Simultaneous presentation of sarcoidosis and Sjogren's syndrome

Sir, A 32‐yr‐old woman presented with pretibial painful red nodules. She had a 6‐month history of migratory pain and swelling of several joints. She complained of daily progressive oral dryness, thirst, impaired swallowing and blurred vision with a feeling of dry eyes for more than 3 h a day. Her medical, family, occupational and environmental histories were non‐contributory. She used an oral contraceptive and ibuprofen. Physical examination showed oedema of the ankles and erythema nodosum on both legs. Laboratory investigations revealed an erythrocyte sedimentation rate of 44 mm/h (normal value <10) and a C‐reactive protein (CRP) level of 42 mg/l (normal value <3). Routine haematological laboratory and blood chemistry values were all within the normal ranges. Urine analysis showed no erythrocytes. Creatinine clearance was normal, with a slight proteinuria of 300 mg/24 h. Serum lysozyme was elevated at 9.7 mg/l (normal range 0.6–2.6) and angiotensin‐converting enzyme (ACE) was 134 U/l (normal value <45). Antinuclear antibody, antibodies to extractable nuclear antigens and anti‐double‐stranded DNA (Farr assay) were all negative. Antibodies against SS‐A (Ro) were positive. IgM rheumatoid factor was elevated at 105 kU/l (normal value <15). The IgG concentration was 19.2 g/l (normal range 8.5–15.0), IgA was 5.4 g/l (normal range 0.9–4.5) and IgM was 2.2 g/l (normal range 0.6–2.6). No cryoglobulins were found. A tuberculin test with purified protein derivative was negative. A chest radiograph showed extensive mediastinal and bilateral hilar lymphadenopathy. Pulmonary function tests were normal. Ophthalmological examination demonstrated a normal Schirmer's tear test [10 and 9 mm/5 min for the right and left eye respectively (normal value ≥5.5 mm/5 min)] but a decreased tear film break‐up time of 2 s for both eyes (normal value ≥10 s) and positive Rose Bengal staining (Van Bijsterveld score 5 and 3 for the right and left eye respectively; normal value <3.5), consistent with keratoconjunctivitis sicca. Sialometric analysis showed reduced unstimulated and stimulated (2% citric acid) flow of whole saliva. The reduced unstimulated flow of whole saliva was caused by a very low rate of secretion of all individual salivary glands (<0.01 ml/min), whereas the reduced stimulated flow of whole saliva was caused mainly by very low secretion from the parotid glands (0.02 ml/min per gland; normal range 0.05–0.5 ml/min). Sialochemistry revealed elevated sodium concentrations and a normal amylase concentration in whole, parotid and submandibular/sublingual saliva. Sialography of the right parotid gland showed globular sialectasia. Histopathological examination of parotid tissue showed epithelioid cell granuloma as well as periductal lymphocytic infiltration and epimyoepithelial islands. Immunohistochemical staining showed a polyclonal plasmacytic infiltrate and a shift in the relative number of IgA‐bearing plasma cells in favour of IgG‐bearing plasma cells. A labial salivary gland biopsy revealed extensive epithelioid cell granuloma and lymphocytic infiltration with a positive focus score of 1 (normal value <1). The patient was diagnosed as having sarcoidosis, presenting as Löfgren's syndrome, with coexisting Sjögren's syndrome. During a 2 yr follow‐up, laboratory investigations revealed normalization of CRP and ACE activity and persistence of anti‐SS‐A antibodies. Repeated sialometric analysis showed persistent reduction in the unstimulated and stimulated flow of whole saliva, with further reduction in submandibular/sublingual salivary secretion and persistent elevation of sodium concentration. A chest radiograph showed a major reduction in mediastinal and hilar lymphadenopathy. Until now, the patient has been treated symptomatically for her sicca complaints.