A 30-year-old, human immunodeficiency (HIV) negative woman with no past medical history presented to the obstetrics clinic in 2014 for an antenatal evaluation. At this time, she was found to have a positive tuberculin skin test and was referred to the tuberculosis clinic for possible treatment. Further workup included a chest radiograph showing right upper and lower zone nodular lung disease necessitating sputum collection for microbial isolation and evaluation (Fig. 1). Induced sputum cultures returned positive for MAC. Given her pregnancy, treatment for latent tuberculosis was deferred and she was subsequently referred to her primary care for follow-up with the instruction that pulmonary follow-up would be necessary post-partum for continued follow up of her MAC infection.
Her postnatal follow-up was erratic and over subsequent years, she received multiple courses of macrolides for sinus infections, broad spectrum antibiotic treatment for "pneumonia" and at times guideline-based therapy (GBT) for MAC with Rifampin, Ethambutol and Clarithromycin through various clinics and specialists. She remained culture positive for MAC during these clinical encounters and confirmed that she was not adherent to her treatment regimen during this time.
In December 2019, she was hospitalized with fever and cough productive of copious purulent sputum. She appeared malnourished and dyspneic. Vital signs revealed tachycardia, BP 91/45 mm Hg, RR 32/min with 94% oxygen saturation at rest. Lung exam demonstrated decreased breath sounds with transmitted bronchial breathing on the right side. Chest X-ray and CT Scan showed destruction of right lung parenchyma, volume loss, right sided air-fluid level and BPF. A large cavity was also seen in the left lung (Fig. 2A,B,2C).
Pleural fluid analysis revealed WBC count at 1.05 x 106/microliters with 100% lymphocytes and LDH at 1 x 105 U/L, glucose 37 mg/dl and protein 2.8 g/dl. Multiple sputum and pleural fluid cultures showed growth of Pseudomonas aeruginosa, macrolide resistance MAC with varying minimal inhibitory concentrations (MIC) to Amikacin. Serologic tests for expanded ANA were negative and Immunoglobulins were within normal range. A1-AT levels were normal. Repeat HIV testing and CFTR screen was negative.
The patient completed a six-week course of parenteral anti-pseudomonas antibiotics and subsequently continued on parenteral Amikacin along with Rifampin, Ethambutol, Clofazimine and Linezolid as long term treatment for macrolide resistant MAC. Chest tube drainage was also concurrently initiated.
CT surgery was planned for long-term surgical drainage with Eloesser flap, but this had to be deferred initially due to patient's poor clinical condition at that time and later due to her reluctance to have this procedure. Meantime, chest tube drainage was continued at home with regular interventional radiology review. On-going multi-drug antibiotic treatment regimen was subsequently supplemented with Amikacin liposomal inhaled solution (ALIS).