contact with sick persons, or acute illness involving the respiratory or gastrointestinal tract. The examination of ANCA and a chest radiograph were unusual. He was diagnosed with ANCA-associated vasculitis after findings of a perinuclear ANCA (p-ANCA) positive reaction (495 U/mL), urine examination, renal insufficiency by creatinine assay, and lung infiltration by CT scan. After starting treatment with cyclophosphamide pulse therapy, sequential therapy with low-dose prednisone (10 mg qid [every other day]), he underwent a 4-year follow-up. During this time, the level of serum creatinine was stable (Figure 1), and both proteinuria and hematuria improved (Figure 2). A chest CT scan was performed, which showed that the lung fibrosis remained unchanged (Figure 3).
However, after the 4-year follow-up, the patient experienced a sudden and rapid deterioration in lung infiltration and exhibited pulmonary hemorrhage (PH) along with a progressively lower hemoglobin (Figure 3). Interestingly, there was not only no increase in creatinine levels (Figure 1), but also a negative conversion of p-ANCA. The galactomannan assay and Mycobacterium tuberculosis antigen-specific interferon (IFN)-gamma release assays (T-SPOT -TB test; Oxford Immunotec Ltd, Oxford, UK) were both negative. After immunosuppressive therapy (methylprednisolone and cyclophosphamide pulse therapy for 1 week), and empirical widened coverage of anti-infective therapy, progressive lung fibrosis occurred, the PH worsened, and he died from respiratory failure.