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Table 1 Systemic disease-associated interstitial lung diseases: suggested clinical features and serotypes

From: Interstitial lung diseases in children

CONNESTIVE TISSUE DISEASE

MAIN CLINICAL

FEATURES

MAIN SEROLOGICAL

FEATURES

HLA

PREDISPOSITION

Rheumatoid arthritis

Arthralgia

RF IgM and IgA

Anti-CCP

Anti-keratin

HLA-DR SE

Systemic sclerosis

Sclerodactyly

ANA-SSc

Anti-centromere

Anti-topoisomerase I

(Scl70) and II

Anti-RNA polymerase

HLA-DR3, HLA-DPB1

Systemic lupus

erythematosus

Skin rash

Arthralgia

Glomerulonephritis

ANA

Anti-native DNA

Anti-nucleosome

Anti-Sm, RNP,

SSA, SSB

Anti-ribosome

CIC

 

Sjögren syndrome

Xerostomia

Serophtalmia

ANA

Anti-SSA, SSB

RF

Anti-RNP

 

Dermatomyositis

and polymyositis

Muscle weakness

Skin rash

ANA

Anti-Jo1

Anti-Mi2

Anti-SRP

C-ADM-140

 

Ankylosing

Spondylitis

Bony ankylosis

 

HLA-B27

Mixed connective

tissue disease

Raynaud phenomenon

Anti-U1-RNP

 

PULMONARY

VASCULITIS

MAIN CLINICAL

FEATURES

MAIN SEROLOGICAL

FEATURES

HLA

PREDISPOSITION

Wegener's

granulomatosis

Glomerulonephritis

Sinusitis

c-ANCA PR3

 

Churg-Strauss

syndrome

Asthma

p-ANCA

 

Microscopic

polyangitis

Glomerulonephritis

Sinusitis

Skin involvement

p-ANCA

 

Goodpasture

syndrome

Glomerulonephritis

anti-GMB

HLA-DRB1*1501

Henoch-Schönlein

purpura

Purpura

Glomerulonephritis

IgA deposition

HLA-DRB1

Cryoglobulinemic

vasculitis

Skin involvment

Hepatitis

Glomerulonephritis

Cryoglobulin

 
  1. Abbreviations: Rheumatoid factor (RF), Immunoglobulin (Ig), Human leucocyte Antigen (HLA), Anticyclic citrullinated peptide (anti-CCP), Antinuclear antibodies (ANA), Systemic sclerosis (SSc), Smith (Sm), ribonucleoprotein (RNP), circulating immune complex (CIC), anti-histidyl-t-RNA synthetase (Jo1), signal recognition particle (SRP), anti-U1-ribonucleoprotein antibody (anti-U1-RNP Ab); Cytoplasmic-staining (c) or Perinuclear-staining (p) anti-neutrophil cytoplasmic antibody (ANCA), anti-glomerular basement membrane (anti-GBM)