Pulmonary Eosinophilias & Vasculitides Prof. Dr. Abdul Hameed Al Qaseer

Pulmonary Eosinophilias : They are a group of disorders of different etiologies in which lesions in the lungs produce a chest x- ray asso- ciated with pulmonary eosinophilia , with or without a peripheral blood eosinophilia.

Causes : Extrinsic ( causes known ): 1. Helminths e.g. Ascaris , Toxocara ,Filaria . 2. Drugs : Nitrofurantoin , PAS , Sulfasalazine ,aspirin , imipramin , tetracycline , sulphonamides , penicillin , nalidixic acid , chlorpropamide, phenyibutazone ……… 3. Fungi : e.g. Aspergillus fumigatus causing allergic bronchopulmonary aspergillosis ABPA .

B. Intrinsic ( cause unknown ): 1. Cryptogenic eosinophilic pneumonia . 2. Churg – Strauss syndrome . 3. Hypereosinophilic syndrome . 4.Polyarteritis nodosa .

Allergic bronchopulmonary Aspergillosis ABPA ABPA is caused by a hypersensitivity reaction to A. fumigatus involving the bronchial wall & peripheral part of the lung . It is associated with asthma . Clinical Features : Fever , SOB ,productive cough ( cast) & worsening asthma. ABPA may lead to bronchiectasis .

Investigations : CXR : Include transient diffuse pul. Infiltrate & lobar or segmental pul. Collapse . Bronchiectasis ( tram-line, ring & gloved-finger shadows) predominantly in the upper lobes. Positive skin test to A. fumigatus . Elevated total IgE > 1000 ng /ml . Elevated serum IgE- A. fumigatus or IgG A. fumigatus . Blood eosinophilia > 0.5 x 109 / L . Fungal hyphae of A. fumigatus in the sputum.

Management of ABPA : 1.Prednisolone 40-60 mg /d + physiotherapy 2. Bronchoscopy ( lavage) to remove impacted mucus. 3. Some advocate high –dose inhaled corticosteroids . 4. Therapy with itraconazole may be beneficial in some .

Acute Eosinophilic Pneumonia It is a rare febrile illness of 5-7 days durations . It may lead to hypoxic respiratory failure . BAL demonstrates > 25% eosinophils . The condition responds to corticosteroids . Chronic Eosinophilic Pneumonia It presented with fever , night sweating , cough , Anorexia , & weight loss , of several weeks to months .

Cryptogenic Eosinophilic Pneumonia (Idiopathic E. P. ) It is presented with malaise , fever, SOB , & dry cough . CXR shows bilateral ,peripheral & predominantly upper lobes . Peripheral eosinophilia ( very high ) , high ESR , & total IgE ,BAL high eosinophils . It responses to prednisolone 20-40 mg /d shows dramatic response.

Hypereosinophilic Syndrome It is characterized by the presence of eosinophilia ( > 1500 / ul ) for > 6 months , associated with signs & symptoms of multisystem organ dysfunction. The heart may involved with tricuspid valve abnormality or endomyocardial fibrosis & res - trictive , biventricular CMP . Other organs include the lung , liver , spleen , skin , & CNS . It associated with T- cell lymphoma . Treatment : Corticosteroid + hydroxyurea .

Charg -Strauss syndrome (CSS) It is a multisystemic vasculitic disorder that frequently involves the skin , kidney, & nervous system in addition to the lung . Bronchial asthma almost found. Fever& eosinophilia usually present at time asthma ease the illness may be fulminating unless treated. Leukotriene - modifying agents has been associated with CSS .

Churg -Strauss Syndrome

Lung Diseases Due to Organic Dusts Hypersensitivity Pneumonitis (HP) ( Extrinsic allergic alveolitis ): HP result from inhalation of certain types of organic dust which rise to a diffuse immune reaction in the wall of the alveoli & bronchioles . -Farmer lung is the term commonly used for HP due to inhalation of antigen present in moldy hay , such as thermophilic actinomyces , Micropolyspora faeni , & Aspergillus's species. -HP first described in 1874 .

Lung Disease Caused by Organic Dust

Clinical Features Of HP 1. Acute :After few hours of re-exposure to the dust , the patient complains flu- like symptoms ( fever , headache , myalgia , malaise ,dry cough & SOB. Chest exam.: end - inspiratory crackles 2. Subacute : Over a period of weeks marked by cough & SOB & may progress to cyanosis . 3. Chronic : due to chronic low level of exposure . The onset is often insidious with progressive SOB if undiagnosed the disease may progress to cause severe respiratory disability , hypoxemia , pul. hypertension , cor pulmonale. Clubbing of finger may be present.

Investigations CXR : diffuse micronodular shadowing (upper ) HRCT : in acute … bilateral areas of consolidation . PFT : restrictive , low DLco , low Pa O2 ,& low PaCO2 . Other : high ESR , CRP , RF , …. BAL : marked lymphocytic alveolitis . Provocative test Lung biopsy .

Predictive factors in the identification of HP Exposure to a known antigen . Positive precipitating Ab . Recurrent episodes of symotoms . Inspiratory crackles . Symptoms occurring 4-8 hs after exposure . Weight loss .

Management of HP 1. Avoid exposure ( in some difficult to achieved ) Or using dust masks with appropriate filters may minimize exposure . 2. In acute cases prednisolone 40 mg /d for 3-4 weeks. 3. Treatment of underlying complications e.g. Oxygen therapy for hypoxia .

Drugs Induced Respiratory Diseases

It is caused by cotton dust inhalation . Byssinosis It is caused by cotton dust inhalation . It causes interstitial infiltration ( Monday fever ) . & acute bronchiolitis with generalized airway obstructions like asthma . CXR usually normal . Smoking aggravates it.

Lung Diseases Due To Inorganic Dust In certain occupations , theinhalation of inorganic dust , fumes , or other noxious substances may give rise to specific pathological changes in the lungs. Pneumoconiosis a diffuse pulmonary fibrosis due to prolonged exposure to inorganic dusts . It is an occupational hazard , therfore a detailed ocupational history is essential .e.g. Coal worker's pneumoconiosis ; Asbestosis ; Silicosis .

Coal Worker's Pneumoconiosis Prolonged inhalation of coal dust overwhelms the alveolar macrophages , which aggregate to form Macules in or near the centre of the secondary pulmonary lobules ,& a fibrotic reaction ensues. 1. Simple coal worker's pneumoconiosis( SCWP): This can be divided into 3 categories on CXR. However, it does not cause symptoms or abnormal PFT .

2. Progressive massive fibrosis ( PMF): ( complicated pneumoconiosis) Large dense masses mainly in the upper lobes , Cavitations may occur & must be differentiated from TB ; carcinomas ; Wagener's disease . PMF is associated with cough , sputum ( may be black , melanoptysis ) , & SOB . This may progress to RF , RVF even after cessation of exposure .

Caplan's syndrome :rheumatoid arthritis & rounded fibrotic nodules 0 Caplan's syndrome :rheumatoid arthritis & rounded fibrotic nodules 0.5- 5 cm in diameters, these are mainly in the periphery of the lung fields. Pathologically similar to rheumatoid nodule including central necrosis . It may occur in other types of pneumoconiosis .

Silicosis It results from the inhalation of crystalline or free silica , usually in the form of quartz . Similar to coal worker's pneumoconiosis , with multiple 3-5 mm nodular opacities predominantly in the mid & upper zones & may coalesce . Enlargement of the hilar glands with an egg-shell pattern of calcifications . Patient may at increased risk of TB ( silicotuberculosis ). Silica is a highly fibrogenic & the disease is usually progressive ( even when exposure ceases) . Acute disease similar to alveolar proteinosis after intense exposure to very fine crystalline silica dust.

Asbestosis ( cont.) 3. Asbestosis : Pulmonary fibrosis ---SOB , finger clubbing , similar to ILD. CXR usually confined to the lower 2/3 . Honeycombing with shaggy cardiac shadow .The condition seldom develops in less than 20 years after exposure. Complication ; RF ,cor pul. Dx may need biopsy . 4.Bronchial carcinoma : 10 time higher risk , & more in smokers. Also laryngeal carcinoma . 5. Mesothelioma of the pleura : It may occur with trivial exposure to blue asbestos . Chest pain , & bloody effusion , Dx by biopsy .