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. 2023 Feb 22;32(167):220188. doi: 10.1183/16000617.0188-2022

TABLE 2.

Biological investigations in childhood interstitial lung disease

Investigation Indicates
Haematology
 Complete blood count
 Reticulocytes Anaemia and/or diffuse alveolar haemorrhage
 Haemostasis Anaemia and/or diffuse alveolar haemorrhage
Biochemistry
 Serum electrolytes, creatinine
 Liver enzymes Hepatomegaly and/or pulmonary alveolar proteinosis
 Thyroxine, thyroid-stimulating hormone Surfactant disorder (NKX2-1)
 Serum protein electrophoresis, sedimentation rate Autoinflammatory/inflammatory disorder
 Angiotensin converting enzyme Sarcoidosis
 Iron, ferritin Anaemia and/or diffuse alveolar haemorrhage and inflammatory syndrome
 Calcium, ionised calcium, phosphorous Sarcoidosis
 Lactate dehydrogenase Alveolar lung injury
 Proteinuria Autoinflammatory/inflammatory disorder
 Calciuria Sarcoidosis
 Ammonaemia
 Chromatography of blood and urine amino acids Metabolic disorder, e.g. lysinuric proteinuria
 Chromatography of urinary organic acids
Serologies
 Epstein–Barr virus serology and viral load
 Cytomegalovirus serology and viral load If subacute, neonatal or immune deficiency
 HIV-1/HIV-2 serology and viral load Pneumocystis jirovecii, immune deficiency
Mycoplasma pneumoniae serology and nasopharyngeal PCR Subacute
Chlamydia pneumoniae serology and nasopharyngeal PCR Subacute
Chlamydia trachomatis serology and nasopharyngeal PCR Subacute in newborns
Ureaplasma urealyticum serology and nasopharyngeal PCR
 IgG precipitins Hypersensitivity pneumonitis, farmer's lung, bird fancier's lung
Immunology
 Post-vaccinal serologies Immune deficiency
 IgG, IgA, IgM and IgG subclasses Immune deficiency, autoinflammatory/inflammatory disorder
 C3, C4, CH50
 Lymphocyte count/differential
 Circulating immune complexes
 Antinuclear antibodies
 ANCAc (PR3), ANCAp (MPO)
 Rheumatoid factor
 Anti-CCP
 Anti-cardiolipin
 Scleroderma, polymyositis and myositis antibodies (KU, PM-Scl75, TIF1γ, MDA-5, PM-Scl100, Ml2, KJ; anti-synthetase (PL7, PL12, OJ, centromere, SRP, JO1), smooth muscle, glomerular basement membrane) Muscular, oesophageal and/or cutaneous involvement
 GM-CSF auto-antibodies Pulmonary alveolar proteinosis
 IFN-α signature, IFN-α dosage Immune deficiency, autoinflammatory/inflammatory disorder

ANCA: anti-neutrophil cytoplasmic antibody; PR3: proteinase 3; MPO: myeloperoxidase; CCP: cyclic citrullinated peptide; GM-CSF: granulocyte–macrophage colony-stimulating factor; IFN: interferon.