结缔组织病合并肺间质病变

Interstitial lung disease, ILD: AKA: Diffuse parenchymal
lung disease, DPLD Why?
Typically a diffuse process all over the lungs: Lung interstitium (the primary site) + Lung parenchyma: airspaces, peripheral airways and vessels Classifications:
Am J Respir Crit Care Med Vol(165):277-304, 2002
2002 ATS/ERS consensus
Epithelial cells
Cell death
Growth factors and other products of epithelial cell Injury
Am J Respir Crit Care Med 2007;176:1026-1034,
The development of ILD is associated with: shortened survival, more severe underlying joint disease and tobacco use
CTX: 1) 1->2 mg/kg.d po, 2) prednisolone 20mg Qod po+ 6* CTX 600mg/m2 Iv Q4w, followed by AZA 2.5 mg/kg/day (maximum 200 mg/d) po as maintenance therapy
Pneumothorax and pneumomediastinum have been described with the majority of patients noted to have DM.
Autoantibodies: ANA, anti-SSA/Ro Ab, tRNA synthetase antibodies such as anti-PL-12 (anti-alanyl-tRNA synthetase) , anti-Jo-1 (histidyl tRNA synthetase) Ab
How is it diagnosed?
Chest radiographs:
ILD
sensitivity
specificity
CXR
80%
82%
HRCT
94%
100%
Pulmonary function test, PFT:
• Restrictive hypoventilation: forced vital capacity (FVC) and total lung capacity (TLC)
Yue Yang, MD. Dep. of Rheumatology, PKUPH
The interstitium:
Connective tissue- rich supportive framework surrounding the air sacs (alveolae) of lungs
Serves as a conduit and fluid channel for lymphatic drainage and the migration of immune cells
3 subdivisions:
Alveolar interstitium: situated between the alveolar and capillary basement membranes
• Reduced diffusing capacity: DLCO
• Decreased static lung compliance
How is it diagnosed? Bronchoalveolar lavage fluid, BALF: • Exclusion of alternative diagnosis: infection, malignancy,
MMF, TNFi, RTX, tocilizumab
Curr Rheumatol Rep.2010; 12(5):363-369. Chest.2009;136(1):23-30 .

Rheumatoid pulmonary nodules: uncommon (1%), may be 15mm, single or multiple, may cavitate
etc.
Lancet 2012; 380: 689–98
Traditionally: pulmonary hypertension- more likely in lcSSc; ILD- more likely in dcSSc.
Scleroderma Lung Study: all SSc pts are at risk for ILD Gastroesophageal reflux: risk factor antireflux medications
Histopathology: UIP pattern has improved survival compared with IPF
Treatment: 1st line: high-dose prednisone Immunosuppressive agents (evidence is week): AZA, CsA,
Mostly: a chronic, slowly progressive course; subacute worsening may occur.
“Fulminant ILD”: rapidly progression with acute hypoxemic respiratory failure from diffuse alveolar damage or fulminant OP is seen most often in patients with amyopathic dermatomyositis, ADM.
vasculature from progressive fibrosis and hypoxia-induced vascular remodeling
Ann Rheum Dis . 2007; 66(12): 1641-1647. Arthritis Rheum.2011; 63(9):2797-2808.
(high-dose PPI and prokinetic medications)? The median survival for patients with SSc-ILD is 5-8 years. Most common findings on radiograph: GGO and fibrosis Staging system according to fibrosis on HRCT and FVC Pulmonary hypertension: destruction of the pulmonary
Severe reticular opacities on HRCT: most likely to benefit
prevention of progression rather than reversal of fibrotic changes
Others: AZA, CsA, FK506, MMF, RTX, stem-cell transplantation
3.5% of patients with RA were given a diagnosis of ILD prior to the diagnosis of RA
HRCT scan: RA-UIP pattern has worsened survival compared with NSIP
Drug-induced pneumonitis: MTX LEF: rare, more common in RA patients with previous
exposure to MTX or with preexisting ILD Chronic rituximab-induced lung disease: macronodular OP,
drug toxicity, environmental exposure, etc • Cell counts and populations: diagnosis and evaluation of the
reaction to treatment Lung biopsy:
Specific autoantibodies of CTDs: • Involved in the pathogenesis of ILD • Eg. Anti-CCP Ab, RF, anti-SSA/Ro Ab, Anti-Jo-1 Ab, AECA,
Chest.2009;136(1):23-30 .
An important variant: the antisynthetase antibody syndrome
Pay attention to underlying tumors
ILD is common (35%-45%) and presents prior to the onset of myositis in 18% to 20% of patients.
Axial interstitium: surrounding the bronchi, arteries, and veins from the lung root to the level of the respiratory bronchiole
Subpleural interstitium: situated beneath the pleura, as well as in the interlobular septae