Bronchoalveolar lavage cytology resembles sarcoidosis in a subgroup of granulomatous CVID

Kollert, Florian; Venhoff, Nils; Goldacker, Sigune; Wehr, Claudia; Lützen, Niklas; Voll, Reinhard; Prasse, Antje; Warnatz, Klaus

doi:10.1183/09031936.00025513

Cited by 17 publications

(14 citation statements)

References 5 publications

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“…We perform broncho-alveolar lavage, prior to biopsy, to exclude infection. It has been suggested that GLILD may be associated with a BAL lymphocytosis (>20%) in the majority of patients [52,53] , but whether further analysis of cells retrieved at BAL is able to avoid surgical biopsy has yet to be properly examined. The CD4/CD8 preponderance has varied between studies, and a higher ratio has been associated with a more favourable prognosis in terms of lung function decline in one study [53] , but not in another [54] .…”

Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

“…It has been suggested that GLILD may be associated with a BAL lymphocytosis (>20%) in the majority of patients [52,53] , but whether further analysis of cells retrieved at BAL is able to avoid surgical biopsy has yet to be properly examined. The CD4/CD8 preponderance has varied between studies, and a higher ratio has been associated with a more favourable prognosis in terms of lung function decline in one study [53] , but not in another [54] . It is recognised that abnormalities present in blood lymphocyte populations in CVID may also be present in BAL [55] , but in general there is a paucity of data on lung lymphocyte populations in CVID One study [53] included an assessment with trans-bronchial biopsy but this was not adequate to make a firm diagnosis in over 2/3 of patients.…”

Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

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Lung disease in primary antibody deficiency

Verma

Grimbacher

Hurst

2015

The Lancet Respiratory Medicine

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This Review summarises current knowledge on the pulmonary manifestations of primary antibody deficiency (PAD) syndromes in adults. We describe the major PAD syndromes, with a particular focus on common variable immunodeficiency (CVID). Respiratory infection is a common presenting feature of PAD syndromes. Respiratory complications are frequent and responsible for much of the morbidity and mortality associated with these syndromes. Respiratory complications include acute infections, the sequelae of infection (eg, bronchiectasis), non-infectious immune-mediated manifestations (notably the development of granulomatous-lymphocytic interstitial lung disease in CVID), and an increased risk of lymphoma. Although minor abnormalities are detectable in the lungs of most patients with CVID by CT scanning, not all patients develop lung complications. Mechanisms associated with the maintenance of lung health versus lung disease, and the development of bronchiectasis versus immune-mediated complications, are now being dissected. We review the investigation, treatment, and management strategies for PAD syndromes, and include key research questions relating to both infectious and non-infectious complications of PAD in the lung.

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Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

Lung disease in primary antibody deficiency

Verma

Grimbacher

Hurst

2015

The Lancet Respiratory Medicine

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“…Both CVID and sarcoidosis may show an increased CD4/CD8 ratio at bronchoalveolar lavage cytology. However, the ratio is more pronounced in sarcoidosis where 3.5 and 4.0 are considered as cut‐off points …”

Section: Discussionmentioning

confidence: 99%

“…However, the ratio is more pronounced in sarcoidosis where 3.5 and 4.0 are considered as cutoff points. 3,20,24 Sarcoidosis is usually associated with increased levels of Ig, ACE, chitotriosidase, vitamin D and calcium. On the other hand, CVID shows decreased levels of Ig, vitamin D, calcium, switched B-memory, although raised levels of ACE may be documented.…”

Section: Discussionmentioning

confidence: 99%

Common variable immunodeficiency presenting as sarcoidosis in a 9‐year‐old child

et al. 2019

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Background Granulomatous diseases are a heterogeneous group of conditions characterized by an inflammatory infiltrate with a core of macrophages, epithelioid, giant cells and a corona of fibroblasts and lymphocytes. They are associated with a wide range of disorders such as mycobacterial and fungal infections, neoplasms, immunodeficiencies and systemic inflammatory disorders as sarcoidosis. Case report We report the case of a previously healthy 9‐year‐old male child who presented with persistent cough, diffuse lymphadenopathy, enlargement of liver and spleen and protracted fever. Anemia, lymphopenia and reduced platelet count was reported, with an increase of inflammatory markers. High levels of Angiotensin‐converting enzyme and chitotriosidases were noted. A PET‐CT scan showed increased uptake of 18F‐FDG glucose in multiple lymph nodes in thorax and abdomen and in the spleen. Biopsy of inguinal and bronchial nodes showed nodal granulomatous inflammation. The child was diagnosed with sarcoidosis and treated with corticosteroids with only transient efficacy. Further tests reported panhypogammaglobulinaemia and a reduced pool of B‐memory lymphocytes. Thus, the diagnosis was revised to common variable immunodeficiency (CVID). Conclusion Common variable immunodeficiency is a heterogeneous condition with a highly variable clinical phenotype and a strong association with autoimmune disorders. The presence of noncaseating granuloma and pulmonary lesions, along with extrapulmonary features required a step by step approach to differentiate between CVID and sarcoidosis. This enables early introduction of immunoglobulin replacement therapy and decreases the morbidity and mortality of CVID.

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“…A restrictive profile at PFTs is observed with reduced carbon monoxide diffusion capacity (D LCO ) [43]. Bronchoalveolar lavage (BAL) is useful for investigating infections, while GLILD has been associated with BAL lymphocytosis (>20%) with a variable CD4/CD8 ratio [37,44]. Granulomatous disease frequently affects the liver, spleen, and skin [37,38,39].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

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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.

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Bronchoalveolar lavage cytology resembles sarcoidosis in a subgroup of granulomatous CVID

Cited by 17 publications

References 5 publications

Lung disease in primary antibody deficiency

Lung disease in primary antibody deficiency

Common variable immunodeficiency presenting as sarcoidosis in a 9‐year‐old child

The Lung in Dysregulated States of Humoral Immunity

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