BY-ANKIT GUPTA AMOL S DAHALE MODERATOR- DR.

ATUL GOEL

BRONCHIOLITIS IN ADULTS: A NEW DISEASE OR A DISEASE NEWLY DEFINED

BRONCHIOLITIS
Broad morphologic spectrum of

inflammatory events centered on small airways measuring less than 2 mm in diameter.

BRONCHI VS BRONCHIOLES
BRONCHI
CONTAIN CARTILAGE, SUBMUCOSAL GLANDS,GLOBET CELLS CILIATED CELLS PRESENT SMOOTH MUSCLE IN WALL UNDER VAGAL CONTROLE SMALL CROSS SECTIONAL AREA

BRONHIOLES
NONE SPARSE NOT UNDER VAGAL CONTROLE LARGE CROSS SECTIONAL AREA

MAJOR CONTRIBUTION TO AIRWAY MINOR CONTRIBUTION RESISTANCE IN NORMAL CONDITION

SMALL AIRWAY VS LARGE AIRWAY

AIRWAYS SMALL THAN 2 MILIMETERS IN DIAMETER ARE CONSIDERED AS SMALL AIRWAYS INCLUDES TERMINAL AND RESPIRATORY BRONCHIOLES MAINLY
BRONCHI AND INITIAL GENERATIONS OF

BRONCHIOLES CONSIDERED AS LARGE AIRWAYS

LARGE AIRWAY DISEASES

CHRONIC BRONHITIS( COPD)
ASTHMA BRONCHIECTASIS

SMALL AIRWAY DISEASES

BRONCHIOLITIS
ASTHMA AND COPD ALSO INVOLVE SMALL

AIRWAYS AS A SECONDARY PROCESS.

ETIOLOGY: CLINICAL SYNDROME ASSOCIATED WITH BRONCHIOLITIS.

A) INHALATIONAL INJURY:
-TOXIC GASES- NITROGEN OXIDE-SILO FILLERS LUNG -FLAVOURING AGENT-DIACETYL- POPCORN FACTORY -MINERAL DUST-ASBESTOS, SILICA, COAL

-CIGARETTE SMOKE
-IRRITANT GASES-CHLORINE

INHATIONAL INJURY
NO SYMPTOMS
MILD INJURY

BRONCHIOLITIS OBLITERANS
2-8 WEEKS

BRONCHIOLITIS HRSDAYS
MODERATE (cough, SOB, cyanosis, hypoxia)

RECOVERY DAYS-WEEKS

BRONCHIOLITIS OBLITERANS 2-8 WEEKS

PULMONARY EDEMA-ARDS- 330HRS

SEVERE INJURY

RECOVERY DAYS-WEEKS

BRONCHIOLITIS OBLITERANS 2-8 WEEKS

 B) IDIOPATHIC FORMS OF BRONCHIOLITIS:

-CRYPTOGENIC ADULT BRONCHIOLITIS: rare clinic-pathologic syndrome clinically distinct from commonly seen COPD and asthma middle aged women presenting as cough and SOB and as a accelerated severe obstructive respiratory disorder of short duration- 6-24 months no history of smoking, sputum, CTDS, inhalational injury is there diagnosis by exclusion- high index of suspicion

-DIFFUSE PAN BRONCHIOLITIS

found in east Asia- Japan, china, Korea familial occurrence- HLA Bw54-enviormental and genetic

men in 4th -7th decade- CHRONIC SINUSITIS

SINUSITIS precedes respiratory symptoms by decades pulm.obstructive symptoms-cough,sputum, SOB, wheeze

characteristic lab-marked increase in cold agglutinins

RESPIRATORY BRONCHIOLITIS ASSOCIATED ILD

H/O smoking with average exposure > 30 pack-years presents in 4th -5th decade with cough, SOB, coarse crackles

CRYPTOGENIC ORGANIZING PNEUMONIAIDIOPATHIC BOOP

presents as slowly resolving pneumonia with symptoms lasting less than 2 months onset as flu-like illness, cough, sore throat, malaise, crackles should be considered as a possibility whenever community acquired pneumonia fails to respond to appropriate therapy

 C) CONNECTIVE TISSUE DISEASES - rheumatoid arthritis

presents as rapidly progressing obstructive pulmonary diseases in middle-aged females with long-standing seropositive R.A abrupt onset of cough, SOB, with crackles role of penicillamine therapy as a potential cause suggested- not confirmed invariably associated with SLE, DERMATOMYOSITIS, SJOGREN SYNDROME.

 D) ORGAN TRANSPLANTATION BONE MARROW TRANSPLANT

pulmonary disease in 40%- 60% of patients with BMT BRONCHIOLITIS - progressive airflow obstruction, cough, SOB and wheeze-most frequent non-infectious resp. complication develops 2-3 months after transplantation MECAHNISM: -GVHD- damage small airways directly -METHOTREXATE prophylaxis for GVHD- antigen priming in lungs Immunosuppression for GVHD-increase in viral infection causing bronchiolitis

 HEART-LUNG TRANSPLANT
bronchiolitis- main pulmonary complication in long term survivors of HL TRANSPLANT presents months- several decades after HL T with cough, SOB and repeated upper respiratory infections Can be asymptomatic-identified by abnormal PFT MECHANISM: primary event- chronic transplant rejection Cofactors- infection (P. carinii, CMV, EBV), altered muco-ciliary clearance (injury to pulmonary nerve supply), Altered blood flow (bronchial artery ligation), loss of cough reflex (aspiration), immunosuppressive drugs( fibro-proliferative action of cyclosporine)

 Rigorous surveillance for evidence of asymptomatic

rejection- serial PFT and trans-bronchial biopsy-decrease the incidence of bronchiolitis in these settings

E) DRUG INDUCED BRONCHIOLITIS

-gold, amiodarone, penicillamine, cephalosporin sauropus androgynus-vegetable used for weight
control-outbreak of rapidly progressive respiratory disorder in Taiwan

F) INFECTIONS-mostly in children- cause

sporadic cases of bronchiolitis in adults- M. pneumoniae, viral infections H/O URI precedes cough, SOB, fever, wheeze, tachypnea

 OTHER ASSOCIATIONS
ulcerative colitis radiation pneumonitis aspiration pneumonitis ARDS( diffuse alveolar damage) vasculitis- wegners granulomatosis

PATHOLOGICAL TYPES AND CLINICAL CORRELATION

 Bronchiolitis obliterans syndrome-clinical entityprogressive airflow limitation secondary to small airway obstruction- after lung transplant

Bronchiolitis-broad histological terminflammation, narrowing, obliteration of small airways- 2 patterns: proliferative and constrictive Similar sequence of events can cause both histological patterns
Difference-type and severity of insult

Both the patterns can coexist

FEATURE
HISTOPATHOLICAL FINDINGS

PROLIFERATIVE
BOOP PATTRN Intra-luminal polyp of exudates in bronchioles, alveolar ducts, alveolar spaces accompanied by organizing exudates in distal parenchyma Intra-luminal fibrotic budsmasson bodies B/L PATCHY AIRSPACE OPACITIES INTERSTITIAL OPACITIES

CONSTRICIVE
OBLITERATIVE bronchiolitis Chronic inflammation in walls of bronchiolesconcentric narrowing, fibrosis, sm. Ms. Hyperplasia, and complete obliteration of lumen Alveoli are spared MAY BE NORMAL INCREASE IN LUNG VOLUME-on serial radiographs

RADIOLOGICAL FINDINGS

PFT

RESTICTIVE OR MIXED

OBSTRUCTIVE WITH HYPERINFLATION

CLINICAL SYNDROME

COP-idiopathic BOOP CTDS dermatomyositis, rare RA

Allograft recipientsBMT,HLT CTDs- RA