Clinical Predictors of a Diagnosis of Common Variable Immunodeficiency-related Granulomatous-Lymphocytic Interstitial Lung Disease

Mannina, Amar; Chung, Jonathan H.; Swigris, Jeffrey J.; Solomon, Joshua J.; Huie, Tristan J.; Yunt, Zulma X.; Truong, Tho Q.; Brown, Kevin K.; Achcar, Rosane Duarte; Olson, Amy L.; Cox, Christian W.; Kligerman, Seth; Curran‐Everett, Douglas; Pérez, Evans R. Fernández

doi:10.1513/annalsats.201511-728oc

Cited by 52 publications

(69 citation statements)

References 36 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Some patients may nevertheless develop bronchiectasis despite IgG replacement therapy [31]. In practice, early diagnosis and prompt initiation of IgG replacement can prevent lower respiratory tract infections and bronchiectasis [32,33]. …”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

“…Patients with GLILD usually present with dyspnea, cough, and crackles [34,37]. The prevalence of GLILD among CVID patients is approximately 10-15% [32,41]. The average delay between CVID diagnosis and GLILD detection is 7.8 years [32,38,42].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

“…The prevalence of GLILD among CVID patients is approximately 10-15% [32,41]. The average delay between CVID diagnosis and GLILD detection is 7.8 years [32,38,42]. A restrictive profile at PFTs is observed with reduced carbon monoxide diffusion capacity (D LCO ) [43].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

“…Granulomatous disease frequently affects the liver, spleen, and skin [37,38,39]. Immune thrombocytopenia or hemolytic anemia, splenomegaly, polyarthritis, a decrease in switched memory B cells, high blood CD4/CD8 T cell ratio, and high serum IgM may help identify patients with GLILD [21,32,34,45]. PFTs and thoracic CT at baseline plus every year for PFTs and every 5 years for CT are recommended in CVID-ILD [7,46].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

“…Lung MRI with diffusion may be useful for detecting bronchial and parenchymal abnormalities for long-term follow-up [48]. On CT, GLILD shows diffuse reticulations, ground-glass opacities and consolidation, with lower lobe predominance [32,34] (Fig. 1).…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

See 4 more Smart Citations

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

View full text Add to dashboard Cite

In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

show abstract

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

See 3 more Smart Citations

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

View full text Add to dashboard Cite

show abstract

Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline

et al. 2020

View full text Add to dashboard Cite

This evidence‐based clinical guideline provides consensus‐recommendations for the treatment and care of patients with primary antibody deficiencies (PADs). The guideline group comprised 20 clinical and scientific expert associations of the German, Swiss, and Austrian healthcare system and representatives of patients. Recommendations were based on results of a systematic literature search, data extraction, and evaluation of methodology and study quality in combination with the clinical expertise of the respective representatives. Consensus‐based recommendations were determined via nominal group technique. PADs are the largest clinically relevant group of primary immunodeficiencies. Most patients with PADs present with increased susceptibility to infections, however immune dysregulation, autoimmunity, and cancer affect a significant number of patients and may precede infections. This guideline therefore covers interdisciplinary clinical and therapeutic aspects of infectious (e.g., antibiotic prophylaxis, management of bronchiectasis) and non‐infectious manifestations (e.g., management of granulomatous disease, immune cytopenia). PADs are grouped into disease entities with definitive, probable, possible, or unlikely benefit of IgG‐replacement therapy. Summary and consensus‐recommendations are provided for treatment indication, dosing, routes of administration, and adverse events of IgG‐replacement therapy. Special aspects of concomitant impaired T‐cell function are highlighted as well as clinical data on selected monogenetic inborn errors of immunity formerly classified into PADs (APDS, CTLA‐4‐, and LRBA‐deficiency).

show abstract

Granulomatous lymphocytic interstitial lung disease: limiting immunosuppressive therapy—a single‐centre experience

Beaton

Gillis

Morwood

et al. 2020

Respirology Case Reports

View full text Add to dashboard Cite

Granulomatous lymphocytic interstitial lung disease (GLILD) is characterized by lymphocytic and granulomatous pulmonary infiltration occurring in common variable immunodeficiency (CVID). It is associated with increased mortality compared with CVID patients without GLILD. There are no treatment guidelines due to the low prevalence and the heterogeneity of the condition. A case review of three patients diagnosed with GLILD was performed from a single Australian centre. Patients met the European Society of Immunodeficiency criteria for CVID and a diagnosis of GLILD was confirmed by a multidisciplinary team. Patients were managed with immunoglobulin (Ig) replacement and immunosuppressive agents if required: the decision for immunosuppression was made on the basis of symptoms and declining pulmonary function. All patients clinically improved. One patient had immunosuppressive treatment ceased. GLILD responds to varying immunosuppressive regimes when IgG monotherapy fails. Immunosuppressive therapy can be discontinued following improvement, but patients require close observation. This series helps inform future GLILD treatment guidelines.

show abstract

Clinical Predictors of a Diagnosis of Common Variable Immunodeficiency-related Granulomatous-Lymphocytic Interstitial Lung Disease

Abstract: Hypersplenism and polyarthritis are strong risk factors for GLILD in patients with CVID. Percent predicted FVC remained stable over time in patients with GLILD.

Cited by 52 publications

References 36 publications

The Lung in Dysregulated States of Humoral Immunity

The Lung in Dysregulated States of Humoral Immunity

Treatment and management of primary antibody deficiency: German interdisciplinary evidence‐based consensus guideline

Granulomatous lymphocytic interstitial lung disease: limiting immunosuppressive therapy—a single‐centre experience

Contact Info

Product

Resources

About