Principal and ancillary diagnostic studies in suspected idiopathic pulmonary hemosiderosis

Laboratory tests

Serologic studies
- Antinuclear antibodies (ANA)
- Antidouble stranded DNA antibodies (anti-dsDNA)
- Antineutrophil cytoplasmic antibodies (ANCA)
- Antiphospholipid antibodies (APLA: IgG and IgM antibodies to cardiolipin and beta2-glycoprotein I) and lupus anticoagulant test
- Antiglomerular basement membrane antibodies
- Rheumatoid factor
- Plasma IgA anti-tissue transglutaminase; IgA and IgG antibody to synthetic deamidated gliadin peptides
- Plasma cryoglobulins
- Plasma immunoglobulins (IgG, IgA, IgM)
- Plasma anti-cow's milk (IgG, IgE) antibodies (optional)*
Sputum examination for hemosiderin-laden macrophages
Serum creatinine, BUN, AST, ALT
Serum brain natriuretic peptide
Urinalysis for protein, hemoglobin, RBC casts

Ancillary testing in selected patients based on clinical presentation

Bone marrow biopsy for stainable iron
Examination for gastrointestinal, urinary, genital tract bleeding
Skin biopsy of cutaneous lesions (leukocytoclastic vasculitis)
Renal biopsy (glomerulonephritis)
Jejunal biopsy (celiac disease)
CTPA to exclude thromboembolic disease
Echocardiogram if heart failure suspected

Evaluation for anemia and bleeding disorders

CBC and differential cell count
Serum iron and transferrin saturation
Serum ferritin, B12, folate
Reticulocyte count
Screening for coagulation defects (PT, INR, PTT)

Pulmonary function tests

Spirometry, lung volumes, DLCO, 6MWT

Imaging

Chest radiograph
HRCT of thorax

Flexible bronchoscopy

Sequential wedged bronchoalveolar lavage
Transbronchial lung biopsy

Lung biopsy

VATS or open

Histopathology

Perls Prussian blue stain
Immunofluorescence or immunohistochemistry
Electron microscopy (optional)

IgG: immunoglobulin G; IgM: immunoglobulin M; IgA: immunoglobulin A; IgE: immunoglobulin E; BUN: blood urea nitrogen; AST: aspartate aminotransferase; ALT: alanine aminotransferase; RBC: red blood cell; CTPA: computed tomography pulmonary angiography; CBC: complete blood count; PT: prothrombin time; INR: international normalized ratio (for prothrombin time); PTT: partial thromboplastin time; DLCO: diffusing capacity for carbon monoxide; 6MWT: six-minute walk test; HRCT: high resolution computed tomography; VATS: video-assisted thoracoscopic surgery; IPH: idiopathic pulmonary hemosiderosis.

* Role of cow's milk antibodies in the pathogenesis of IPH is unclear.

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خرید پکیج

Principal and ancillary diagnostic studies in suspected idiopathic pulmonary hemosiderosis