Ace in the Hole!

negative. Serum complement levels were normal. A kidney biopsy confirmed the diagnosis of focal segmental glomerulonephritis. Chest radiography showed cystic shadows in the left lower zone ( fig. 1 a). Spirometry was normal (forced vital capacity of 2.16 liters, 86% predicted) and carbon monoxide diffusing capacity showed moderate reduction (3.32 mmol/min/kPa, 40% predicted). The 6-min walking distance was 512 m at baseline, without significant desaturation (baseline saturation 98% and final saturation at 6 min was 96%). High-resolution computed tomography (CT) showed the presence of multiple thin-walled cysts with no effusion or adenopathy ( fig. 2 ). Ultrasound of the abdomen did not show any angiomyolipomas or lymphadenopathy. Electrophoresis revealed polyclonal hypergammaglobulinemia; Schirmer’s test was normal (13 mm bilaterally). Bronchoscopic lavage was lymphocytic by differential count (neutrophils 6%, lymphocytes 56%, monocytes 38%) and was negative for Pneumocystis jirovecii cysts by Grocott’s staining. Surgical lung biopsy was advised but refused by the patient. What is your diagnosis and how would you proceed next? A 36-year-old woman presented with nonproductive cough and breathlessness on level walking for 6 months. She was a homemaker and did not have any exposure to pets or cigarettes. Her past history was significant for symmetrical upper limb predominant small joint arthritis for 8 years; she had received symptomatic analgesics for several years but had not been evaluated for the etiology of arthritis. General physical examination revealed pallor, swan neck deformity of distal interphalangeal joints and subluxation of metacarpophalengeal joints. Respiratory examination was normal. The hemoglobin level was 9.3 g/dl (normal 12–14), the mean corpuscular volume was 72 fl (normal 86 8 10), the reticulocyte index was 1.2% and serum ferritin was 300 ng/ml (normal 30–150), consistent with anemia of chronic disease. Urine examination showed dysmorphic red blood cells, with 24-hour urine protein of 560 mg/m 2 /day (normal ! 30), and serum creatinine was 1.8 mg/dl (normal 0.8– 1). Radiographs were consistent with deforming rheumatoid arthritis ( fig. 1 b); the serum rheumatoid factor was positive at 1: 160 (normal ! 1: 20) by latex agglutination, and anticyclic citrullinated peptides were strongly positive by ELISA. Antinuclear antibody, antineutrophil cytoplasmic antibody, human immunodeficiency virus ELISA and antiglomerular membrane antibody were all Received: December 8, 2010 Accepted after revision: March 2, 2011 Published online: May 13, 2011