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Rheumatic pneumonia (RP) is a well described and poorly understood complication of acute rheumatic fever (ARF). It has been reported for more than a century and it has been traditionally associated with a high mortality rate. However, the existence and specificity of primary pulmonary lesions has remained controversial, because similar features may be seen in ARF with complicating congestive failure or uraemia.1 2 We report a case of RP that was successfully treated with steroids.
An 18 year old man was admitted to our hospital because of a 10 day history of fever, malaise, and dry cough. The patient had had ARF with carditis at age 7, which resolved without sequels. A tonsillectomy was performed three years later. Since then, he had received a benzathine penicillin G injection monthly until three years before his actual admission. On admission, physical examination disclosed a temperature of 38.5°C, respiratory rate of 26/min, and rales were heard at the left lower lung. The rest of the examination was unremarkable.
Laboratory values were white blood cells 14.3×109/l, with 80% neutrophils and 9% band cells, haemoglobin 132 g/l, and platelets 410×109/l. The erythrocyte sedimentation rate was 90 mm/1st h. Urine analysis, coagulation studies, renal and hepatic function tests, and arterial blood gas value analysis during room air breathing were normal. A chest x ray examination showed an ill-defined and non-homogeneous area of consolidation at the left lower lobe with a normal cardiac silhouette. As there was a clinical suspicion of pneumonia, acquired in the community, intravenous cefuroxime (750 mg three times a day) and oral roxithromycine (150 twice daily) were given.
On the fourth day after admission to hospital, fever and tachypnoea persisted. Consolidation of the air space in the posterior segment of the left lower lobe and pleural effusion were seen on chestx ray examination and thoracic computed tomography (fig 1). Thoracentesis yielded a serous fluid containing 1.2×109 leucocytes/l, with 70% neutrophils, glucose 1.4 mmol/l, protein 27 g/l, and lactate deyhdrogenase 477 IU/l. Doppler echocardiography showed a posterior pericardial effusion without any other abnormality. Bronchoscopic examination showed inflammatory changes in left lower bronchi. There were no neoplastic cells in bronchoalveolar lavage specimens. Repeated serological tests for Mycoplasma pneumoniae, Chlamydia pneumoniae, Legionella species, cytomegalovirus, and Epstein-Barr virus were negative. Tests for antineutrophil cytoplasmic antibodies, rheumatoid factor, and antinuclear antibodies were negative, and the serum level of angiotensin converting enzyme was normal.
On the 14th day after admission to hospital the patient developed arthritis at the left knee and the right wrist. Arthrocentesis showed a turbid fluid which contained 15.2×109 leucocytes/l and glucose 1.3 g/l; no crystals were seen. Titres of antistreptolysin O and C reactive protein were 400 Todd units (normal <200) and 1.20 g/l, respectively. Blood, sputum, pleural fluid, bronchoalveolar lavage, and joint effusion repeated cultures were negative, and no acid fast bacilli were seen.
The antibiotic treatment was discontinued and acetylsalicylic acid (1.5 g four times a day) was started. A dramatic improvement in fever and arthritis occurred, though the radiological lesion remained unchanged. A histological lung specimen obtained by thoracoscopy showed inflammatory changes with thickness and fibrosis of alveolar septa, and nodular aggregates of mature lymphocytes. No micro-organism was seen or cultured in tissue specimens. Prednisone 60 mg daily was substituted for aspirin. The radiological lesion improved progressively, and antistreptolysin O and C reactive protein normalised. Corticosteroid treatment was tapered over two months without any further relapse, and prophylaxis with benzathine penicillin G monthly was resumed. In a 24 month follow up period the patient remained asymptomatic.
For unknown reasons, in the past decades there has been a decline in the incidence and severity of rheumatic fever in developed countries.3 Thus unusual complications, such as RP, are a challenging diagnosis for clinicians without experience of this illness.4 To the best of our knowledge, only six isolated case reports have been reported in English or Spanish over the past 20 years (table 1).4-9
Pulmonary disease may occur in both the first and recurrent attacks of ARF, either as part of the rheumatic process, or secondary to the cardiac lesion, congestive heart failure, uraemia or intercurrent infection.2 The incidence of pulmonary disease in ARF has greatly varied (2–60%) depending on the rigidity of the criteria used and the nature of the series.1 2 The clinical spectrum of RP varies from a mild to a fulminant course. Dyspnoea and non-productive cough are the most commonly reported symptoms.1 2 Multiple radiological patterns have been described, including consolidation, diffuse bilateral and migratory infiltrates, pleural effusion, and confluent nodular lesions.1 4 A pathological examination shows no unique lung lesions in ARF, and the most commonly reported pulmonary findings have been alveolar haemorrhage, acute bronchopneumonia, organising fibrinous oedema, interstitial inflammatory exudates, inflammation and necrosis of septa, arteriolitis, and globular tufts of intra-alveolar fibrous tissue, so-called Masson bodies.10 It may be difficult to separate the contribution of the heart failure or uraemia to the RP by itself.1 2 10 Because of the lack of specific criteria, RP remains an exclusion diagnosis.
Our patient satisfied the updated Jones criteria for the diagnosis of ARF.3 The absence of response to antimicrobial treatment, the histological findings, and the response to anti-inflammatory treatment favoured a diagnosis of RP. Despite the fact that salicylates have been advocated in the management of RP,6 8 steroids seems to be the most suitable treatment. None the less, deaths have occurred despite the use of this treatment.5 9
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