IgG4 Related Disease Emerging

Abstract

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated involvement documented in widespread organs including the pancreas, lung, salivary glands, and orbital glands. CASE PRESENTATION: We present a 64 year old Caucasian male with medical history of recurrent colon cancer status post resections, chemoradiation, and uncontrolled diabetes mellitus who presented with bilateral lung infiltrates on surveillance CT abdomen. He had several biliary stents placed for obstructive jaundice due to chronic pancreatitis over three years. He was a nonsmoker and firefighter for 37 years. He reported nonproductive cough with increased dyspnea on exertion for three years and new, worsening dry eyes and mouth. DISCUSSION: Anti-Nuclear Antibody, Anti-Neutrophil Cytoplasmic Antibody, Acid Fast Bacilli, and fungal serology were negative. Initial transbronchial lung biopsy showed chronic inflammation. Pleural fluid was transudative with eosinophils 1, lymphocytes 41, macrophages 2, monocytes 9, segmented neutrophils 42, monocytes 5. CT chest showed prominent interstitial markings bilaterally; ground glass opacities, moderate pleural effusions and reticulonodular infiltrates. He underwent hepaticojejunostomy for obstructive jaundice and repeat transbronchial lung biopsy. Biopsies of lung, pancreas, gallbladder, and common bile duct stained positive for IgG4. Repeat bronchoalveolar lavage showed increased eosinophils 100 with lymphocytes 18, macrophages 9, segmented neutrophils 71. His serum IgG4 was elevated at 136 with normal IgG1, 2, and 3. He was diagnosed with IgG4-RD demonstrated by pancreatic and pulmonary features. He was started on prednisone with marked improvement in pulmonary function tests, exercise tolerance, and diabetes control. CONCLUSIONS: Our patient received surgical therapy for obstructive jaundice due to chronic pancreatitis for 3 years. IgG4-RD is steroid responsive, however, duration of therapy is unknown. Relapse has been shown in 50% of patients with alteration of therapy. Exact diagnostic criteria have not been established but multiorgan involvement especially autoimmune pancreatitis clinches the diagnosis. IgG4-RD is a condition that affects many organs thereby having protean manifestations. A high index of suspicion for this emerging condition needs to be maintained for early diagnosis, initiation of corticosteroid therapy, and avoidance of unnecessary surgeries. With earlier recognition, diagnosis, and treatment patients with IgG4-RD have increased quality of life and overall mortality.