Sarcoidosis presents in a variety of ways, but historically, renal involvement has been considered rare with an incidence of 0.7% and is seldom the presenting feature of the illness. Concomitant involvement of kidney and bone marrow is extremely rare. Atypical forms of presentation, such as in this case, may pose a true diagnostic challenge. A 20-year-old African-American male presented to the emergency department with vague symptoms including fatigue, malaise, anorexia, right-sided lower back pain, and nausea. Acute kidney injury was clearly evident, creatinine was 19.78 mg/dL (normal range 0.60-1.20 mg/dL), and BUN was 124.0 mg/dL (normal range 5.0-25.0 mg/dL). Laboratory results were also remarkable for leukopenia, microcytic anemia, hyperkalemia, anion gap metabolic acidosis, and non-PTH dependent hypercalcemia. Interestingly, urinalysis was equivocal and both chest x-ray (CXR) and abdominopelvic computed tomography (CT) scan were unrevealing. The patient was admitted to the hospital and required renal replacement therapy to stabilize his clinical condition while planning for a renal biopsy that was later performed. While awaiting pathological results, pancytopenia developed, and a bone marrow biopsy was then obtained. On further investigation, angiotensin-converting enzyme (ACE) turned out to be significantly elevated suggesting sarcoidosis. Renal biopsy showed moderate acute tubular injury, tubulitis, extensive interstitial edema, and infiltration by numerous non-caseating granulomas, which confirmed the diagnosis of sarcoidosis. Bone marrow histopathology revealed hypocellularity but no granulomatous infiltration. The patient remained largely asymptomatic throughout his hospital stay, with no signs or symptoms suggesting the involvement of other organs. High-dose corticosteroids were started and continued outpatient after discharge while still on hemodialysis. Pancytopenia resolved while on glucocorticoids and improvement in renal function was such that after roughly two months of steroids, renal replacement therapy was no longer necessary. Overall, kidney injury severe enough to require hemodialysis associated with pancytopenia in a previously healthy 20-year-old constitutes a rather rare sarcoidosis presentation. This highlights the importance of considering sarcoidosis as a possible cause of kidney and bone marrow dysfunction and emphasizes the need for timely biopsy to facilitate accurate diagnosis and early initiation of appropriate therapy to avoid delayed or inadequate care, especially considering that even severe damage is potentially reversible when identified early and treated promptly.
Keywords: acute renal failure; granulomatous interstitial nephritis; pancytopenia; renal sarcoidosis; sarcoidosis.
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