Case Report: Disease Mechanisms and Medical Management of Calcium Nephrolithiasis in Rheumatologic Diseases

Overview

Journal BMC Urol

Publisher Biomed Central

Specialty Urology

Date 2023 Mar 24

PMID 36959633

Authors

Omar Osman

Susan Manzi

Mary Chester Wasko

Barbara A Clark

Affiliations

Soon will be listed here.

Abstract

Background: Nephrolithiasis as a feature of rheumatologic diseases is under recognized. Understanding presenting features, diagnostic testing is crucial to proper management.

Case Presentation: A 32 year old woman with a history of recurrent complicated nephrolithiasis presented to a rheumatologist for a several month history of fatigue, dry eyes, dry mouth, arthralgias. She had a positive double-stranded DNA, positive SSA and SSB antibodies. She was diagnosed with Systemic Lupus erythematosus (SLE) and Sjogren's syndrome and was started on mycophenalate mofetil. Of relevance was a visit to her local emergency room 4 years earlier with profound weakness with unexplained marked hypokalemia and a non-anion gap metabolic acidosis. Approximately one year after that episode she developed flank pain and nephrocalcinosis. She had multiple issues over the ensuing years with stones and infections on both sides. Interventions included extracorporeal shockwave lithotripsy as well as open lithotomy and eventual auto-transplantation of left kidney for recurrent ureteric stenosis. 24 h stone profile revealed marked hypocitraturia, normal urine calcium, normal urine oxalate and uric acid. She was treated with potassium citrate. Mycophenolate was eventually stopped due to recurrent urinary tract infections and she was started on Belimumab. Because of recurrent SLE flares, treatment was changed to Rituximab (every 6 months) with clinical and serologic improvement. Her kidney stone frequency gradually improved and no further interventions needed although she continued to require citrate repletion for hypocitraturia.

Conclusions: Nephrolithiasis can be a prominent and even presenting feature in Sjogrens syndrome as well as other rheumatologic diseases. Prompt recognition and understanding disease mechanisms is important for best therapeutic interventions for kidney stone prevention as well as treatment of underlying bone mineral disease.

References

Evan A, Lingeman J, Coe F, Shao Y, Miller N, Matlaga B . Renal histopathology of stone-forming patients with distal renal tubular acidosis. Kidney Int. 2007; 71(8):795-801. DOI: 10.1038/sj.ki.5002113. View

Zanuto A, Bueno T, Delfino V, Mocelin A . Nephrocalcinosis in a patient with Sjögren's syndrome/systemic lupus erythematosus. Rev Assoc Med Bras (1992). 2012; 58(3):279-80. View

Sayers J, Hynes A, Srivastava S, Dowen F, Quinton R, Datta H . Successful treatment of hypercalcaemia associated with a CYP24A1 mutation with fluconazole. Clin Kidney J. 2015; 8(4):453-5. PMC: 4515887. DOI: 10.1093/ckj/sfv028. View

Ramos-Casals M, Tzioufas A, Font J . Primary Sjögren's syndrome: new clinical and therapeutic concepts. Ann Rheum Dis. 2004; 64(3):347-54. PMC: 1755414. DOI: 10.1136/ard.2004.025676. View

Trinchieri A, Lizzano R, Castelnuovo C, Zanetti G, Pisani E . Urinary patterns of patients with renal stones associated with chronic inflammatory bowel disease. Arch Ital Urol Androl. 2002; 74(2):61-4. View

Tebben P, Singh R, Kumar R . Vitamin D-Mediated Hypercalcemia: Mechanisms, Diagnosis, and Treatment. Endocr Rev. 2016; 37(5):521-547. PMC: 5045493. DOI: 10.1210/er.2016-1070. View

Worcester E . Stones from bowel disease. Endocrinol Metab Clin North Am. 2002; 31(4):979-99. DOI: 10.1016/s0889-8529(02)00035-x. View

Garza-Alpirez A, Arana-Guajardo A, Esquivel-Valerio J, Villarreal-Alarcon M, Galarza-Delgado D . Hypokalemic Paralysis due to Primary Sjögren Syndrome: Case Report and Review of the Literature. Case Rep Rheumatol. 2017; 2017:7509238. PMC: 5556603. DOI: 10.1155/2017/7509238. View

Moutsopoulos H, CLEDES J, Skopouli F, Elisaf M, Youinou P . Nephrocalcinosis in Sjögren's syndrome: a late sequela of renal tubular acidosis. J Intern Med. 1991; 230(2):187-91. DOI: 10.1111/j.1365-2796.1991.tb00429.x. View

10.

Curhan G, Willett W, Speizer F, Stampfer M . Intake of vitamins B6 and C and the risk of kidney stones in women. J Am Soc Nephrol. 1999; 10(4):840-5. DOI: 10.1681/ASN.V104840. View

11.

Curhan G, Willett W, Speizer F, Spiegelman D, Stampfer M . Comparison of dietary calcium with supplemental calcium and other nutrients as factors affecting the risk for kidney stones in women. Ann Intern Med. 1997; 126(7):497-504. DOI: 10.7326/0003-4819-126-7-199704010-00001. View

12.

Coe F, Evan A, Worcester E . Kidney stone disease. J Clin Invest. 2005; 115(10):2598-608. PMC: 1236703. DOI: 10.1172/JCI26662. View

13.

Kumar V, Lieske J . Protein regulation of intrarenal crystallization. Curr Opin Nephrol Hypertens. 2006; 15(4):374-80. DOI: 10.1097/01.mnh.0000232877.12599.f4. View

14.

Basok A, Haviv Y, Rogachev B, Vorobiov M . Renal Tubular Acidosis Type I with Prominent Hypokalemia and Nephrolithiasis as a Presentation of Sjögren's/Systemic Lupus Erythematosus Disease. Case Rep Nephrol Dial. 2021; 11(2):247-253. PMC: 8436655. DOI: 10.1159/000515050. View

15.

Cherif E, Hassine L, Kaoueche Z, Khalfallah N . Osteomalacia as inaugural manifestation of Sjögren syndrome. BMJ Case Rep. 2013; 2013. PMC: 3822150. DOI: 10.1136/bcr-2013-201052. View

16.

Bianchi L, Gaiani F, Bizzarri B, Minelli R, Cortegoso Valdivia P, Leandro G . Renal lithiasis and inflammatory bowel diseases, an update on pediatric population. Acta Biomed. 2018; 89(9-S):76-80. PMC: 6502195. DOI: 10.23750/abm.v89i9-S.7908. View

17.

Krieger N, Bushinsky D . The relation between bone and stone formation. Calcif Tissue Int. 2012; 93(4):374-81. PMC: 3625692. DOI: 10.1007/s00223-012-9686-2. View

18.

Dormohammadi Toosi T, Naderi N, Movassaghi S, Seradj M, Khalvat A, Shahbazi F . Secondary Sjogren's syndrome presenting with hypokalemic periodic paralysis. Oxf Med Case Reports. 2015; 2014(8):135-7. PMC: 4369992. DOI: 10.1093/omcr/omu052. View

19.

Jirackova J, Hyspler R, Alkanderi S, Pavlikova L, Palicka V, Sayer J . Novel CYP24A1 Mutation in a Young Male Patient with Nephrolithiasis: Case Report. Kidney Blood Press Res. 2019; 44(4):870-877. DOI: 10.1159/000500922. View

20.

Bossini N, Savoldi S, Franceschini F, Mombelloni S, Baronio M, Cavazzana I . Clinical and morphological features of kidney involvement in primary Sjögren's syndrome. Nephrol Dial Transplant. 2001; 16(12):2328-36. DOI: 10.1093/ndt/16.12.2328. View