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Clinical Meetings at RH Year 2004

2004 - A patient with parotid swelling and lung infiltrates

Dr CW Yim, Respiratory Medical Department, Kowloon Hospital

Case history
The patient was a forty-year-old lady. She had history of primary Sjogren's syndrome and bronchiectasis followed up in PWH Rheumatology Clinic. She was admitted into PWH from 9/8/99-21/8/99 because of cough, intermittent fever and mild weight loss. CXR showed left lingular consolidation. Sputum was positive for AFB smear. She was thus started on HRZM since 20/8/99 and referred to chest clinic for DOT and follow up. She was referred to RMD Kowloon Hospital in 11/99 because sputum culture turned up to be MDR-TB. There was only one single isolated smear and culture-positive specimen (20/8/99). The sensitivity result was resistant to HR but sensitive to all other first- and second-line drugs. All subsequent sputum specimens were smear and culture-negative. Anti-tuberculosis treatment was modified in KH. She had intolerance to multiple anti-TB drugs. She got severe dizziness with Streptomycin, gastrointestinal upset with prothionamide, blurred vision with ethambutol and anaphylactic shock with PAS. Finally she was put on Pyrazinamide and Levofloxacin since 7/1/00. However, serial CXRs showed increasing BLZ shadows. CT thorax revealed bronchiectasis and multiple patchy areas in bilateral lower zones and left lingular lobe. Bronchoscopy was performed which showed no endobronchial lesion. Bronchial aspirate was unremarkable. Transbronchial biopsy at left lingular lobe revealed features compatible with lymphocytic interstitial pneumonitis.

She was referred back to PWH Rheumatology. However she was not put on steroid or immunosuppressant therapy in view of MDR-PTB. She was readmitted in 5/01 because of increased RML shadow on CXR. Bronchoscopy was performed, showing no endobronchial lesion. Bronchial aspirate was unremarkable and transbronchial biopsy at right middle lobe showed atypical lymphoid hyperplasia. The case was reviewed in medical conference in RMD, Kowloon Hospital. The suggestion was to start prednisolone 20mg daily and azathioprine 100mg daily for the treatment of lymphocytic interstitial pneumonitis under the cover of anti-TB drugs (HRZ Cravit). The isolated positive culture of MDR- TB was regarded as clinically insignificant. The above treatment regimen was started on 14-5-01. However, anti-TB treatment was stopped on 30-5-01 because of repeated vomiting and deranged liver function (peak ALT 78). Azathioprine was also stopped on 16-6-01 in view of liver function impairment. Thereafter, the liver function reverted to normal. Anti- TB drugs (HRZ Cravit) were resumed on 21-6-01 and azathioprine was resumed on 26-6-01.

The patient noticed bilateral parotid swelling since 30-6-01. The right parotid mass resolved by itself spontaneously. Fine needle aspiration of the left parotid mass showed scanty atypical lymphoid cells. LDH was normal. Azathioprine was stopped again on 19-7-01 because ALTrose up to 77. Another immunosuppressive drug, cyclophosphamide was started on 31-7-01. The patient stopped prednisolone on 19- 8-01 by herself because of Cushingnoid features. Cyclophosphamide was stopped on 21-9-01 because of diarrhea and low white cell count of 3. The patient also refused to continue anti-TB drugs on 28-9-01. Despite incomplete treatment course, there was already marked radiological improvement with resolving right middle zone shadow.

The patient was referred to Prince of Wales Hospital for exisional biopsy of the left parotid mass, which showed features compatible with Sjogren's syndrome. A new right parotid mass developed since 29-1-02. Ultrasound of neck showed that both parotid and submandibular glands were atrophic. The palpated right parotid mass was a prominent intraparotid node. Fine needle aspiration of the intraparotid node showed lymphoid proliferation.

A new right middle zone shadow developed in CXR since May 2003. Bronchoscopy was repeated and showed no endobronchial lesion. Transbronchial lung biopsy at right middle lobe showed chronic inflammation. The patient was referred to Cardiothoracic Unit of Queen Elizabeth Hospital for video-assisted thoracoscopic surgery. The lung biopsy showed extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (Maltoma). She was thus referred to Oncology Department of Prince of Wales Hospital for chemotherapy.

Discussion
Sjogren's syndrome is a chronic, slowly progressive inflammatory autoimmune exocrinopathy of unknown etiology. It has features of keratoconjunctivitis sicca and xerostomia. Primary Sjogren's syndrome occurs alone, while secondary Sjogren's syndrome is associated with connective tissue disease. Pulmonary complications in Sjogren's syndrome occur in 1.5-75% of patients. Interstitial lung disease of Sjogren's syndrome most commonly presents as lymphoid interstitial pneumonia (LIP). There are isolated reports of usual interstitial pneumonia, non-specific interstitial pneumonia and bronchilolitis obliterans organizing pneumonia.
Lymphoid interstitial pneumonia usually occurs in middle age group, with slight female preponderance. Pulmonary symptoms include cough, dyspnea and chest pain. Systemic symptoms include weight loss, fever and maybe arthralgia. Physical examination is usually unremarkable with bilateral basal end-inspiratory crackles. Clubbing is uncommon. There may be peripheral lymphadenopathy. There is abnormal production of immunoglobulin production. It can be polyclonal hyperglobulinaemia, monoclonal gammaopathy or hypogammaglobulinemia. Pathologically, the alveolar septa are enlarged, with infiltrate that is prominent in relation to lymphatics, more marked around bronchovascular bundles, bordering interlobular septa. There are round aggregates around small intralobular vessels. Radiologically, LIP can present as diffuse fine interstitial infiltrates to reticulonodular appearance. They are more prominent in bilateral lower zones. There may be confluent areas of consolidation. In late stage, there is honeycomb change. LIP has restrictive defect and there might be reduction in gas transfer.

A spectrum of lymphoid proliferation has been described in the lung. The most benign disorder is lymphocytic interstitial pneumonia. The terms 'pseudolymphoma' and 'lymphoid interstitial pneumonia' (LIP) are well established. It was widely used to describe supposedly benign, reactive lymphoid hyperplasia which is localized and diffuse respectively. Some interpreted these conditions as premalignant conditions. Treatment is usually with corti co steroids :t cytotoxic therapy (variable response: remission/stable/progress). Malignant lymphocytic lymphomas may also occur if the lymphoproliferation is frankly malignant. Lymphoma of the lung is substantially less common than lymphoma at other sites & has been reported to occur in 1 % - 2 % of patients with Sjogren's syndrome. 43-fold increased risk of developing a malignant lymphoma. In patients with Sjogren's-associated lymphoma, 20 % have pulmonary involvement. The malignant transformation from a benign lymphoproliferative process to a malignancy is believed to result from chronic excessive B-cell stimulation. The prevalence of non-Hodgkin's lymphoma in Sjogren's syndrome patients is 4.3%. The median time from diagnosis of Sjogren's syndrome to that of lymphoma is 7.5 years. The predictors of lymphoma development include persistent enlargement of parotid gland, splenomegaly, lymphadenopathy, palpable purpura, and leg ulcers. Serologically it includes low levels of C4, mixed monoclonal cryoglobulinaemia. Treatment of malignant lymphomas depends on their location, histology and extent. It does not differ from the treatment of lymphoma without Sjogren's syndrome. The histologic grade is very important prognostic factor for survival. For low-grade lymphoma, the median survival is 6.3 years. For high-grade lymphoma, the median survival is 1.8 years. The presence of B symptoms and a large tumor (7cm) are independent risk factors for death.

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