A 43-year-old woman presented to the emergency department with cough, weight loss and progressive difficulty with breathing of 2 months. Cough was insidious in onset, productive of copious thick whitish sputum, which was not foul-smelling but worse early in the morning. There was a history of fever, but no hemoptysis, drenching night sweats or contact with persons with chronic cough. At about the same period, she developed difficulty with breathing which was gradual in onset, provoked by ordinary activities such as walking and doing house chores. Difficulty with breathing progressively worsened, and became present even at rest which made her present at the emergency department. There was associated easy fatigability and orthopnea, but no Paroxysmal Nocturnal Dyspnea. She had bilateral leg and abdominal swelling, but no swelling in other parts of the body and no change in urine volume or frequency. She also complained of unintentional weight loss. Prior to the current bout of symptoms, she has been having recurrent cough for 22 years and had received four courses of anti-tuberculosis medications in the past;1st treatment in 1996, 2nd treatment in 2001, 3rd treatment in 2007 and 4th treatment in 2013. The basis of pulmonary Tuberculosis (PTB) diagnosis could not be ascertained in all cases and she did not complete 6 months of treatment in at least 2 of the courses. There is history of use of firewood for cooking for about 25 years, but no history of cigarette-smoking. She is not diabetic or hypertensive. No history of chronic use of immunosuppressives, exposure to asbestos, sharing of sharps, multiple sexual partners or blood transfusion. She is not a known asthmatic and no history of atopy. No history of recurrent joint pains, skin rash, mucosal sores, recurrent sinusitis or symptoms suggestive of malabsorption. No history of recurrent childhood upper respiratory tract infections. Immunization history could not be ascertained. She does not take alcohol. She is a petty trader and has been divorced for over 5 years due to recurrent ill health and has 4 children. Over the years, she has been patronizing patent medicine dealers, and has been to several hospitals where she had repeated chest x-rays and been on medications including courses of antibiotics with short lived clinical improvement. She also had several courses of antibiotics in the current illness with no improvement prior to presentation. At presentation, she was ill-looking, in respiratory distress, not pale, febrile (38.2 C.), anicteric, cyanosed, not dehydrated, no asterexis, no peripheral lymphadenopathy, had grade 1 finger clubbing and bilateral pitting pedal edema up to the knee. SpO2 was 84%, weight was 47kg, and height was 1.65m with BMI of 17.3kg /m2.
Chest examinations revealed respiratory rate of 32cpm. Modified medical research council (MMRC) dyspnea scale was 4. Other findings were bibasal coarse crepitations and left lower lobe consolidation. Cardiovascular examination revealed pulse rate of 110 bpm with regular rate and rhythm, Blood pressure 120/70 mmHg, elevated Jugular venous pressure with distended neck veins, Apex beat is displaced laterally with left parasternal heave. She has a third heart sound with loud P2 and pansystolic murmur loudest in the tricuspid area. There was tender hepatomegaly but neurological and musculoskeletal examinations were normal. The most recent Chest X-ray (CXR) done three days prior to presentation showed reticulonodular opacities, cystic lesions especially in the mid to lower zones, moderate cardiomegaly with mild vascular engorgement with perivascular cuffing (Figure 1). Differentials at presentation were PTB complicated by cor pulmonale with superimposed bacterial infection, Post-TB Bronchiectasis, Chronic Obstructive Pulmonary Disease (COPD), Congestive heart failure secondary to valvular heart disease and Interstitial lung disease. Patient was admitted and various investigations were ordered. She was nursed in cardiac position and the following treatments were commenced; Intranasal oxygen at 5L/min, intravenous (IV) Frusemide 60mg daily, Tab Spironolactone 25mg daily, IV Augmentin 1.2g 12 hourly, Tab clarithromycin 500mg bd and chest physiotherapy. Chest Computerized Tomography (CT) scan revealed lung fields showing mosaic pattern with patchy areas of ground glass attenuation. Multiple poorly defined nodules of varying sizes and shapes are seen in a random distribution in all the lung segments with upper lobe predominance. Conferencing nodules are seen in the apical segment of the right lung, apicoposterior segement of left lung with extension into the oblique fissure and lateral aspect of superior lingular segment. Generalized cylindrical, varicoid and cystic bronchiectatic changes with signet ring sign is seen in both lung fields but worse at the right middle lobe and left lingular segments. There is thickening of the intralobular septae and the walls of the dilated bronchi. The impression was Bronchiectasis with mosaic perfusion and randomly distributed pulmonary nodules most likely to Post infectious (Figure 2, Figure 3, Figure 4).
Sputum studies microscopy, culture and sensitivity yielded growth of upper respiratory flora; AFB and GeneXpert were negative for MTB. Blood culture yielded no growth. Fingerstick glucose was 6.6mmol/L at presentation. Basal metabolic panel was within normal limit (HCO3: 28 mmol/L, Na:128 mmol/L, K: 3.5 mmol/L, Urea: 2.4 mmol/L, Cr:81 umol/L). FBC revealed PCV: 42%; WBC: 12,000 cells/mm3, Platelets: 372,000 cells/mm3, Neutrophil: 84%, Lymphocytes: 44%, Eosinophils: 02%. ESR was 33mm/hr (westergren method). ECG showed Sinus tachycardia and Right axis deviation. Echocardiography revealed Right heart disease. Anti-nuclear antibody and HIV screening were both negative. Serum proteins were within normal limits. Spirometry revealed obstructive pattern (Table 1). Based on the Chest CT scan findings and result of other investigations, a diagnosis of Bronchiectasis probably post-PTB or secondary to poorly treated pneumonia, with infective exacerbation, complicated by corpulmonale was made. Nebulized salbutamol was added to her current treatment regimen. After 6 days on admission, patients condition deteriorated evidenced by persistent fever, respiratory distress, confusion and hypoxia (SpO2 82%) and was considered for Intensive care unit admission, bronchoscopic sampling of the lower respiratory tract, while IV Augmentin was changed to IV Cefepim. The relatives however, objected to further treatment due to financial constraint and was subsequently discharged against medical advice. She was also advised to get multivalent pneumococcal polyvalent vaccine at the nearest primary health care center upon discharge.
Chest CT scan showing Signet ring appearance in both lung fields in the upper lobes.