I.a I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.c I - Interstitial/parenchymal lung disease
I.c - Eosinophilic pneumonia (pulmonary infiltrates and eosinophilia) I.d I - Interstitial/parenchymal lung disease
I.d - Organizing pneumonia pattern (an area or areas of consolidation on imaging) I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis I.m I - Interstitial/parenchymal lung disease
I.m - ILD with a granulomatous component I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD/pulmonary fibrosis (Hamman-Rich syndrome) I.z I - Interstitial/parenchymal lung disease
I.z - An area or areas of consolidation II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma V.d V - Pleural and/or pericardial involvement
V.d - Pleural/pericardial effusion, ANA positive (DI lupus) V.m V - Pleural and/or pericardial involvement
V.m - Pleuropericarditis - Pleuropericardial effusion (ANA unknown or negative) IX.j IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.j - Phrenic nerve injury - Hemi- or bilateral diaphragmatic paralysis X.b X - Systemic/Distant conditions, syndromes and reactions
X.b - Antiphospholipid antibodies w/wo the APL syndrome X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.j X - Systemic/Distant conditions, syndromes and reactions
X.j - Myopathy-Myositis-Polymyositis (see also under Xba) X.k X - Systemic/Distant conditions, syndromes and reactions
X.k - Sarcoid-like granulomatosis (endo-/extrathoracic) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive XII.a XII - Cardiovascular involvement / toxicity
XII.a - Left or biventricular dysfunction/failure XV.a XV - Pathology
XV.a - Path: NSIP-cellular pattern (see also Ia, Ib) XV.c XV - Pathology
XV.c - Path: Organizing pneumonia (OP/BOOP) pattern (see also Id) XV.e XV - Pathology
XV.e - Path: ILD with a granulomatous component (see also Im) XVI.k XVI - Imaging
XVI.k - Imaging: An area or areas of consolidation XVII.a XVII - Infections & related conditions
XVII.a - Respiratory tract infection incl. pneumonia XVII.b XVII - Infections & related conditions
XVII.b - Opportunistic pulmonary/systemic infections XVII.c XVII - Infections & related conditions
XVII.c - Aspergillosis, pulmonary (invasive, allergic [ABPA], or mycetoma) XVII.e XVII - Infections & related conditions
XVII.e - Pneumocystis jiroveci pneumonia XVII.f XVII - Infections & related conditions
XVII.f - Tuberculosis, miliary XVII.g XVII - Infections & related conditions
XVII.g - Tuberculosis (pulmonary, pulmonary, extrapulmonary or disseminated). Reactivation or de novo XVII.z XVII - Infections & related conditions
XVII.z - Histoplamosis (reactivation) XVII.aa XVII - Infections & related conditions
XVII.aa - Coccidioidomycosis

I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) VI.d VI - Pulmonary vasculopathies
VI.d - Pulmonary vasculitis or capillaritis X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive

III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) V.a V - Pleural and/or pericardial involvement
V.a - Pleural effusion (uni- or bilateral) (can accompany DI-LDs) V.b V - Pleural and/or pericardial involvement
V.b - Eosinophilic pleural effusion VI.d VI - Pulmonary vasculopathies
VI.d - Pulmonary vasculitis or capillaritis X.m X - Systemic/Distant conditions, syndromes and reactions
X.m - Vascultis (pulmonary, extrathoracic or systemic)-ANCA status unknown or negative) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive

I.a I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.d I - Interstitial/parenchymal lung disease
I.d - Organizing pneumonia pattern (an area or areas of consolidation on imaging) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma V.d V - Pleural and/or pericardial involvement
V.d - Pleural/pericardial effusion, ANA positive (DI lupus) V.i V - Pleural and/or pericardial involvement
V.i - Pleuritis (can cause chest pain) V.m V - Pleural and/or pericardial involvement
V.m - Pleuropericarditis - Pleuropericardial effusion (ANA unknown or negative) VI.d VI - Pulmonary vasculopathies
VI.d - Pulmonary vasculitis or capillaritis VIII.a VIII - Central-large-upper airway (incl. pharyngeal-nasal) involvement
VIII.a - Angioedema (may cause UAO, asphyxia and death) VIII.c VIII - Central-large-upper airway (incl. pharyngeal-nasal) involvement
VIII.c - Upper airway obstruction other than angioedema or hematoma VIII.h VIII - Central-large-upper airway (incl. pharyngeal-nasal) involvement
VIII.h - Vocal cord dysfunction, adduction, closure, injury X.a X - Systemic/Distant conditions, syndromes and reactions
X.a - DRES syndrome - DRESS-like reaction X.b X - Systemic/Distant conditions, syndromes and reactions
X.b - Antiphospholipid antibodies w/wo the APL syndrome X.d X - Systemic/Distant conditions, syndromes and reactions
X.d - Lupus - Lupus syndrome (see also Vd) X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.m X - Systemic/Distant conditions, syndromes and reactions
X.m - Vascultis (pulmonary, extrathoracic or systemic)-ANCA status unknown or negative) X.o X - Systemic/Distant conditions, syndromes and reactions
X.o - Neutrophilic dermatosis (Pyoderma, Sweet syndrome) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.ak X - Systemic/Distant conditions, syndromes and reactions
X.ak - Dual MPO-/PR3-ANCA systemic disease/vasculitis/pseudovasculitis XII.c XII - Cardiovascular involvement / toxicity
XII.c - Pericardial effusion (w/wo tamponade) XII.k XII - Cardiovascular involvement / toxicity
XII.k - Constrictive pericarditis - Pericardial thickening XV.f XV - Pathology
XV.f - Path: Diffuse alveolar damage (DAD-pattern) (see also IL)

III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) VI.b VI - Pulmonary vasculopathies
VI.b - Pulmonary arterial hypertension IX.t IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.t - CNS involvement/dysfunction/damage X.b X - Systemic/Distant conditions, syndromes and reactions
X.b - Antiphospholipid antibodies w/wo the APL syndrome X.j X - Systemic/Distant conditions, syndromes and reactions
X.j - Myopathy-Myositis-Polymyositis (see also under Xba) X.m X - Systemic/Distant conditions, syndromes and reactions
X.m - Vascultis (pulmonary, extrathoracic or systemic)-ANCA status unknown or negative) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.ad X - Systemic/Distant conditions, syndromes and reactions
X.ad - Skin purpura, ecchymoses or necrosis X.ak X - Systemic/Distant conditions, syndromes and reactions
X.ak - Dual MPO-/PR3-ANCA systemic disease/vasculitis/pseudovasculitis XI.n XI - Miscellaneous
XI.n - Neutropenia, agranulocytosis (w/wo infection/sepsis) XII.g XII - Cardiovascular involvement / toxicity
XII.g - Coronary artery disease (acute) - Myocardial ischemia/infarction XV.al XV - Pathology
XV.al - Path: Pulmonary hypertension incl. angiomatoid vasculopathy XV.aw XV - Pathology
XV.aw - Path: Skin vasculopathy/vasculitis/fibrin thrombi XVIII.t XVIII - Distinctive patterns - 'Eye-catchers'
XVIII.t - Eye catcher: Purpuric/Ecchymotic/Cyanotic lesions of the earlobes and/or nose

III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma V.b V - Pleural and/or pericardial involvement
V.b - Eosinophilic pleural effusion VI.d VI - Pulmonary vasculopathies
VI.d - Pulmonary vasculitis or capillaritis VIII.c VIII - Central-large-upper airway (incl. pharyngeal-nasal) involvement
VIII.c - Upper airway obstruction other than angioedema or hematoma X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.x X - Systemic/Distant conditions, syndromes and reactions
X.x - Subclinical ANCA positivity XI.n XI - Miscellaneous
XI.n - Neutropenia, agranulocytosis (w/wo infection/sepsis)

I.a I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.c I - Interstitial/parenchymal lung disease
I.c - Eosinophilic pneumonia (pulmonary infiltrates and eosinophilia) I.d I - Interstitial/parenchymal lung disease
I.d - Organizing pneumonia pattern (an area or areas of consolidation on imaging) I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD/pulmonary fibrosis (Hamman-Rich syndrome) II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma IV.c IV - Airway involvement
IV.c - Obliterative bronchiolitis (a pattern consistent with) (see also IVn, XVx) IV.n IV - Airway involvement
IV.n - Obstructive airway dysfunction (see also IVc, XVx) V.a V - Pleural and/or pericardial involvement
V.a - Pleural effusion (uni- or bilateral) (can accompany DI-LDs) IX.a IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.a - Diaphragm/inspiratory muscle weakness/paralysis (w/wo ARF) IX.r IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.r - Myasthenia gravis - Myasthenic-like syndrome X.d X - Systemic/Distant conditions, syndromes and reactions
X.d - Lupus - Lupus syndrome (see also Vd) X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.j X - Systemic/Distant conditions, syndromes and reactions
X.j - Myopathy-Myositis-Polymyositis (see also under Xba) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.ac X - Systemic/Distant conditions, syndromes and reactions
X.ac - Anti-GBM antibody disease (Goodpasture-like or flare of preexisting GS) XI.t XI - Miscellaneous
XI.t - Yellow nail syndrome XV.z XV - Pathology
XV.z - Path: Obliterative (constrictive) bronchiolitis (see also IVc, IVn) XVII.w XVII - Infections & related conditions
XVII.w - Hypogammaglobulinemia - Antibody deficiency

I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.c I - Interstitial/parenchymal lung disease
I.c - Eosinophilic pneumonia (pulmonary infiltrates and eosinophilia) I.d I - Interstitial/parenchymal lung disease
I.d - Organizing pneumonia pattern (an area or areas of consolidation on imaging) I.k I - Interstitial/parenchymal lung disease
I.k - Lung nodule or nodules II.b II - Pulmonary edema - Acute lung injury - ARDS
II.b - ARDS - Acute lung injury III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.b III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.b - Pneumorenal syndrome (w/wo ANCAs) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma IV.r IV - Airway involvement
IV.r - Airway pigmentation (black, bluish, petechial) V.b V - Pleural and/or pericardial involvement
V.b - Eosinophilic pleural effusion V.d V - Pleural and/or pericardial involvement
V.d - Pleural/pericardial effusion, ANA positive (DI lupus) V.m V - Pleural and/or pericardial involvement
V.m - Pleuropericarditis - Pleuropericardial effusion (ANA unknown or negative) V.u V - Pleural and/or pericardial involvement
V.u - Eosinophilic pleuritis VI.d VI - Pulmonary vasculopathies
VI.d - Pulmonary vasculitis or capillaritis X.d X - Systemic/Distant conditions, syndromes and reactions
X.d - Lupus - Lupus syndrome (see also Vd) X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.m X - Systemic/Distant conditions, syndromes and reactions
X.m - Vascultis (pulmonary, extrathoracic or systemic)-ANCA status unknown or negative) X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.x X - Systemic/Distant conditions, syndromes and reactions
X.x - Subclinical ANCA positivity X.ak X - Systemic/Distant conditions, syndromes and reactions
X.ak - Dual MPO-/PR3-ANCA systemic disease/vasculitis/pseudovasculitis XI.n XI - Miscellaneous
XI.n - Neutropenia, agranulocytosis (w/wo infection/sepsis) XV.a XV - Pathology
XV.a - Path: NSIP-cellular pattern (see also Ia, Ib) XV.c XV - Pathology
XV.c - Path: Organizing pneumonia (OP/BOOP) pattern (see also Id) XV.h XV - Pathology
XV.h - Path: NSIP-fibrotic pattern XV.an XV - Pathology
XV.an - Path: Eosinophilic pleuritis

I.a I - Interstitial/parenchymal lung disease
I.a - Pneumonitis (ILD), acute and/or severe (may cause ARDS) I.b I - Interstitial/parenchymal lung disease
I.b - Pneumonitis (ILD) I.g I - Interstitial/parenchymal lung disease
I.g - Pulmonary fibrosis I.m I - Interstitial/parenchymal lung disease
I.m - ILD with a granulomatous component I.q I - Interstitial/parenchymal lung disease
I.q - Pulmonary nodulosis I.w I - Interstitial/parenchymal lung disease
I.w - Rapidly progressive ILD/pulmonary fibrosis (Hamman-Rich syndrome) I.y I - Interstitial/parenchymal lung disease
I.y - Progression, acceleration or exacerbation of preexisting ILD/fibrosis III.a III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.a - Alveolar hemorrhage (AH), diffuse AH (DAH) III.d III - Pulmonary/alveolar./airway hemorrhage/bleeding
III.d - Alveolar hemorrhage, diffuse (ANCA-positive/-related) IV.a IV - Airway involvement
IV.a - Bronchospasm - Wheezing - Asthma V.a V - Pleural and/or pericardial involvement
V.a - Pleural effusion (uni- or bilateral) (can accompany DI-LDs) V.d V - Pleural and/or pericardial involvement
V.d - Pleural/pericardial effusion, ANA positive (DI lupus) IX.q IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.q - Obstructive sleep apnea IX.t IX - Neuromuscular / CNS involvement - Disordered breathing during sleep
IX.t - CNS involvement/dysfunction/damage X.b X - Systemic/Distant conditions, syndromes and reactions
X.b - Antiphospholipid antibodies w/wo the APL syndrome X.d X - Systemic/Distant conditions, syndromes and reactions
X.d - Lupus - Lupus syndrome (see also Vd) X.e X - Systemic/Distant conditions, syndromes and reactions
X.e - Autoimmunity-Autoimmune conditions (+ANA, +anti-ds-DNA, +ANCAs, other auto-Abs) X.f X - Systemic/Distant conditions, syndromes and reactions
X.f - Anaphylaxis-Anaphylactoid reaction (can be fatal) X.g X - Systemic/Distant conditions, syndromes and reactions
X.g - Hypersensitivity reaction (may manifest with fever and/or skin, throat or airway involvement) X.j X - Systemic/Distant conditions, syndromes and reactions
X.j - Myopathy-Myositis-Polymyositis (see also under Xba) X.k X - Systemic/Distant conditions, syndromes and reactions
X.k - Sarcoid-like granulomatosis (endo-/extrathoracic) X.m X - Systemic/Distant conditions, syndromes and reactions
X.m - Vascultis (pulmonary, extrathoracic or systemic)-ANCA status unknown or negative) X.p X - Systemic/Distant conditions, syndromes and reactions
X.p - Serum sickness-like reaction X.s X - Systemic/Distant conditions, syndromes and reactions
X.s - Vasculitis, pulmonary (w/wo AH), extrapulmonary, systemic: ANCA-positive X.x X - Systemic/Distant conditions, syndromes and reactions
X.x - Subclinical ANCA positivity X.ae X - Systemic/Distant conditions, syndromes and reactions
X.ae - Antisynthetase antibodies (ASA) - ASA syndrome XII.a XII - Cardiovascular involvement / toxicity
XII.a - Left or biventricular dysfunction/failure XV.ba XV - Pathology
XV.ba - Path: Granulomatous lymphadenopathy XV.cs XV - Pathology
XV.cs - Path: Pleural granulomas XVII.a XVII - Infections & related conditions
XVII.a - Respiratory tract infection incl. pneumonia XVII.b XVII - Infections & related conditions
XVII.b - Opportunistic pulmonary/systemic infections XVII.d XVII - Infections & related conditions
XVII.d - Pneumocystis jiroveci pulmonary colonization XVII.e XVII - Infections & related conditions
XVII.e - Pneumocystis jiroveci pneumonia XVII.f XVII - Infections & related conditions
XVII.f - Tuberculosis, miliary XVII.g XVII - Infections & related conditions
XVII.g - Tuberculosis (pulmonary, pulmonary, extrapulmonary or disseminated). Reactivation or de novo XVII.h XVII - Infections & related conditions
XVII.h - Nontuberculous pulmonary mycobacterial infection/superinfection XVII.m XVII - Infections & related conditions
XVII.m - Fungal airway infection XVII.n XVII - Infections & related conditions
XVII.n - Legionella pneumophila pneumonia XVII.q XVII - Infections & related conditions
XVII.q - Viral pneumonia XVII.r XVII - Infections & related conditions
XVII.r - Fungal infection (pulmonary/extrapulmonary) XVII.v XVII - Infections & related conditions
XVII.v - Strongyloides stercoralis hyperinfection (w/wo DAH) XVII.y XVII - Infections & related conditions
XVII.y - Cryptococcus pulmonary and/or systemic infection XVII.z XVII - Infections & related conditions
XVII.z - Histoplamosis (reactivation) XVII.ab XVII - Infections & related conditions
XVII.ab - Protozoan infection (incl. leishmaniasis) XVII.ak XVII - Infections & related conditions
XVII.ak - Mycobacterium leprae infection (Leprosy)