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Year : 2014  |  Volume : 5  |  Issue : 2  |  Page : 182-184

Primary Sjogren's Syndrome presenting with Interstitial lung disease and Myositis

1 Department of Tropical Medicine, School of Tropical Medicine, Kolkata, India
2 Department of Rheumatology, North Bengal Medical College and Hospital, West Bengal, India

Date of Web Publication1-Jul-2014

Correspondence Address:
Kumkum Sarkar
Department of Tropical Medicine, School of Tropical Medicine, Room No-20, 108, C R Avenue, Kolkata - 700 073, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0975-9727.135771

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Sjogren's syndrome (SS) is an autoimmune disease, is the most common autoimmune disease following rheumatoid arthritis but remains undiagnosed in more than half. The hallmark histopathological features of SS are periepithelial lymphocytic infiltration of the affected organs. Antibodies to SSA (Ro) and SSB (La) are characteristically associated with SS. The reported frequency of pulmonary involvement in primary SS varies widely, ranging from 9% to 75% and consists of various forms of small airway diseases and interstitial lung diseases (ILD). We are reporting a case of primary SS presenting with ILD and myositis.

Keywords: Interstitial lung disease, myositis, primary Sjogren′s syndrome

How to cite this article:
Sarkar K, Haldar SN, Das S, Gonjhu D, Pramanik N. Primary Sjogren's Syndrome presenting with Interstitial lung disease and Myositis. Muller J Med Sci Res 2014;5:182-4

How to cite this URL:
Sarkar K, Haldar SN, Das S, Gonjhu D, Pramanik N. Primary Sjogren's Syndrome presenting with Interstitial lung disease and Myositis. Muller J Med Sci Res [serial online] 2014 [cited 2023 Mar 23];5:182-4. Available from: https://www.mjmsr.net/text.asp?2014/5/2/182/135771

  Introduction Top

Sjogren's syndrome (SS) is characterized by lymphocytic infiltration of exocrine glands resulting in xerostomia and keratoconjunctivitis sicca. The disease may be primary (idiopathic) or secondary to other diseases like rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis or biliary cirrhosis. [1] The clinical consequences are ranging from difficulty in speaking and eating, oral candidiasis, rampant caries to sialoadenitis due to xerostomia, diminished secretion of tear and destruction of corneal and bulbar conjunctival epithelium with ocular complaints of itching and dryness. [2] Systemic involvement of the disease is the lungs, kidneys, blood vessels and muscles. Treatment of SS is aimed at symptomatic relief and limiting the damaging local effects of chronic xerostomia and keratoconjunctivitis sicca by substituting or stimulating the missing secretions.

  Case Report Top

This was a case report of a 40-year-old female patient presented at School of Tropical Medicine with gradually progressive, multiple peripheral joint pain without significant morning stiffness, low grade intermittent fever, exertional dyspnea and generalized weakness since last 1 year. Further history revealed difficulty in eating, adhering of food to the oral mucosa, gritty sensation in eyes, dyspareunia and proximal muscle weakness for same duration. She had history of weight loss about 5 kg during this illness. Her past history of illness was not contributory. On examination she had mild pallor and bilateral non-tender parotid gland swelling [Figure 1]. She had swelling and tenderness at right middle proximal metacarpo-phalangeal and interphalangeal joints, both knee and ankle joints. She had mild hepato-splenomegaly and bilateral basal end-inspiratory fine crepitations. She had no history of Raynaud's phenomenon. Examination of other systems were normal. Her hemogram showed mild normocytic normochromic anemia (haemoglobin-9.9 mg/dl) with high erythrocyte sedimentation rate (82 in 1 st h). Her serum glutamic-oxaloacetic transaminase was 223 IU/L, serum glutamic-pyruvic transaminase-154 IU/L and total protein-9.7 g/dl, albumin-2.5 g/dl with normal bilirubin and alkaline phosphatase. Serological test for hepatitis B surface antigen, anti-hepatitis C virus and human immunodeficiency virus enzyme-linked immunosorbent assay (ELISA) were non-reactive. Her chest X-ray was normal and ultrasonography of abdomen showed mild hepato-splenomegaly. Blood for rheumatoid factor was positive (57 IU/L, normal <20) by ELISA method and other autoantibodies like antinuclear antibodies, SS-A, SS-B and Ro-52 were highly positive but Scl-70, ribonucleoprotein, double-stranded deoxyribonucleic acid, JO-1 were negative by using EUROIMMUNE (Medizinische Labordiagnostica AG) kit and by automatic evaluation of test strips using the EURO Line Scan software. Schirmer's test and unstimulated saliva secretion were normal. Rose Bengal test was positive in right eye which denotes breach in the corneal epithelium due to xerophthalmia. Parotid ultrasonography showed multiple hypoechoic areas (average 1 mm) in both parotid and submandibular glands. Pulmonary function test revealed restrictive type of lung disease and high-resolution computed tomography thorax showed fine intralobular fibrosis with interstitial thickening of lower zone of both hemithorax without evidence of cysts that means reticulonodular pattern type 1 [Figure 2]. Blood for lactate dehydrogenase (728 U/L) and creatine phosphokinase (1903 U/L) were very high and electromyogram and nerve conduction velocity study of all four limbs revealed changes suggestive of myopathy. Her lip biopsy of minor salivary glands showed scattered and aggregates of lymphocytes and plasma cells, which is consistent with sialoadenitis with focus score grade 3, suggestive of SS [Figure 3]. So finally the case was diagnosed as a case of primary SS with interstitial lung disease (ILD) and myositis. The patient was treated with artificial tear (methylcellulose), advice for frequent dental care, hydroxychloroquine, prednisolone for arthritis and cyclophosphamide for ILD.
Figure 1: Parotid swelling

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Figure 2: Interstitial thickening in interstitial lung diseases

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Figure 3: Lip biopsy of minor salivary glands showed scattered and aggregates of lymphocytes and plasma cells, which is consistent with sialoadenitis with focus score grade 3

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This patient fulfills the American European Consensus Group Classification criteria of SS. Patient usually presents with subjective xerostomia or xerophthalmitis, but the disease can also begin with systemic (articular, vascular, lung, etc.) manifestations. [3] Extraglandular manifestations are seen in one-third of SS patient. Arthralgia or arthritis is seen about 60% cases of SS. Myositis is rarely seen in primary SS (1%). [4] Pulmonary manifestations are frequent in primary SS (14%) but often not clinically significant; the most common are xerotrachea, ILD and small airway obstruction. Pulmonary manifestations in SS have a slow progression and favorable prognosis, with the exception of primary pulmonary lymphoma and pulmonary hypertension. [5] A review of 343 patients with classic SS (both primary and secondary) seen at Mayo clinic revealed pulmonary involvement in 31 patients (9%). [6] ILD was seen in 13 of the 31 patients (42%). Other studies dealing with primary SS only have reported ILD in 8-25% cases. [6] We are reporting a case of primary SS with arthritis, ILD and myositis. Pulmonary lesions, whereas not being rare in primary SS, often get over looked. In patients with ILD, taking a history of sicca symptoms may provide a valuable clue to the underlying disease as illustrated in this case report. As it has been observed that these patients are at a risk of malignant transformation into lymphomas (5%), a regular follow-up is mandatory. [7]

  References Top

1.Rajendran R. Shafer′s Textbook of Oral Pathology. 5 th ed. Noida: Elsevier Publishers; 2005. p. 346-9.  Back to cited text no. 1
2.Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 2004;164:1275-84.  Back to cited text no. 2
3.Pavlidis NA, Karsh J, Moutsopoulos HM. The clinical picture of primary Sjögren′s syndrome: A retrospective study. J Rheumatol 1982;9:685-90.  Back to cited text no. 3
4.Fauci, Braunwald, Kasper, Hauser, Longo, Jameson, Loscalzo. Sjogren′s syndrome. In: Harrison′s Principles of Internal Medicine. 17 th ed. New York: McGraw Hill Medical; 2008. p. 2107-8.  Back to cited text no. 4
5.Available from: http://www.ncbi.nlm.nih.gov/pubmed/20692541. [Last accessed on Apr 2012].  Back to cited text no. 5
6.Strimlan CV, Rosenow EC 3rd, Divertie MB, Harrison EG Jr. Pulmonary manifestations of Sjögren′s syndrome. Chest 1976;70:354-61.  Back to cited text no. 6
7.Pillemer SR. Lymphoma and other malignancies in primary Sjögren′s syndrome. Ann Rheum Dis 2006;65:704-6.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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