Sara Cardoso Fernandes
1* 
, Joana Marques
1 , Mário Pinto
2 , Mário Góis
1,3 , Helena Sousa
1,3 , Fernando Nolasco
1,4 1 Department of Nephrology, Hospital Curry Cabral, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal
2 Department of Pulmonology, Hospital Santa Marta, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal
3 Laboratory of Renal Morphology, Department of Nephrology, Hospital Curry Cabral, Centro Hospitalar Universitário de Lisboa Central, Lisboa, Portugal
4 Nova Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal
Abstract
Sjögren’s syndrome is a chronic inflammatory disorder mostly involving the exocrine glands. Extraglandular disease may occur in up to one quarter of patients. Kidney involvement is rare, more often manifested by tubular dysfunction secondary to tubulointerstitial nephritis. Primary glomerular disease is uncommon. The authors present the case of a 73-year-old woman with xerostomia and positivity for anti-Ro and anti-La antibodies admitted for acute kidney injury and exudative pleural effusion. Biopsy of salivary glands was compatible with Sjögren’s syndrome. Extraglandular involvement was also confirmed by renal and pleural deposition of AA-amyloid. The patient was started on prednisolone followed by azathioprine with rapid improvement of lung disease. However, due to progressing renal disease and clinical deterioration, prognosis was guarded and the patient died. We describe a case of secondary amyloidosis with systemic involvement and infrequent clinical manifestations, briefly reviewing the key aspects of Sjögren’s syndrome and AA-amyloidosis.
Implication for health policy/practice/research/medical education:
This clinical report describes a rare complication of Sjögren’s syndrome presenting with multisystem involvement and rare manifestations. This case highlights the importance of making a prompt diagnosis of inflammatory conditions in order to avoid the development of AA amyloidosis, a serious illness that lacks effective therapeutic options.
Please cite this paper as: Fernandes SC, Marques J, Pinto M, Góis M, Sousa H, Nolasco F. Renal failure and pleural effusion; a diagnostic challenge. J Nephropathol. 2023;12(3):e17309. DOI: 10.34172/jnp.2023.17309.