Bronchocentric granulomatosis associated with seropositive polyarthritis.

Abstract

Bronchocentric granulomatosis was first described by Liebow' and is a form of pulmonary granulomatosis and angiitis. The disease is characterised by its clinical and morphological features but the clinical2 and radiographic, manifestations are rather non-specific. Lung biopsy is necessary to establish the diagnosis and granulomatous inflammation, centred predominantly on the bronchi and bronchioles, is pathognomonic. According to the original description of Liebow,' one of the characteristics of bronchocentric grantilomatosis is the absence of extrapulmonary lesions. We here report a case of bronchocentric granulomatosis associated with seropositive polyarthritis. Case reportIn August 1981 a 58 year old man was admitted to another hospital with fever and an abnormal chest radiograph. He was treated initially with penicillin for suspected bacterial pneumonia; later doxycycline and erythromycin were given without definite improvement. When tomography subsequently showed enlarged hilar glands with persistent pulmonary shadowing, sarcoidosis was suspected. No biopsy was performed and prednisolone (20 mg daily) was started. After initial improvement, the clinical and radiographic manifestations worsened. In November 1981 he was referred to our hospital for further investigation. He was reported to have had mild asthmatic symptoms 20 years earlier, a myocardial infarction in 1975, and surgery for diverticulitis of the colon in early 1981.Shortly before transfer the patient developed polyarthritis. On admission he appeared ill, with a temperature of 390C, a persistent cough, haemoptysis, dyspnoea at rest, and chest pain. He had no history of Raynaud's phenomenon. On physical examination breath sounds were diminished over the lower part of the right lung and no wheezes were heard. The metacarpophalangeal and proximal interphalangeal joints were swollen and painful but there were no subcutaneous nodules. The chest radiograph (fig 1) showed bilateral macronodular lesions; the right lung, which showed cavitation, was more affected than the left. (fig 2) showed necrotising granulomatous inflammation centred predominantly on and destroying bronchioles. The adjacent pulmonary arteries showed vasculitis without evidence of bacteria or fungi. These features were consistent with bronchocentric granulomatosis. The dose of prednisolone was increased to 100 mg daily and the patient responded well. Aft r the dose had been tapered to 20 mg daily over an eight week period, the polyarthritis again worsened and the dose was increased temporarily to 30 mg.

Bronchocentric Granulomatosis Associated with Influenza-A Virus Infection

“…It rarely causes respiratory failure due to pneumothorax or adult respiratory distress syndrome (ARDS) [11]. Although extrapulmonary symptoms are usually absent, a growing number of reports suggest an immunologic link with arthritis, ankylosing spondylitis, scleritis, uveitis, and glomerulonephritis [12, 13, 14, 15]. …”

Allergic bronchopulmonary aspergillosis

“…The pathology of the peribronchial areas and parenchyma may differ from that described above: bronchocentric granulomatosis with bronchial remodelling and dilation have been described (39). However, bronchocentric granulomatosis is a distinct entity often associated with a pseudo‐tumoral radiologic pattern (38), and possibly with other conditions such as tuberculosis, inflammatory diseases of the bowel and rheumatoid arthritis (40–43). The infiltration of the parenchyma with mononuclear cells, eosinophils and lymphocytes leads to inflammation that mimics or is associated with the patterns observed in individuals with other forms of interstitial disease such as granulomatous bronchiolitis, exsudative bronchiolitis or obliterans bronchiolitis (38).…”

Sarcoidosis: Differential Diagnosis

“…A case with seropositive polyarthritis was recently described. 125 Therapy of choice is prednisolone. The prognosis is usually good.…”