Bronchial purging: Atypical pulmonary sarcoidosis presenting with bronchorrhea.

Sir,

Sarcoidosis is a granulomatous disorder of unknown etiology with a multisystem involvement, and the most common involved organ is lung. Due to multisystem involvement, atypical presentations are common and varied in sarcoidosis; however, a patient of sarcoidosis presenting with bronchorrhea has not yet been reported in literature. We present a case of a 45-year-old male, a smoker (smoking index 1 pack year) and with no comorbidities, who came with complaints of cough and breathlessness (Modified Medical Research Council Grade II) with bronchorrhea (>100 ml/day) for 1 year and weight loss for the last 4 months. Clinical examination was insignificant. Chest X-ray is shown in Figure 1. Laboratory parameters were within normal limits. He was on empirical antituberculosis treatment (4 drugs-RHZE) for 1 month from outside with no relief. Sputum cytology was negative for any malignancy, acid fast bacilli smear and gene expert were also negative. Keeping in view of malignancy as the most common cause of bronchorrhea, positron emission tomography - computed tomography (PET-CT) imaging was done which revealed metabolically active soft-tissue dense fibrous lesion with peribronchovascular thickening in bilateral upper lobes and in the right middle lobe along with metabolically active multiple mediastinal lymph nodes [Figure 2]. Spirometry showed (moderate airway obstruction with no reversibility) forced expiratory volume 1/forced vital capacity (FEV1/FVC) ratio of 65.8% with FVC pre/post: 3.40 L/3.62 L (77%/82% of predicted normal), FEV1 pre/post: 2.24 L/2.49 L (62%/69% of predicted normal) with absolute increase of 250 ml and 11%. Videobronchoscopy showed diffuse endobronchial nodules. Bronchoalveolar lavage lymphocytes were 25% and CD4/CD8 = 3.36.

Figure 1

X-ray chest showing the bilateral hilar prominence with homogeneous infiltrates in the left side mid and lower zones

Figure 2

Positron emission tomography-computed tomography images showing fludeoxyglucose uptake in lesions in bilateral upper lobe and right middle lobe with mediastinal lymphadenopathy

Endobronchial cryobiopsy, transbronchial lung biopsy and endobronchial ultrasound from lymph nodes stations right paratracheal (4R) and subcarinal (7) were done and showed compact, discrete, multiple, noncaseating granulomas [Figure 3] in all tissues sampled (lung, lymph node, and endobronchial mucosa). Further investigations revealed serum angiotensin-converting enzyme of 107 U/L (normal value 8–52 U/L). Mantoux test and interferon-gamma release assays were negative. The case was further discussed in multi disciplinary meeting (MDD) with radiologist and pathologist in view of atypical clinical. typical radiological and pathological findings. Judson et al.[1] have stratified confidence level for the diagnosis of sarcoidosis – highly probable, probable, possible, and unlikely based on clinicoradiological and pathological description. Alternative diagnosis of infections and malignancy were ruled out, and diagnosis of sarcoidosis was made with highly probabale level of confidence and the patient was started on steroids (Tablet deflazacort 6 mg twice daily), on which he showed marked improvement.

Figure 3

Pathological samples from lymph nodes, endobronchial mucosa, and lung parenchyma showing noncaseating granuloma

We did extensive search on PubMed, Medline, Google Scholar, and EMBASE; however, we could not find any case of sarcoidosis presenting with bronchorrhea which is defined as the production of more than 100 ml of watery sputum per day and is mostly associated with bronchioloalveolar cell carcinoma (mucinous type), rarely reported with nonmalignant processes such as chronic bronchitis, asthma, tuberculosis, and scorpion stings. Although the exact pathophysiology of bronchorrhea is unknown, Takeyama et al.[2] suggested the stimulation of epidermal growth factor receptor causing MUC5AC expression in airway epithelial cells while inflammatory stimuli, increased transepithelial chloride secretion and excessive transudation of plasma proteins[3] are the other possible mechanisms.

Bronchorrhea leads to significant morbidity by affecting the quality of life as in our patient too. Homma et al.,[4] in 1975, reported bronchorrhea in alveolar cell carcinoma with dehydration, electrolyte imbalance, and respiratory failure. Various drugs have been tried to treat bronchorrhea. Table 1 exemplifies unmet need in its treatment as many drugs have been tried. Our patient improved well with low-dose steroids.

Table 1

Various drugs used for bronchorrea cases by multiple authors

A diagnosis of sarcoidosis is as such difficult in India even in typical cases but is arduous in atypical cases. With increasing awareness of the disease, we will see further increase in cases of sarcoidosis with both typical and atypical presentation.[10] Our case highlights an emergent need for algorithmic approach incorporating various clinical, radiological, pathological, and biochemical markers to make a confident diagnosis of sarcoidosis.

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Conflicts of interest

There are no conflicts of interest.