Sjögren's syndrome co-existing with limited Wegener's granulomatosis

G. Karargyris, K. Stamatopoulos, C. Kittas1, D. Loukopoulos and G. Vaiopoulos

First Department of Medicine, University of Athens School of Medicine, Laikon General Hospital, Athens and
1 Pathology Department, University of Athens School of Medicine, Athens, Greece

SIR, Wegener's granulomatosis (WG) is a systemic vasculitis mainly involving the respiratory tract and the kidneys; a limited form of the disease is distinguished from the classical form by sparing of the kidneys [1].

We describe a case of limited, pulmonary Wegener's granulomatosis accompanied by Sjögren's syndrome (SS). The two conditions were diagnosed simultaneously in the investigation of a 61-yr-old housewife with fever of 1 month duration, myalgias and pulmonary opacities; her past medical history was unremarkable. On examination, she was weak, with bilateral atrophy of the gastrocnemial muscles; deep tendon reflexes were depressed in the right lower extremity. Schirmer's test was positive (0 mm). No other abnormal physical findings were noted.

Laboratory examinations were as follows: haemoglobin 9.8 g/dl; white blood cell count and differential normal; ESR 80 mm in first hour; CRP 122 mg/l (normal range 0–5 mg/l); serum aminotransferases, alkaline phosphatase, gamma-glutamyltranspeptidase and lactate dehydrogenase mildly elevated; total serum proteins 6.7 g/dl with increased immunoglobulins. The following tests were negative or normal: serum creatine phosphokinase and aldolase; immunofixation; virus, Brucella and Toxoplasma serology; urinalysis; 24-h urine collection for protein; repeated blood, urine and sputum cultures; electrocardiogram; heart ultrasound scan. Chest radiography revealed confluent opacities of the right middle lobe. CT scan of the chest showed multiple nodules, up to 3 cm in diameter, predominantly in the right middle lobe; CT scans of the facial skeleton and the abdomen were normal. The patient tested positive for rheumatoid factor (503 IU; normal range 0–20 IU), antinuclear antibodies (1:640, speckled pattern), anti-Ro (SS-A) antibodies, cryoglobulins (polyclonal) and anti-neutrophil cytoplasmic antibodies (ANCA, c-ANCA 78.3 U/l, p-ANCA negative); antibodies to cardiolipin, DNA and ribonucleoprotein were negative. Serum complement levels were normal. Pathological examination of a salivary gland biopsy specimen revealed 10 large lymphoplasmacytic infiltrates in 20 mm2 of tissue. Fibre-optic bronchoscopic examination was normal; bronchial washings and bronchoalveolar lavage specimens contained 150 000 cells/ml with 85% histiocytes. No acid-fast bacilli were detected. Cultures were negative. The patient underwent an open biopsy of the middle lobe of the right lung. Gross examination of the specimen showed several discrete nodules. Microscopic examination revealed granulomatous lesions with central parenchymal necrosis in the form of neutrophilic microabscesses, surrounded by histiocytes and vasculitis of medium-sized vessels, typical of WG. DNA from the lung biopsy material tested positive by multiplex polymerase chain reaction for the presence of the 65-kDa gene of the Mycobacterium tuberculosis complex (M. tuberculosis, M. bovis, M. africanum, M. microti).

Based on these findings, possible diagnoses for our patient include: (i) primary SS associated independently with limited WG; and (ii) secondary SS as a feature of the syndrome of Wegener's vasculitis. An association between WG and SS has been investigated in the past [26]. Several clinical reports and limited clinical studies have indicated that both the limited and the generalized form of WG may involve the sclera, lachrymal glands and salivary glands [5, 6]. It has been reported that major salivary gland involvement may be associated with a limited form of WG and a more favourable prognosis; in these cases, whenever pathological examination was performed the findings were highly suggestive of WG [5].

It is generally accepted that limited forms of WG have a more indolent course and are said to have a better prognosis than the classical disease, but may be extremely challenging to recognize and diagnose. Our patient was treated with methylprednisolone and trimethoprim–sulfamethoxazole per os which has proven effective either as monotherapy or with corticosteroids for the induction of remission in limited WG [7]; the drugs have also been used effectively as relapse prophylaxis after remission in patients with generalized WG [7]. The patient responded favourably to this combined treatment and remains in complete remission.

In conclusion, the laboratory results presented herein suggest that, in our patient, primary SS and limited WG evolved independently, SS probably preceding the development of WG and running a mild, asymptomatic course. This suggestion is given further support by the fact that, 2 years after diagnosis, our patient is still free of sicca symptoms.

Notes

Correspondence to: G. Vaiopoulos, Vardousion 13, Ampelokipi, Athens 115 26, Greece. Back

References

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Accepted 10 January 2000





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