Gujjar Lung: An Unusual Case Report and Systematic Review of Literature.

We report a 65 year old female patient who had presented with dry cough and shortness of breath for the last 5 years and had also received anti-tubercular therapy but without any benefit. Evaluation revealed the presence of obstructive airway disease with nodular opacities in bilateral lungs. Histopathological examination including electron microscopy was suggestive of domestically acquired pneumoconiosis.A diagnosis of Gujjar lung was made based on history of exposure to wood smoke, characteristic histological and radiological features. Anti-tubercular therapy was stopped and bronchodilators were initiated along with removal from source of exposure to which she showed significant improvement. We also did a systematic review of literature pertaining to Gujjar lung. Copyright:

INTRODUCTION

Gujjar lung, is a form of domestic pneumoconiosis caused by deposition of particulate matter of biomass combustion in households.[1] The entity “ Gujjar lung” was first introduced in 1991 by Dhar and Pathania from Kashmir when they noticed miliary mottling and reticulonodular pattern in the chest radiographs of patients belonging to Gujjar community.[1] Since then 4 case reports and a case series of 25 women has been reported in medical literature wordwide. The disease is typically characterized by progressive cough and shortness of breath and histopathological evidence of anthracotic nodules with carbonladen macrophages and fibrosis. As this entity is less commonly identified, these patients are being empirically put on therapeutic trials with antituberculosis treatment, with no improvement in symptoms, adding to the morbidity.

CASE REPORT

A 65-year-old female patient, resident of Uttaranchal, presented to our hospital with complaints of cough with scanty expectoration for the past 5 years along with shortness of breath for the past 2 years. She gave history of significant exposure to wood smoke from fire place for almost her entire lifetime. The expectoration was mucoid in nature without any offensive smell and was partially relieved on taking cough syrups prescribed by local doctors. The shortness of breath was gradual in onset, progressive (from Modified medical research council scle for dyspnea (mMRC) grade 1 to 4) with no particular periodicity or diurnal variation, no wheeze, and partially relieved by cough syrups. The patient complained around 5 kg of weight loss in the past 5 years but without anorexia or other complaints including fever. She was also evaluated at a local medical facility for her ailment. While her blood tests were normal, her chest X-ray (CXR) reportedly had nodular opacities on the basis of which she received antitubercular therapy (ATT) for around 2 years. But there was no relief of her symptoms even after completing the course of ATT.

She was subsequently referred to our hospital where thorough clinical examination and relevant investigations were planned. Her Chest X-Ray was suggestive of hyperinflation with nodular opacities more prominent in the lower zones of both the lungs [Figure 1]. Spirometry revealed features of obstructive airway disease (FEV1 42% predicted, FVC 60% predicted, FEV1/FVC 55). A subsequent high-resolution computed tomography of thorax showed emphysematous changes (more in the lower lobes) along with centrilobular nodules [Figure 2]. Fiber-optic bronchoscopy with bronchoalveolar lavage (BAL) and transbronchial lung biopsy were done. Her BAL fluid showed normal cellular pattern with negative bacterial and fungal stain. The histopathology showed mild infiltration of lung parenchyma by chronic inflammatory cells including lymphocytes with focal type II pneumocyte hyperplasia. There were areas of fibrosis with increased carbon pigment deposition and carbon-laden macrophages forming anthracotic nodules [Figure 3]. Scanning electron microscopy of the biopsy specimen revealed carbonaceous particles in alveolar macrophages [Figure 4]. On the basis of history, radiological, and histopathological features, a diagnosis of “Gujjar lung” was made. She was treated with bronchodilators (long-acting bronchodilators and anticholinergics) and also told to avoid further exposure to wood smoke. She responded to the above treatment with gradual improvement of her symptoms. At one month of follow-up, her shortness of breath had decreased to mMRC grade 2 with significant improvement of her cough.

Figure 1

Chest X-ray showing bilateral diffuse pulmonary nodules

Figure 2

CT thorax showing bilateral centrilobular nodules predominant in the lower lobes

Figure 3

Transbronchial lung biopsy showing increased deposition of carbon pigment in the interstitium

Figure 4

Scanning electron microscopy shows an alveolar macrophage (a) whose cytoplasm contains carbonaceous particles (arrowhead) along with oxalate crystals (arrow) (b)

DISCUSSION

Gujjar lung, a type of “hut lung” refers to a form of domestic pneumoconiosis caused due to inhalation of smoke and deposition of particulate matter from combustion of biomass fuel in ill-ventilated households.[1] Gujjar is a community of people inhabiting hilly regions of Indian subcontinent (Jammu Kashmir, Himachal Pradesh, Rajasthan, Pakistan, Tibet, etc.). They normally reside in ill-ventilated mud houses which commonly house the “chullah” or oven which is used to burn biomass. Biomass commonly involves wood, cow dungs, dried grasses, and so on which are used typically to cook food, for home-heating, or lighting and are typically burnt for 12–16 h a day.[2] Thus, the residents of these dwellings are exposed to high level of indoor pollution typically leading to the disease of “Gujjar lung.” The disease is typically characterized by progressive cough and shortness of breath, onset beyond the fourth decade, radiological features of reticulonodular infiltrates, and histopathological evidence of anthracotic nodules with carbon-laden macrophages and fibrosis.[2] The term “Gujjar lung” was coined in 1991 by Dhar et al.[3]

Grobbelaar et al. had coined the term “hut lung” after studying 25 Trankei women in Africa who had developed pneumoconiosis following exposure to mainly dust and smoke from biomass-fuelled fires.[4] In developing nations, more than 50% of households are dependent on such forms of energy as against cleaner form of fuels like oil or natural gas mainly due to financial constraints.[5] The chronic exposure to smoke in the affected individuals might result in a number of changes including obstructive airway diseases, respiratory tract infection, lung malignancy, pneumoconiosis, and so on.

The content of the biomass smoke has been studied extensively and found to contain substances such as carbon monoxide, nitrogen dioxide, sulfur dioxide, particulate matter, polycyclic aromatic hydrocarbons, volatile organic compounds, and particulate matter. Soot refers to the black powdery substance consisting mainly of partially combusted hydrocarbons in fuels consisting mainly of amorphous carbon. The incidence of pneumoconiosis may be as high as 22.5% as seen by Saiyed et al. in 449 subjects of three villages in Ladakh.[6] In the same series, it was reported that in patients with pneumoconiosis, a majority (91.5%) had small opacities less than 10 mm. The radiological findings range from regular nodular lesions to massive fibrosis. Histopathological examination is usually diagnostic consisting of anthracosis with or without macule formation as well as fibrosis to a variable degree. The lung function may range from variable to restrictive, obstructive, and mixed ventilator defects. The treatment of the condition usually involves further avoidance of exposure and treatment of underlying airway obstruction in the line of chronic obstructive pulmonary disease. Although there are a few histopathologically proven case reports of “Gujjar lung,” electron microscopic evaluation has been reported only in one prior report.[7]

We also did a systematic review of literature in MEDLINE database using the term “Gujjar lung” for all type of articles without any specific restriction. We identified five articles and included the details in an table [Table 1]. The patients were from Kashmir belonging to the Gujjar community in two case reports, while similar cases were also reported in rural women of Transkie district in Africa, Bhutan, and Pakistan. Most of the patients were females as they were more exposed to household biomass smoke. There was one male patient who was a baker exposed to smoke for 6 years.[8] Diagnosis in most of the cases was made by clinical, radiological, physiological, and histopathological findings, although there were no definite diagnostic criteria. Most of the patients had chronic cough with shortness of breath as their chief complaints. Spirometry showed obstructive pattern in many patients, while normal and restrictive patterns were also observed in few cases.[4] Histopathological features were documented in all case reports which showed mild to moderate fibrogenic reaction with carbon nodules and interspersed collagen fibers. Macrophages and giant cells laden with carbon particles were also observed. Electron microscopic study was done in one case (apart from ours) which showed carbonaceous particles, silica, aluminum silicates, and a few metallic particles containing iron or titanium.[7] The treatment has been primarily symptomatic with inhaled bronchodilators and/or steroids.[7]

Table 1

Systematic review of literaturein MEDLINE database using the term “Gujjar lung”

Author	Journal	Year of publication	Age (years)	Sex	Symptoms	Duration of exposure	Imaging features	Bronchoscopy	Lung biopsy done or not	Biopsy report	PFT	Treatment received	Follow-up
Sanjay Mukhopadhyay et al.5	Chest	2013	60	F	Progressive dyspnea over 3 years, productive cough with blood streaked sputum	45 years	CT imaging showed bilateral tiny centrilobular upper lobe ground-glass nodules	Cytology showed numerous dust-filled macrophages	Yes	Heavy deposition of black dust within the interstitium accompanied by mild interstitial fibrosis, particle types identified were carbonaceous particles, silica, aluminum silicates, and a few metallic particles containing iron or titanium	FEV 1:FVC ratio of 63%, with total lung capacity of 3.29 L (95%)	Inhaled budesonide/formoterol	Remained dyspneic at 5 months of follow-up
Sunil Vallurupalli et al.9	BMJ	2013	68	Female	Chronic dyspnoea on exertion associated with a nonproductive cough	30 years	Multiple bilateral pulmonary nodules in a perilymphatic distribution associated with hilar and mediastinal lymphadenopathy	Extensive anthracotic pigmentation of the tracheobronchial mucosa	yes	Ill-defined nodules in a perivascular subpleural deposition, carbon pigment deposition around the terminal bronchioles, dust macules and negatively birefringent needles on polarized light microscopy consistent with mixed dust pneumoconiosis	Not stated	Not stated	Not stated
G Hassan, GQ Khan, Waseem Qureshi8	JK SCIENCE	2006	60	Male	Progressive dyspnea, cough with mucoid sputum	Since early age	Chest radiograph revealed reticulo nodular shadows on left involving mid and lower zones. On the right sight there were more dense mass shadows, with sparing of apices.(HRCT) shows bilateral reticulo nodular shadows and fibrous septa on both sides	Anthracotic staining of right middle and lower lobe bronchi, and bronchoalveolar lavage showed predominantly macrophages laden with carbon pigment	Yes	Clumps of carbon-laden macrophages and anthracotic nodules	Obstructive pattern with FEV1/FVC ratio of 56%	Not mentioned	Not mentioned
Raison et al.6	Clinical Radiology	2000	31	Male	Intermittent mild productive cough	Not stated	Multiple centrilobular nodules which in bilateral and diffuse	Scattered anthracotic spots and linear mucosal streaks but no other significant endobronchial lesions	Yes	Anthracotic nodules (2–3 mm diameter) were seen surrounding blood vessels, with associated perifocal emphysematous changes	Small airway obstruction	Not stated	Not stated
J P Grobbelaar et al.2	Thorax	1991	43 (20–84)	Females	Mild acute respiratory tract symptoms	Not stated	Diffuse fine rounded regular nodulation to coarser irregular nodules to extensive fibrosis	Yes, large proportion of the alveolar macrophages were heavily laden with inorganic inclusions	Yes	1. Carbon pigment deposited within the septal and perivascular areas and terminal bronchiole 2.Carbon pigmentation with focal collections of dust laden macrophages occurring at-the division of respiratory bronchioles and within alveoli as well as extending into the peribronchiolar interstitium, with associated reticulin deposits 3.Mixed dust fibrosis	Obstructive pattern	Not stated	Not stated

PFT: Pulmonary function test; CT: computed tomography; HRCT: high-resolution computed tomography

CONCLUSION

”Gujjar lung” is a type of domestic pneumoconiosis commonly seen in an ethnic group residing in the hilly regions of Indian subcontinent. Patients with history of significant exposure to wood smoke often present with chronic respiratory complaints. Meticulous history taking, spirometry, and thoracic imaging can aid in the diagnosis. Besides, histopathological examination can provide tell-tale features substantiating the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.