Skip to main content

Table 3 Key clinical information of patients

From: Multidisciplinary approach to connective tissue disease (CTD) related pleural effusions: a four-year retrospective evaluation

Connective tissue disease diagnosis

Presenting symptoms / Autoimmune serology

Evidence for diagnosis*

Pleural fluid glucose (mmol/L)

Pleural fluid LDH (IU/L)

Pleural fluid protein (g/L)

Pleural micro

Pleural fluid appearance

Pleural fluid cytology

Histology

CT chest findings

Outcome

Rheumatoid arthritis

Pleuritic chest pain, dyspnoea, polyarthralgia, fatigue anti-CCP + ve

Clinical assessment, autoimmune serology

3.4

794

55

Fluid MCS/AFB neg

Biopsy AFB neg

Turbid yellow fluid

2/3/16 Neutrophils, lymphocytes, macrophages, prominent eosinophils. Few reactive mesothelial cells.

23/3/16 Mixed lymphoid cell population; plasma cells and eosinophils. Occasional mesothelial cells.

Pleural biopsy: moderate acute and mild chronic inflammation, surface mesothelial denudation, macrophages and fibrin.

Bilateral hilar lymphadenopathy. Left-sided pleural effusion and bibasal groundglass change with intralobular septal thickening demonstrated.

Resolution of pleural effusions on CXR by week 7 on weaning prednisolone (after pulsed methylprednisolone)

Rheumatoid arthritis

Fever, cough, fatigue, pleuritic chest pain, dyspnoea anti-CCP and RhF + ve

Previous CTD diagnosis, clinical assessment, autoimmune serology

None

None

None

n/a

n/a

None

None

Pericardial hyperattenuation, either in keeping with thickening and low-grade enhancement or a complex pericardial effusion. Shallow left pleural effusion.

Resolution of pleural effusions 5 months after pleural review (one prior week of prednisolone)

Rheumatoid arthritis

Cough, fever

anti-CCP and RhF + ve

Previous CTD diagnosis, clinical assessment, pleural fluid glucose, pleural biopsy

0

5947

55

Fluid MCS/AFB neg

Biopsy AFB neg

Turbid yellow fluid

Lymphocytes and macrophages and scattered histiocytic multi-nucleate giant cells

Pleural biopsy: fibrous tissue with a dense lymphoplasmacytic infiltrate. Suggestion of histiocyte palisading; possibility of rheumatoid nodule, but insufficient for definitive diagnosis.

Moderate right pleural effusion. A calcified granuloma is noted in the right lower lobe. Minor right subpleural nodularity is seen. Emphysema.

Pleural effusion stable 1 year after discharge from pleural clinic (during which 5 mg prednisolone daily started for joint pains)

IgG4-related disease

Dyspnoea, cough, oedema

IgG4 2.71 (0.23–1.11 g/L)

IgG1 11.3 (4.8–9.5 g/L)

Pericardiectomy histology, autoimmune serology

None

None

None

Fluid MCS/AFB neg

Biopsy AFB/MCS neg

Bloodstained fluid

A few benign mesothelial cells

(Pleural biopsy: mildly inflamed pleural tissue. Single non-necrotising granuloma.)

Pericardial biopsy: dense keloid-like fibrosis. Foci of chronic inflammation and in these IgG4 plasma cells comprise a high population of IgG+ plasma cells.

Right pleural effusion and ascites. Cervical and mediastinal lymphadenopathy.

Pericardial calcification and thickening suggest previous pericardial effusion/pericarditis.

Pleural effusion improved over 2 months (prednisolone started then weaned off onto methotrexate)

IgG4-related disease

Dyspnoea, dizziness

IgG4 1.53 (0.23–1.11 g/L)

IgG1 12.4 (4.8–9.5 g/L)

Pericardiectomy histology, clinical assessment, autoimmune serology

None

27

6

Fluid Scanty growth Staphylococcus aureus, AFB neg

Slightly cloudy yellow fluid

Mesothelial cells and mixed inflammatory cells (predominantly neutrophil polymorphs).

Pericardial biopsy: Diffuse moderate to severe fibrosis associated with focal calcification and mild chronic inflammatory cell infiltrate. IgA shows non-specific patchy mild staining.

Small bilateral pleural effusions. No pericardial effusion although some pericardial calcification is noted. Likely reactive mediastinal nodes.

Left pleural effusion resolved and small right pleural effusion 1 year after pleural review (started on furosemide)

Adult Still’s disease

Fever, cough, dyspnoea, chest pain, myalgia, sore throat, rash

Clinical assessment, raised ferritin

10.3

894

33

Fluid MCS/AFB neg

Straw coloured fluid

Acute inflmmatory cells. A few reactive mesothelial cells, histiocytes and lymphocytes.

None

Small bilateral pleural effusions with bibasal collapse/consolidation. Moderate pericardial effusion.

Pleural effusions resolved after 1 month (weaning prednisolone and colchicine)

Adult Still’s disease

Pleuritic chest pain, dyspnoea, fever, sore throat, arthralgia

Clinical assessment

None

None

34

Fluid MCS/AFB neg

Cloudy yellow fluid

Numerous neutrophils (95%) with occasional macrophages and mesothelial cells.

None

Small bilateral pleural effusions and a trace of pericardial fluid.

Pleural effusions resolved after 2 months (weaning prednisolone and colchicine)

Vasculitis

Dyspnoea, weight loss, appetite loss

P-ANCA, Anti-MPO 222 (0-10 U/ml)

Clinical assessment, autoimmune serology, CT showing adrenal infarcts

6.9

279

48

Fluid MCS/AFB neg

Bloodstained fluid

Mixed lymphoid cell population along with neutrophil polymorphs, histiocytes and very occasional mesothelial cell.

none

Bilateral calcified pleural plaques. Right pleural thickening and effusion. Emphysema. Multiple solid subpleural and peri-fissural bilateral nodules

Pleural effusion resolved after 5 months (4 month course of weaning prednisolone)

SLE

Pleuritic chest pain, dyspnoea

Anti ds-DNA 415 (0-10 IU/ml), anti-Smith +ve

Previous CTD diagnosis, clinical assessment

5.2

304

59

Fluid MCS/AFB neg

Cloudy light brown fluid

Mesothelial cells, macrophages, neutrophils and lymphocytes.

none

None

Pleural effusion resolved after 7 months (prednisolone weaned to maintenance)

Drug-induced lupus (cabamazepine)

Dyspnoea, rash, ulceration of hands and feet

ANA +ve, ENA RNP + ve, ENA SSA (Ro) + ve, ENA Sm + ve

ANCA neg

Clinical assessment, autoimmune serology

6.5

228

47

Fluid MCS/AFB neg

Biopsy AFB/MCS neg

Turbid orange fluid

16/11/12 Reactive mesothelial cells, polymorphs, lymphocytes and histiocytes.

5/12/12 Numerous mesothelial cells, macrophages and a mixed population of lymphocytes.

11/12/12 Histiocytes, a few lymphocytes and reactive mesothelial cells.

18/12/12 Reactive mesothelial cells, lymphocytes and histiocytes.

27/12/12 Histiocytes, lymphocytes, reactive mesothelial cells, neutrophils, plasma cells.

13/1/13 Blood only.

16/11/12 pleural biopsy: Single focus of large cells at the edge of one fragment, most likely reactive mesothelial cells. Nuclei are pleomorphic, irregular nuclear membranes and nuclear chromatin clearing.

13/1/13 pleural biopsy: Pleura markedly oedematous; granulation tissue at the surface, scattered foci of chronic inflammatory cells.

Left pleural effusion with minimal nodularity and mild volume loss in the left hemithorax. Shallow pericardial effusion. Widespread mild lymphadenopathy.

Pleural effusion resolved after 9 months (prednisolone weaned to maintenance)

Behcet’s

Chest tightness

Clinical assessment

4.8

380

44

Fluid MCS/AFB neg

Turbid bloodstained fluid

Numerous lymphocytes, histiocytes and blood.

none

Large right main pulmonary artery thrombus. Multiple left sided pulmonary emboli. Bilateral pleural effusions with consolidaton/atelectasis. 2 small round pulmonary foci and a band of consolidation in the right upper lobe. Wedge-shaped consolidation in the middle and right lower lobes.

Pleural effusions resolved after 1 year (cyclophosphamide and weaning prednisolone)

  1. *These were the key factors used by the mutldisciplinary team in securing a diagnosis