Since 2001, increasing attention has been drawn to immunoglobulin G4 (IgG4) associated lymphoplasmacytoid infiltration, inflammation and fibrosis. This rare hyperIgG4 disease of unknown etiology has been increasingly recognized in pancreas, salivary glands, liver, lachrymal glands, kidney, aorta as well as lung. We report a case of atypical IgG4 positive lymphoplasmacytic cell infiltrate of the lungs; that had the disease process for 10 years before diagnosis.
A 43-year old African American male with history of right eye optic neuritis, migraine, beta thalassemia trait presented to our Institute with chronic dry cough, generalized body ache and fatigue for several months. His past history was significant for extensive work up of bilateral upper lobe lung infiltrates and fever 10 years ago. He had an open lung biopsy which revealed atypical lymphoplasmacytic cells infiltrating the lung. On flow cytometry no monoclonality demonstrated. No conclusive diagnosis was reached; the lung lesions underwent a spontaneous resolution. Almost five years before presentation he was diagnosed with inflammatory bowel disease which responded to a short course of mesalamine. The patient had quit smoking 10 years ago and before that he used to smoke 1 pack per day. He worked as a tailor. His family, social and personal history were non contributory. On examination the patient’s vitals were with in normal range, chest exam was significant for wheezing and inspiratory crackles in left interscapular region. Chest roentgenogram revealed a nodular lesion in the left lower lobe apical region. The pulmonary function tests showed restriction with reduced diffusion for carbon monoxide. Computerized tomogram revealed a 2.7 cm soft tissue mass involving the left lower lobe apical segment. Transbroncial biopsies on fiberoptic bronchoscopy revealed a lymphoplasmacytoid infiltrate similar to the one seen 10 years ago. Connective tissues diseases and HIV work up were negative. He had elevated ESR and CRP level. His work up for human herpes virus type 6 DNA, parvovirus B19 DNA was negative. PET scan showed abnormal FDG uptake in superior segment of left lower lobe with SUV of 4.7. Patient underwent repeat computerized tomography guided needle biopsy of left lung mass, which showed atypical lymphoplasmacytic infiltrate. A scan of the abdomen revealed left hydronephrosis with hydroureter without obstruction. A repeat computerized tomography guided needle biopsy was consistent with hyperIgG4 disease of the lung. Patient was initiated on 60 mg of prednisone per day with gratifying clinical as well as radiological improvement.
HyperIgG4 disease is characterized by chronic inflammatory state. HyperIgG4-related lung disease can be categorized into four types on the basis of the predominant CT findings: solid nodular, round-shaped ground-glass opacity, alveolar interstitial, and bronchovascular. Our patient 10 years ago had interstitial disease process which spontaneously resolved, and at current presentation had a nodular disease process. The nodular disease process responded to therapy with prednisone. We believe the inflammatory bowel disease our patient had 5 years ago, hydroureter and hydronephrosis was also a part of the hyperIgG4 disease clinical manifestation.
Our case highlights the need to increase the awareness of the disease process in the clinicians as well as pathologists. The delay in diagnosis can lead to increased morbidity and unneccessary invasive procedures as was seen in our case. For a decade he underwent repeated bronchoscopies and biopsies by interventional readiology. The multisystem involvement further complicates the clinical picture.
Ketan Patel, No Financial Disclosure Information; No Product/Research Disclosure Information