CYSTIC LUNG DISEASE by Nick Mark MD ONE onepagericu.

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DEFINITIONS: Very Thin (<1mm) · COPD (UL) · Ritalin Lung
or no walls
EMPHYSEMA
· ɑ1AT (LL) (LL predom) Emphysema descriptors: Cyst mimics:
Are air-filled areas present within Thick walled Thin walled · Malignancy · Septic emboli · BLEBS <2 cm CYSTIC BRONCHIECTASIS –
(>4mm) CAVITY · BULLAE > 2 cm • Dilated airways (not real cysts)
the lung parenchyma? (1-4mm) · Infection · Vasculitis
· GIANT BULLAE > 30% of hemithorax

v1.0 (2020-11-15)
HONEYCOMBING –
Thin walled
(1-4mm)
CYST • ≥3 adjacent air-filled spaces

CC BY-SA 3.0
• seen with emphysema, not
with cysts
Upper Lobe predominant Diffuse Lower Lobe predominant
LANGERHANS CELL HISTIOCYTOSIS NEUROFIBROMATOSIS TYPE 1 LYMPHANGIOLYOMIOMATOSIS BIRT-HOGG-DUBE LYMPHOCYTIC INTERSITIAL
(LCH) (NF1) (LAM) (BHD) PNEUMONITIS (LIP)
· Smoking-associated inflammation · Genetic; neurofibromin (NF1) · Genetic or spontaneous, assoc. · Genetic; AD folliculin (FLCN) · Autoimmune (Sjogren’s
♂>♀ · Skin: café-au-lait spots,axillary w/ tuberous sclerosis (TSC),♀>>♂ mutation, ♀=♂ prevalence syndrome) & immunodeficiency
· Chest: UL freckling, neurofibromas, · Skin: facial angiofibromas · Kidney: chromophobe RCC (HIV) associated inflammation
predominant · Chest: UL · Kidney: Angiomyolipoma tumors · Skin: fibrofolliculomas and · Dense lymphocyte infiltrates;
‘bizarre shaped’ predominant · Chest: Uniform size angiofibromas overlap with FB
cysts & cysts, diffuse distribution · Chest: LL · Chest: LL
‘stellate shaped’ emphysema, & of cysts, Intralobular predominant Predominant cysts
centrilobular thickening, & lentiform cysts; in bronchovascular
bullae, & LL
nodules (1-5mm) fibrosis pleural effusions often presents distribution
(chylothorax) with PTX

PNEUMOCYSTIS JIROVECI PARACOCCIDIOIDOMYCOSIS LIGHT CHAIN DEPOSITION DISEASE DESQUAMATIVE INTERSTITIAL FOLLICULAR BRONCHIOLITIS
PNEUMONIA (PJP) (LCDD) PNEUMONITIS (DIP) (FB)
· Infectious; occurs in individuals · Infectious; occurs in rural workers · Lymphoproliferative disease assoc · Smoking-associated ILD often · Associated with collagen vascular
with severe immunocompromise (immunocompentent) in S. America (esp multiple myeloma) causing associated with RB-ILD; ♂>♀ disease & immunodeficiency
(HIV CD4<200, BMT, etc) ♂>♀ non-amyloid deposition of Ab;♀=♂ · Chest: LL · Chest: centrilobular
· Chest: UL · Causes diffuse LAD and can cause · Kidney: Proteinuria/nephrotic predominant and GGOs and nodules,
pneumotoceles & granulomas in many organs syndrome Subpleural/basilar sometimes with
subpleural plebs, · Chest: scattered cysts without lobar · Chest: variable predominant cysts medium to large LL
GGOs (UL if on predominance, reverse halo sign, sized cysts, of uniform small predominant cysts
PPx, LL if not; cavitations, and bronchiectasis. nodules, & LAD size with running along
peripheral associated GGOs bronchovascular
sparing GGOs) bundles

HYPERSENSITIVITY PNEUMONITIS
· Inflammation due to inhaled
antigens, forming granulomas
· Usually causes GGO and mosaicism
rarely may cause UL cyst formation
EHLERS-DANLOS SYNDROME
· Genetic connective tissue disease,
rarely may develop diffuse cysts
AMYLOIDOSIS
· Can occur with 1° or 2 ° amyloidosis
· Chest: diffuse peripheral thin-
walled cysts, often also with nodules
(including endobronchial) or masses
PROTEUS SYNDROME
· Rare Genetic syndrome (AKT1) that
may present with diffuse cysts
CYSTIC PULMONARY
METASTATIC DISEASE
· Metastastic malignancy usually
causes cavitary (thick walled)
lesions.
· Diffuse cysts can be seen with
epitheliod metastasis, & rarely with
adenocarcinomas/sarcomas as
reported here
PULMONARY PAPPILLOSIS (PP)
· Infectious; vertically transmitted
HPV infxn; very rare.
· Chest: usually endobronchial
lesions, rarely diffuse pulmonary
nodules that turn into cysts.
FIRE-EATERS LUNG
· Aspiration of flammable petroleum
compounds causes inflammation;
leading to cavitary or cystic disease
CONSTRICTIVE BRONCHIOLITIS
· Occurs due to viral, autoimmune,
or GVHD. Typically causes mosaic
attention & bronchiectasis. Rarely
causes few small diffuse cysts
HYPER-IGE SYNDROME
· Immunodeficiency/STAT3 mutation
causing sinopulmonary infections, &
rarely pneumatoceles & cysts.