Brit.
J.
Dis.
Chest
(1973)
67,
146
RHEUMATOID LUNG DISEASE WITH PULMONARY FIBROSIS NECROBIOTIC NODULES AND PLEURAL EFFUSION U. Department
of Cardiology,
THADANI” Kingston
General
Hospital,
Hull
The association of pulmonary disease with rheumatoid arthritis is well recognized. The lung lesions usually follow but occasionally precede the joint manifestations. The subject has been fully reviewed by Rubin et al. (1967) Scadding ( r 969) ; Turner-Warwick ( 1969) and Sluis-Cremer (I 970). Among the lesions reported are interstitial pulmonary fibrosis or fibrosing alveolitis (Ellman & Ball I 948 ; Dixon & Ball 1957 ; Edge & Rickards 1957 ; Cruickshank 1959; Brannan et al. 1964; Scadding 1964, 1969; Turner-Warwick & Doniach 1g65), and pleurisy and pleural effusions, which are sometimes associated with pleural nodules and plaques (Baggenstoss & Rosenberg 1943; Emerson 1956; Horler & Thompson 1959; Lee et al. 1959; Walker & Wright 1967). Intrapulmonary necrobiotic (rheumatoid) nodules have only rarely been recorded (Gruenwald 1948 ; Raven et al. I g&3 ; Scadding I g6g), although these occur relatively frequently in association with a variety of pneumoconioses (Caplan 1953 ; Hayes & Posner rg6o ; Telleson 1961; Lindars & Davies 1967). These nodules may cavitate or ulcerate into the pleural cavity (Dumas et al. 1963 ; Davies 1966; Panettiere et al. 1968). Although these lesions have been shown to occur in various combinations (Ellman et al. 1954; Portner & Gracie 1966; Stengel et al. 1966; Rubin et al. 1967, Scadding rg6g), there remains some debate whether they are a direct manifestation of the rheumatoid process (Arnoff et al. 1955; Talbott & Calkins 1964; Stack & Grant 1965). Since a search of the literature has revealed only z cases of rheumatoid lung disease with the combination of pulmonary fibrosis, necrobiotic nodules and pleural effusions (Flatley 1959; Cudkowicz et al. 1961) it was thought worth while documenting a further case. Case Report A 6g-year-old man with previous good health developed an unproductive cough in February 1966. There was no history of exposure to irritating dusts or vapours. A chest radiograph revealed bilateral basal pulmonary fibrosis (Fig. I). The cough persisted and in July 1970 he developed a painful arthritis which involved the hands, wrists, ankles, elbows and knees. Later his shoulders, * Present
address:
Department
of Cardiology, (Receiued,for
publication,
The
General
Infirmary,
January1973)
Leeds
LSI
SEX.
RHEUMATOID
sternocostal therapy was Clinical phalangeal, subcutaneous
FIG.
LUNG
I47
DISEASE
joints and cervical spine were affected. Conventional analgesic ineffective and he entered hospital in August 1970. examination revealed fever, an acute polyarthritis (metacarpoproximal interphalangeal, wrists, elbows, ankle and knee joints), ulnar nodules, vasculitis of the elbows and nail beds with clubbing
I. Radiograph,
February
I 966,
showing bilateral basal on the left side
pulmonary
fibrosis,
more
marked
of the fingers. There was also evidence of a right pleural effusion and persistent fine crepitations were heard at the left base. The chest radiograph confirmed the right pleural effusion and showed ‘fibrotic’ changes in both lung fields (Fig. 2). The ESR was 103 mm/hour (Westergren) with a total white cell count of 14 ooo/mm3 (gy& oesinophils). The Rose Waaler titre was 1/40. The Latex fixation test and the serum antinuclear factor (titre 1/40) were also positive. Blood smears for LE cells were negative on 3 occasions. The pleural fluid was opaque and yellow and contained many polymorphs, lymphocytes and a moderate number of eosinophils. Bacteriological studies on the pleural fluid were negative. No malignant cells were found in either the sputum or the pleural fluid. A subsequent bronchoscopic examination was normal. Needle biopsy of
148
FIG.
2. Radiograph,
THADANI
September
3. Photomicrograph necr ‘otic tissue surrounded FIG.
1970,
showing
bilateral effusion
pulmonary
from lung biopsy specimen showing by partially palisading epitheloid cells. H. & E. x 40
fibrosis
necrobiotic cells and
and
nodule. chronic
a right
pleural
Note fil xinoid inflame matory
RHEUMATOID
LUNG
I49
DISEASE
the pleura revealed fibrosis. A barium meal and follow through examination was normal. The diagnosis of rheumatoid lung was considered likely, and prednisolone 40 mg daily was started on 14 September 1970, with rapid improvement of the arthritis and resolution of his fever. However, on 22 September he again became febrile, with clinical and radiographic evidence of an acute chest infection. This responded satisfactorily to ampicillin.
FIG.
4. Photomicrograph
from biopsy specimen. Note interstitial pulmonary fibrosis.
dense lymphocytic H. & E. x 160
infiltrate,
extensive
On 8 October a right diagnostic thoractomy was performed and 2 litres of turbid fluid were removed. The pleura was generally thickened, but no nodules were seen or felt. The underlying lung was collapsed and covered with a fairly thick layer of fibrin. A lung biopsy and a further pleural biopsy were taken. The histological reports were as follows: ‘The pleura shows a fibrinoid exudate at its surface, which is undergoing organization. One of the sections contains a small area of necrosis within the fibrous tissue, surrounded by several layers of palisading histiocytes. The lung histology shows fibrotic lung tissue containing fairly large circular necrotic areas surrounded by histiocytes and fibroblasts which are partially palisaded (Fig. 3). The lung tissue in general shows interstitial fibrosis with obliteration of alveoli. and is densely infiltrated with lymphocytes, plasma cells and occasional polymorphs (Fig, 4). Some differentiation of smooth muscle cells appears to have occurred within the interstitial tissue. There is a marked muscular hypertrophy of the walls of small pulmonary arteries and arterioles. No acid fast bacillae are seen on ZN smears. The histological changes are consistent with a rheumatoid lung.’ Further bacteriological studies on the pleural fluid were negative. On 29 October he was discharged home on prednisolone 15 mg daily, and 1971, the arthritis was quiescent. He was when last seen on I 7 November
THADANI
150 only slightly dyspnoeic on exertion. significantly since his thoractomy.
The chest radiograph
had not changed
Discussion Rheumatoid arthritis is a common disease in women, although pulmonary manifestations occur more frequently in men. The patient described had had radiological evidence of pulmonary fibrosis for 4 years before the development of a pleural effusion, polyarthritis and subcutaneous nodules. The diagnosis of rheumatoid lung was established only after the demonstration of microscopic pleural and intrapulmonary necrobiotic (rheumatoid) nodules, which are accepted as pathognomic of this form of lung disease (Edge & Rickards r 957). Whether or not the interstitial pulmonary fibrosis in the present case was a manifestation of the rheumatoid process remains debatable. The absence of other aetiological factors leading to pulmonary fibrosis and the subsequent development of arthritis, vasculitis, subcutaneous nodules and the demonstration of pleuropulmonary necrobiotic (rheumatoid) nodules strongly suggest a direct relationship. This assumption is also supported by Turner-Warwick’s (1968) observations of a positive rheumatoid factor in I 7% of rag patients with interstitial pulmonary fibrosis but without evidence of arthritis and by the demonstration of pleuropulmonary necrobiotic nodules in association with interstitial pulmonary fibrosis in previously reported cases (Gresham & Kellaway 1958, Case 2 ; Cruickshank 1959, Case 2 ; Flatley 1959; Cudkowicz et al. 1961; Ramirez & Campbell r 966, Case 2; Rubin et al. 1967, Case 2). It is a clinical point of interest that whereas in the previously reported cases (Flatley 1959; Cudkowicz et al. 1961) similar pulmonary lesions followed the onset of arthritis by several years, the reverse relationship is noted in the present case. The therapeutic value of corticosteroids in rheumatoid lung disease is not clearly defined. While some of the reported cases appear to have responded to this form of treatment (Ellman 1956; Doctor & Snider 1962 ; Edge & Rickards 1957) others as well as the present case either show no improvement or may actually deteriorate (Rubin 1955; Emerson 1956; Dixon & Ball 1957; Cudkowicz et al. 1961; Stack & Grant 1965). As routine pleural and lung biopsies are not performed in all cases of it seems likely that their true rheumatoid arthritis with pulmonary lesions, incidence is underestimated. Further detailed case reports and histological observations may throw more light on the interrelationship between rheumatoid arthritis and its lung manifestations. Summary The case of a patient with rheumatoid lung disease showing pulmonary fibrosis, necrobiotic nodules and a pleural effusion is described. Radiological evidence of pulmonary fibrosis was detected 4 years before the development of rheumatoid lung was arthritis and a right pleural effusion. The diagnosis of rheumatoid
RHEUMATOID
LUNG
151
DISEASE
confirmed by demonstration of pleuropulmonary necrobiotic probability that interstitial pulmonary fibrosis in this case was a of rheumatoid process is discussed. Corticosteroid therapy did improve the pulmonary fibrosis, although the arthritis remained this treatment.
nodules. The manifestation not seem to quiescent on
ACKNOWLEDGEMENTS
I am grateful to Dr C. P. Aber for allowing me to publish this case, to Mr K. C. Moghissi who performed thoracotomy and to Dr A. J. Shillitoe for histological reports on the lung biopsy. I wish to thank Dr M. E. Turner-Warwick, of the Institute of Diseases of the Chest, for her advice on the case summary. REFERENCES
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