NDT Advance Access originally published online on January 18, 2007
Nephrology Dialysis Transplantation 2007 22(5):1485-1486; doi:10.1093/ndt/gfl823
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Nodular pulmonary amyloidosis and Sjõgren's syndrome in a patient treated with intermittent haemodialysis (IHD)
Email: leszek.niepolski{at}avitum.com.plSir,
Nodular pulmonary amyloidosis associated with Sjõgren's syndrome is a very rare condition [1]. In our practice, such a case occurred in a patient with chronic renal failure.
Case
A 53-year-old woman was admitted to the hospital with a 3-month history of malaise, general weakness, high blood pressure and progressive renal failure. Laboratory tests at admission showed elevated erythrocyte sedimentation rate 112 mm/h. Serum creatinine level was 5.7 mg/dl. Electrophoresis of serum proteins revealed hypergammaglobulinaemia. The 24-h urine protein output was 2.73 g. On renal ultrasound, the kidneys measured 9.2 and 10.0 cm on the right and left respectively, with increased echogenicity of renal cortex. Due to end-stage renal disease at the time of diagnosis, renal biopsy was not performed. A month later she was readmitted to the hospital because of dyspnoea and worsening kidney function with serum creatinine level of 7.0 mg/dl. Renal replacement therapy with intermittent haemodialysis was started. She complained of eye itching and sore swallowing. Physical examination revealed dryness of the eyes, as well as reddening and dryness of buccal cavity. The Schirmer's test was positive. The rheumatologist recognized Sjõgren's syndrome. Her lungs were clear at auscultation. The chest radiograph showed solitary nodular shadows in both the lungs (Figure 1A). Computer tomography scan showed 5–6 hemispherical nodules, from 5–40 mm in diameter, in each lung. Serological tests for autoimmune diseases revealed rheumatoid factor 1:80 (normal range <1:80), perinuclear and cytoplasmatic antineutrophil cytoplastic antibodies negative, antinuclear antibodies 1:160 (normal range <1:20), and antinuclear antibodies profile with increased anti-SS-A and anti-SS-B, 5.02 and 6.03, respectively (result >1.0 positive). Bence-Jones protein was not detected. Bronchofiberoscopy revealed no endobronchial lesions. The bronchial washings were negative for malignant cells. Bacterial culture of the washings, including tuberculosis, did not reveal bacterial growth. Bronchial biopsy revealed features of chronic bronchitis.
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After 2 years of treatment with intermittent haemodialysis, a thoracotomy with enucleation of the nodules from the right lung was done. Histological examination showed massive deposits of amyloid (Figure 1B). Based on the histological examination, radiological pictures and pulmonological consultation, we recognized diffusive nodular pulmonary amyloidosis. At present, the patient is typically haemodialysed for 4 hours per session, three times a week and is on a waiting list for renal transplantation.
Discussion
Several studies indicate that the majority of patients with primary Sjõgren's syndrome have pulmonary problems [2]. Our report presents the case of nodular pulmonary amyloidosis associated with Sjõgren's syndrome. The current case illustrates the fact that renal insufficiency makes the diagnosis of Sjõgren's syndrome and nodular pulmonary amyloidosis more difficult [3]. The thoracotomy with a subsequent histological examination was the final diagnostic procedure.
In summary, the co-existence of pulmonary nodules and Sjõgren's syndrome should incline us towards a diagnosis of localized amyloidosis. Thoracotomy is a good diagnostic procedure to specify the character of tumour changes in lungs.
Conflict of interest statement. None declared.
1B. Braun Avitum Poland
Dialysis Center
Nowy Tomysl
2Chair and Department of Nephrology
Transplantology and Internal Diseases
Karol Marcinkowski University
of Medical Sciences
Poznan
3Department of Clinical Pathology
Karol Marcinkowski University of
Medical Sciences
Poznan
Poland
References
- Gillmore JD and Hawkins PN. (1999) Amyloidosis and the respiratory tract. Thorax 54:444–451.
[Free Full Text] - Gudbjornsson B, Hedenstrom H, Stalenheim G, Hallgren R. (1991) Bronchial hyperresponsiveness to methacholin in patients with primary Sjõgren's syndrome. Ann Rheum Dis 50:36–40.
[Abstract/Free Full Text] - Sung JM, Kuo SC, Guo HR, Chuang SF, Lee SY, Huang JJ. (2006) The role of oral dryness in interdialytic weight gain by diabetic and non-diabetic haemodialysis patients. Nephrol Dial Transplant 21:92521–2528.
[Abstract/Free Full Text]
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