Lady Windermere syndrome in Sjögren’s syndrome

Lady Windermere syndrome was first described in 1992 [1,2]. It is characterized that female patient without apparent predisposing pulmonary disease had habit of voluntary suppression of cough which induce to unable to clear the secretions, always caused by non-tuberculosis mycobacterium (NTM), from the right middle and left lingual lobe of lung. Sjögren’s syndrome is a chronic autoimmune disease with exocrinopathy, which could induce severe dryness of airway mucosa to exacerbate inability to clean-out pathogen or inflammatory secretions.

We present a case of a 58-year-old female with chronic cough for 20 years, from time to time, dry cough or purulent and bloody sputum. She was diagnosed as having NTM infection three times at different clinics previously, and she received anti-NTM antibiotics treatment once. There were no other symptoms, such as fever, dyspnea, body weight loss, lethargy or night sweats. She came to our clinic due to xerophthalmia and xerostomia with hemoptysis. Initially, Sjögren’s syndrome was impressed with serum anti-Ro-antibody level of 75 U/mL, decreased lacrimal gland secretion (Schirmer’s test: 0 mm per 5 min) and decreased concentration and delayed dynamics with bilateral salivary glands involvement by sialoscitingraphy [3]. To exclude other autoimmune diseases, which may be associated with lung disease, other antibodies (such as anti-neutrophil cytoplasmic antibody, rheumatoid factor, anti-Jo-1-Ab, and anti-ribonucleoprotein-Ab) were tested but all of them revealed negative result. More than that, tests for sputum ordinary and nocardia culture were all negative. Three sets of sputum acid fast stains showed strongly positive, while sputum mycobacterium tuberculosis (TB)-qPCR and whole blood interferon gamma release assay test for TB were negative. Chest-computed tomography (Fig. 1) showed centrilobular infiltrations with thickening bronchial wall, bronchiectasis, small consolidation and subpleural reticulation in both lungs, more found in right middle lobe and left lingual lobe. During the hospitalization, she was isolated initially for the first positive acid-fast stain result, being transferred to ordinary ward on the next day owing to negative (TB)-qPCR result. In the following, all sputum culture showed mycobacterium abscessus.

Fig. 1.

Chest-CT revealed diffuse patchy consolidations, bronchiectasis, centrilobular infiltrations and inter-septal thickening with mucus formation in the right middle and left lingual lobe of lung.

In clinical approach, infection pathogens, such as tuberculosis, non-tuberculous mycobacteria (NTM), cytomegalovirus, fungus and atypical pathogens should be considered in these patients with autoimmune diseases. In immunocompromised status, either caused by underlying autoimmune diseases or ever receiving immunosuppressive agents, these patients always have a higher risk of infections. For the reasons given above, our patient has Sjögren’s syndrome, theoretically develops NTM infection in easier situation. The image pattern of chest CT coincidentally presents as the classic pattern of NTM infection related Lady Windermere syndrome with underlying Sjögren’s syndrome.

Therefore, under the findings of chest-CT image, NTM by sputum culture result and clinical manifestation, she was diagnosed as Lady Windermere Syndrome [[1], [2], [3]] with underlying Sjögren’s syndrome.

Conflicts of interest

None of the authors has any conflicts of interest to declare.

Sources of funding

Nil.

Consent

This article (“Lady Windermere syndrome in Sjögren’s syndrome”) only contained one chest-CT image without neither patient’s name nor chart number. It is also completely de-identified file and anonymized without any photographs of the subject's face or other visual characteristics.

Authors contribution

Yu-Ling Pan and Hsien-Tzung Liao took care of this patient and wrote this manuscript, together.