Follicular bronchiolitis in an adult patient with common variable immunodeficiency: 3-year follow-up
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Pneumonology Department, Aristotle University of Thessaloniki, G. Papanikolaou” General Hospital, Exochi, Thessaloniki
Pathology Department, “G. Papanikolaou” General Hospital, Thessaloniki
2nd Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokratio Hospital, Thessaloniki
Publication date: 2021-07-05
Corresponding author
Despina Papakosta   

Pneumonology Department Aristotle University of Thessaloniki, 13 P. Kavda Street, N. 751 Panorama, 55236 Thessaloniki
Pneumon 2014;27(2):163-168
Pulmonary manifestations of common variable immunodeficiency, apart from lung infections, include interstitial lung disease such as bronchiolitis. Follicular bronchiolitis as a result of immunodeficiency is mostly described in children. The interesting case of a 35 year old female patient is presented. The patient was admitted to our Department for the investigation of fever up to 38.5 οC for a period of 3 months. The patient had been diagnosed with follicular bronchiolitis 2 years ago. Upon admission the patient was febrile and presented mild cough. Blood tests revealed anemia and leucopenia. Chest-X ray was normal, but the high-resolution CT scan showed subcarinal lymph nodes, ground glass opacities and nodules. Bronchoscopy revealed no specific findings. Bronchoalveolar lavage showed a high percentage of lymphocytes. The results of pulmonary function tests revealed a restrictive disorder. Due to raised suspicion for a lymphoproliferative disease, the patient underwent bone marrow biopsy that was normal. A significant decrease of IgA, IgM and IgG was afterwards detected and the patient was diagnosed with common variable immunodeficiency. Subsequently she started treatment with intravenous immunoglobulin. The patient’s follow-up 3 years after the immunodeficiency diagnosis as well as response to treatment are presented.
Thickett KM, Kumararatne DS, Banerjee AK, Dudley R, Stableforth DE. Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings. Q J Med 2002; 95:655–662.
Park JH, Levinson AI. Granulomatous Lymphocytic Interstitial Pneumonia in Common Variable Immunodeficiency. Clin Immunol 2010; 134:97-103.
Chase NM, Verbsky JW, Hintermeyer MK et al. Use of Combination Chemotherapy for Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) in Patients with Common Variable Immunodeficiency (CVID). J Clin Immunol 2013; 33:30–39.
Bierry G, Boileau J, Barnig C et al. Thoracic Manifestations of Primary Humoral Immunodeficiency: A Comprehensive Review. RadioGraphics 2009; 29:1909–1920.
Ηowling SJ, Hansell DM, Wells AU, Nicholson AG, Flint JD, Muller NL. Follicular Bronchiolitis: Thin-Section CT and Histologic Findings. Radiology 1999; 212:637-642.
Ryu JH, Myers JL Swensen SJ. Bronchial Disorders. Am J Respir Crit Care Med 2003; 168:1277-1292.
Travis WD, Galvin JR. Non-neoplastic pulmonary lymphoid lesions. Thorax 2001; 56:964–971.
Papiris SA, Malagari K, Manali ED, et al. Bronchiolitis: adopting a unifying definition and a comprehensive etiological classification. Expert Rev Respir Med 2013;7:289-306.
Saldías PF, Díaz PO, González BS, Osses AR. Bronchiolar disorders: clinical-radiological assessment and classification. Rev Med Chil 2011; 139:1218-1228.
Romero S, Barroso E, Gil J, Aranda I, Alonso S, Garcia-Pachon E. Follicular bronchiolitis: clinical and pathologic findings in six patients. Lung 2003c; 181:309-319.
Perotin JM, Deslee G, Dury S, Patey M, Toubas O, Lebargy F. Follicular bronchiolitis Rev Mal Respir 2007; 24:1133-1137.
Kinane BT, Mansell AL, Zwerdling RG, Lapey A, Shannon DC. Follicular bronchitis in the pediatric population. Chest 1993; 104:1183-1186.
Salzer U, Warnatz K, Peter HH. Common variable immunodeficiency - an update. Arthritis Res Ther 2012; 14:223.
Guinee DG Jr. Update on Neoplastic Pulmonary Lymphoproliferative Disorders and Related Entities. Arch Pathol Lab Med. 2010; 134:691-70.1.
Hare SS, Souza CA, Bain G et al. The radiological spectrum of pulmonary lymphoproliferative disease. Br J Radiol 2012; 85:848-864.
Devakonda A, Raoof S, Sung A, Travis WD, Naidich D. Bronchiolar Disorders A Clinical-Radiological Diagnostic Algorithm. Chest 2010; 137:938-951.
Webster ADB. Humoral imunodeficiency and the lung. Eur Respir Mon 2006; 34:220-233.
Chapel H, Cunningham-Rundles C. Update in understanding Common Variable Immunodeficiency Disorders (CVIDs) and the management of patients with these conditions. Br J Haematol 2009; 145:709–727.
Tarzi MD, Grigoriadou S, Carr SB, Kuitert LM, Longhurst HJ. Clinical Immunology Review Series: An approach to the management of pulmonary disease in primary antibody deficiency Clin Exp Immunol 2009; 155:147-155.
Cunningham-Rundles C. The many faces of common variable immunodeficiency. Hematology Am Soc Hematol Educ Program 2012; 2012:301-305.
Tam JS, Routes JM. Common variable immunodeficiency. Am J Rhinol Allergy 2013; 27:260–265.
Cunningham-Rundles C. How I treat common variable immune deficiency. Blood 2010; 116:7-15.
Pereira AC, Kokron CM, Romagnolo BM et al. Analysis of the sputum and inflammatory alterations of the airways in patients with common variable immunodeficiency and bronchiectasis. Clinics (Sao Paulo) 2009; 64:1155-1160.
Fernández Pérez ER. Granulomatous lymphocytic interstitial lung disease. Immunol Allergy Clin North Am 2012; 32:621-632.
Bates CA, Ellison MC, Lynch DA, Cool CD, Brown KK, Routes JM. Granulomatous-lymphocytic lung disease shortens survival in common Variable immunodeficiency. J Allergy Clin Immunol 2004; 114:415-421.
Costa-Carvalho BT, Wandalsen GF, Pulici G, Aranda CS, Solé D. Pulmonary complications in patients with antibody deficiency. Allergol Immunopathol (Madr) 2011; 39:128-132.
Wheat WH, Cool CD, Morimoto Y et al. Possible role of human herpesvirus 8 in the lymphoproliferative disorders in common variable immunodeficiency. J Exp Med 2005; 202:479-484.
Wong GK, Goldacker S, Winterhalter C, et al; Clinical Working Party of the European Society for Immunodeficiencies (ESID). Outcomes of splenectomy in patients with common variable immunodeficiency (CVID): a survey of 45 patients. Clin Exp Immunol 2013;172:63-72.
Aerni MR, Vassallo R, Myers JL, Lindell RM, Ryu JH. Follicular bronchiolitis in surgical lung biopsies: clinical implications in 12 patients. Respir Med 2008;102:307-312.
Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immunodeficiency over 4 decades. Blood 2012; 119:1650-1657.
da Silva SP, Resnick E, Lucas M et al. Lymphoid Proliferations of Indeterminate Malignant Potential arising in Adults with Common Variable Immunodeficiency Disorders: Unusual Case Studies and Immunohistological Review in the Light of Possible Causative Events J Clin Immunol 2011; 31:784-791.
Busse PJ, Farzan S, Cunningham-Rundles C. Pulmonary Complications of Common Variable Immunodeficiency. Ann Allergy Asthma Immunol 2007; 98:1-8.
Gregersen S, Aaløkken TM, Mynarek G et al. Development of pulmonary abnormalities in patients with common variable immunodeficiency: associations with clinical and immunologic factors. Ann Allergy Asthma Immunol 2010; 104:503-510.
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