A diagnostician's field guide to crystalline nephropathies.

The kidney's role in filtration of blood and production of urine occurs via a combination of size and charge filtration at the glomerular basement membrane and resorption and excretion of molecules through a complex tubular system embedded within an ion gradient. This delicate system provides the kidney with a unique propensity for substrate saturation and crystal nucleation within the nephron. While crystalline nephropathies may seem exotic to the uninitiated, they are comprised of easily recognizable morphologies and generally lack complicated classification schemas. Additionally, unlike many intrinsic kidney diseases, crystalline nephropathies are often associated with systemic conditions that, upon further investigation, may elucidate critically important information. This review focuses on practical, diagnostically relevant and high yield information that can be utilized by diagnosticians. Our hope is to equip the reader who reviews renal tissue with a practical toolkit that they feel empowered to use when faced with crystal formation in a kidney biopsy, pre-implantation biopsy, or nephrectomy specimen. Short Abstract The kidney's role in filtration of blood and production of urine provides a unique propensity for substrate saturation and crystal nucleation within the nephron. While crystalline nephropathies may seem exotic to the uninitiated, they are comprised of easily recognizable morphologies and generally lack complicated classification. Additionally, crystalline nephropathies are often associated with systemic conditions that, upon further investigation, may elucidate critically important information.

Collapsing glomerulopathy: a 30-year perspective and single, large center experience.

Collapsing glomerulopathy (CGP) is a pattern of kidney injury seen on renal biopsy with multiple associations and etiologies. It is most commonly described in African-Americans and others with recent African ancestry. The disease is rapidly progressive and often presents with abrupt onset of renal failure and nephrotic-range proteinuria. Since its description 30 years ago, this entity has transformed from a morphologic diagnosis typically seen in the setting of HIV infection to a complicated diagnosis with numerous etiologies, many of which are associated with underlying apolipoprotein L1 (APOL1)-risk variants or other genetic disorders. We review the evolution of CGP, and its history and proposed pathomechanisms. We also present the disease spectrum from our experience with emphasis on recognizing the lesion, distinguishing from mimics and linking the histopathological pattern to a specific cause. Our understanding continues to evolve as clinicians and scientists work toward a more complete understanding of the molecular pathways of injury in this disease and how these might be disrupted for therapeutic purposes. Much still remains to be discovered in CGP as the molecular underpinnings leading to disease are still not completely understood and no effective treatment exists despite the high morbidity. Based on this rapid evolution, CGP is a modern template of how we diagnose and think about kidney disease. The story of CGP represents the current shift in nephrology and nephropathology from morphology-alone-based diagnosis to a comprehensive approach including molecular diagnostics. We believe this new, holistic approach will lead to pathogenesis-centered diagnoses that will help to individualize risk stratification and treatment protocols.