The prevalence and clinical significance of renal manifestations in patients with sarcoidosis, overall disease prognosis and management strategies

Abstract

Background: Sarcoidosis is a multisystem disease characterized by the formation of non-caseating granulomas, predominantly affecting the lungs, skin, and lymph nodes. Although renal failure is an uncommon manifestation, renal involvement in sarcoidosis primarily results from disruptions in calcium metabolism. These disturbances can lead to hypercalcemia, hypercalciuria, nephrolithiasis, nephrocalcinosis, and conditions such as glomerulonephritis or interstitial granulomatous nephritis (GIN). Early diagnosis and prompt treatment are crucial to restore renal function and prevent end-stage renal disease (ESRD) progression. This case report discusses a 29-year-old male diagnosed with refractory combined pulmonary (stage III) and cutaneous sarcoidosis, who presented with subcutaneous thickenings on the forearms and the dorsal surfaces of the hands. His condition is further complicated by chronic interstitial nephritis, hypercalcemia, nephrocalcinosis, and chronic kidney disease (CKD) (stage II, A2). He also experiences secondary arterial hypertension, secondary hyperuricemia, secondary erythrocytosis, dyslipidaemia, and osteoporosis due to severe intolerance toward first-line therapy. Additionally, the patient has been diagnosed with seronegative rheumatoid arthritis with high disease activity (DAS28), stage 1-2, and functional joint failure (level 2). He has experienced recurrent episodes of hypercalcemia and a single episode of perforated diverticulitis. This case exemplifies the complexity of managing multiple integrated serious health conditions.

Aim: This study aims to investigate and highlight the renal manifestations of sarcoidosis while examining its prevalence, clinical significance, prognosis, and management strategies.

Methods: An electronic search of online databases was conducted. The following databases were utilized: PubMed, ScienceDirect, Oxford Academic ProQuest, ATS Journals, UpToDate, and Frontiers. The analysis focused on studies addressing sarcoidosis, its renal manifestations, and therapeutic options. The search was limited to papers written in English.

Keywords: Sarcoidosis, Acute kidney injury (AKI), Extrapulmonary sarcoidosis, Granulomatous interstitial nephritis (GIN), End-stage renal disease (ESRD), Cutaneous sarcoidosis, Renal replacement therapy.

Conclusion: Renal sarcoidosis, though rare, often progresses to end-stage renal disease (ESRD) primarily due to calcium metabolism dysfunction, which can lead to chronic kidney disease (CKD). Granulomatous interstitial nephritis (GIN) is a classic pathological finding but may be clinically silent. Glucocorticoids are the first-line therapy, with steroid-sparing agents and anti-TNF-α (tumour necrosis factor-alpha) drugs used for resistant cases. Early diagnosis and treatment are essential to prevent progression to advanced CKD or the need for renal replacement therapies.