PMC5925957_01
a) CXR and representative HRCT images taken at presentation, 9 months and 21 months and 6 months post right talc pleurodesis. (i) CXR at presentation demonstrates blunting of the costophrenic angles with scattered peribronchial thickening in the mid and lower zones. (ii) CXR 9 months later following a tapering course of prednisolone therapy and maintenance of 10mg daily, demonstrating blunting of the costophrenic angles with worsening of the right sided effusion. iii) CXR at 21 months following repeated trials of tapering prednisolone, attempted diuresis and trial of methotrexate therapy in conjunction with 10mg prednisolone: CXR demonstrates persistent pleural effusions with apparent worsening of both right and left effusions. iv) CXR 6 months' post pleurodesis on prednisolone 10mg once daily, hydroxychloroquine 200mg twice daily and azathioprine 150mg once daily. No reaccumulation of right effusion. b) Representative HRCT images taken at presentation and 9 months later: Soft tissue windows above and lung windows below of i) HRCT images taken at presentation demonstrating mediastinal and hilar nodal calcification, perilymphatic nodularity and bilateral pleural effusions, left larger than right. ii) Nine months later following a tapering course of prednisolone therapy and maintenance of 10mg daily. Stable mediastinal and hilar nodal calcification and perilymphatic nodularity with bilateral pleural effusions, worsening on the right. iii) Four weeks post right talc-pleurodesis. Worsening nodularity in the context of slight increase of left sided-effusion. iv) Six months after right-talc pleurodesis. Stable appearances of lung nodularity in the context of worsening left effusion on prednisolone, hydroxychloroquine and azathioprine. c) Histological analysis i) Endobronchial biopsy demonstrates non-caseating granuloma. ii) Right sided VATS pleural biopsy demonstrating pleural and iii) subpleural based non-caseating granulomatous inflammation with surrounding lymphocytic infiltration and accompanying fibrosis. iv) Concurrent VATS lung wedge biopsy demonstrates an additional interstitial sarcoidal granuloma. ZN and Wade-Fite stains were negative excluding tuberculosis. No malignancy was seen. Scale bar indicates 500 mum.
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Chest X-ray showed blunting of the costophrenic angles with scattered peribronchial thickening predominantly within the mid and lower zones (Fig. 1a). High resolution computed tomography (HRCT)-Chest demonstrated bilateral hilar and central mediastinal nodal calcification, in the absence of significant lymphadenopathy, with perilymphatic nodularity in the upper and mid zones of the lungs and bilateral pleural effusions, left larger than right (Fig. 1bi). Endobronchial biopsies (Fig. 1 ci) demonstrated discrete non-necrotising epithelioid granulomas consistent with sarcoidosis. HRCT demonstrated stable parenchymal appearances but worsening right effusion (Fig. 1 a and bii). Attempted diuresis, a repeat trial of tapering doses of prednisolone then introduction of methotrexate (12.5mg weekly) in conjunction with prednisolone 10mg had no significant benefit, with gradual worsening of bilateral effusions and symptoms over the subsequent 12 months (Fig. 1a). Pleural biopsies showed features of chronic pleuritis with associated granulomata (Fig. 1c ii-iii). The lung VATS biopsy sections (Fig. 1 c iv) showed pleural and subpleural granulomatous inflammation.
Laboratory investigations and pleural fluid analysis results. Abbreviations: ACE angiotensin-converting enzyme, ADA adenosine deaminase, NT-Pro-BNP N-terminal pro b-type natriuretic peptide.
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Laboratory investigations revealed a mildly elevated ACE level (summarised in Fig. 2). Left sided chest ultrasound demonstrated an anechoic effusion, with an exudative lymphocytic yellow aspirate (lymphocytes 70%), with a normal adenosine deaminase (ADA) level that was negative to TB culture (summarised in Fig. 2).