Locations:
Search IconSearch
September 20, 2021/Pulmonary/Case Study

Case Report: Common Variable Immunodeficiency and Lung Disease

Multidisciplinary treatment of rare and complex immunodeficiencies

21-PUL-2311233 Comples Case Immunology Fernandez_CQD

By James Fernandez, MD, PhD

Advertisement

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Presentation

A 36-year-old female presented to Cleveland Clinic Respiratory Institute’s Immunology Clinic as a referral for immune dysfunction. Her medical history had been unremarkable except for a recent fall from her horse. Testing in an outside emergency department had uncovered a platelet count of 46 k/uL. She was then diagnosed with immune thrombocytopenia (ITP) and treated appropriately. She was referred to our clinic for long-term management.

Our clinic ordered further lab testing, which revealed an IgG of 191 mg/dL, an IgA of 26mg/dL and an IgM of 55 mg/dL. After showing poor response to multiple vaccine challenges, she was diagnosed with Common Variable Immunodeficiency (CVID) and began intravenous IgG.

Soon after, she developed bloody diarrhea. Abdominal computed tomography (CT) showed multiple lung nodules and diffuse adenopathy. A CT chest confirmed the nodules.

Pre-treatment. CT chest shows numerous bilateral upper lung and lower lung pulmonary nodules without cavitation or calcification, measuring up to 17 mm.

Fecal analysis showed camphylobacter and Shiga toxin-producing E. coli, and an infectious disease specialist prescribed levofloxacin. Axillary lymph node biopsies showed follicular hyperplasia. Shortly after, she developed worsening shortness of breath. An open lung biopsy showed chronic lymphocytic bronchiolitis with areas of organizing pneumonia and lymphoid hyperplasia.

Wedge biopsies of upper and lower lobes of right lung. A. Lymphocytic bronchiolitis with a giant cell (arrow). B. Organizing pneumonia characterized by alveolar plugs of fibromyxoid tissue (short arrow) in association with the lymphoid follicles (long arrows).

Advertisement

Managing CVID and comorbidities

Managing a patient with CVID, lung nodules and lymphadenopathy with potential for lymphoma requires a multidisciplinary approach. About one-third of patients with CVID have developed chronic pulmonary disease by the time of diagnosis. Furthermore, lymphomas also occur at much higher rates in patients with CVID, so a lymph node biopsy is important early in the course of treatment. A swift diagnostic and treatment approach is important in all immunodeficient patients.

This patient’s disease was originally treated with prednisone and had responded well, but shortness of breath recurred upon weaning to 5 mg every other day. Her reactive lymphadenopathy also responded well to the steroids.

The patient was presented at a weekly interstitial lung disease (ILD) clinic meeting, and the team decided to treat her with rituximab. Shortly thereafter, she received her first course, with excellent response clinically and radiographically. She receives periodic dosing (approximately once a year) of rituximab with continued control of her respiratory disease.

CT chest showing significant improvement in number and size of pulmonary nodules.

This patient is one of the many with rare and complex immunodeficiencies seen in our immunology and ILD clinics each week. This expertise has allowed Cleveland Clinic to build a team approach with immunologists, pulmonologists, hematologists, oncologists and other subspecialty physicians that are uniquely familiar with these types of patients.

Dr. Fernandez is staff in the Department of Allergy and Clinical Immunology.

Advertisement

Related Articles

POCUS
November 16, 2022/Pulmonary/Case Study
Case Study: Point of Care Ultrasound to Guide Complex Hemodynamic Management

A recent case illustrates how timely and appropriate use of POCUS can provide benefits at all stages of the care path

CT of chest
November 2, 2022/Pulmonary/Case Study
Case Report: Pulmonary Hypertension in a Patient with IgG4-Related Disease

An inability to obtain a biopsy of the patient’s hilar lymphadenopathy due to severe PAH and severe hypoxemia made diagnosis verification challenging

Sarcoidosis
July 12, 2022/Pulmonary/Case Study
Case Study: Pulmonary Sarcoidosis

A multidisciplinary approach to treatment can be beneficial since other organs in addition to the lungs can be affected by the systemic disease

chest examination
April 21, 2022/Pulmonary/Case Study
Dyspnea, Wheezing and Cough: A Case Study

This case illustrates the importance of looking at the bigger picture when considering a patient’s respiratory symptoms.

21-PUL-2223308_Fig3B 650×450-hero
August 18, 2021/Pulmonary/Case Study
Case Study: Invasive Cardiopulmonary Exercise Testing Helps Uncover True Cause of Dyspnea

Ruling out a common diagnosis and identifying an elusive condition

650×450-Chronic-thromboembolic-pulmonary-hypertension
February 2, 2021/Pulmonary/Case Study
Expert CTEPH Centers Provide Valuable Diagnostic and Therapeutic Skill

Pulmonary endarterectomy improves outcomes for many patients

650×450-vape-CT-chest-coronal
November 1, 2019/Pulmonary/Case Study
A Closer Look at Vaping-Associated Lung Injury: What You Can Do for Your Patients

Physicians present a case study, discuss clinical guidelines and the value of a multidisciplinary approach

650×450-Bacteria
August 19, 2019/Pulmonary/Case Study
Phage Therapy for Multidrug Resistant Bacterial Infections

Cleveland Clinic experience with a lung transplant patient

Ad