Key Clinical Message: Antinuclear antibody-negative full-house lupus nephritis though previously reported, is fairly uncommon. Some patients go on to develop antibodies later in the disease course. The presence of RO-52 antibody in this case suggests an underlying immunological cause. Swift management based on strong clinical suspicion can be life-saving to the patient.
Abstract: Lupus nephritis (LN) is a serious complication of systemic lupus erythematosus (SLE) and is more likely to progress to end-stage renal disease (ESRD). With the recent EULAR/ACR criteria mandating antinuclear antibody (ANA) positivity as an entry criterion, clinicians are faced with a diagnostic dilemma in diagnosing cases of seronegative SLE. We present the case of a 25-year-old female who presented with photosensitive malar rash, hair loss, oral ulcers, menorrhagia, and kidney dysfunction, suggestive of SLE. Her ANA tests were negative, raising doubts about the diagnosis. Biopsy was delayed owing to anemia and thrombocytopenia, and clinical judgment led to the patient being diagnosed with LN, with prompt treatment resulting in significant improvement. Renal biopsy subsequently confirmed the case as diffuse class IV LN with full-house nephropathy. This case highlights the limitations of relying solely on ANA positivity in diagnosing LN and underscores the need for a comprehensive diagnostic approach for SLE that incorporates clinical features, immunological markers, and patient demographics. ANA-negative SLE patients demand heightened clinical suspicion, especially when other diagnostic parameters align with the disease. Swift intervention with immunosuppressive therapy, as seen in this case, can be life-saving.
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http://dx.doi.org/10.1002/ccr3.9231 | DOI Listing |
Key Clinical Message: Non-lupus full house nephropathy is a rare entity that is still poorly understood. It can complicate post-transplant kidneys and result in a de novo process. Treatment is difficult but can be possibly achieved with optimization of immune suppression.
View Article and Find Full Text PDFClin J Am Soc Nephrol
June 2024
Department of Pathology and Medical Biology, University Medical Center, University of Groningen, Groningen, The Netherlands.
Key Points: Nonlupus full house nephropathy is a rare, complex entity: confusion arises by the low-quality evidence and the lack of consensus on nomenclature. This systematic review supports that systemic lupus erythematosus and nonlupus full house nephropathy are distinct clinical entities, with comparable outcomes. The identification of three pathogenetic categories provides further clues for a shared clinical and diagnostic approach to the disease.
View Article and Find Full Text PDFAm J Kidney Dis
August 2024
Department of Nephrology, Graduate School of Medicine, Chiba University, Chiba, Japan. Electronic address:
We present the case of a 61-year-old man who developed nephrotic syndrome as a result of syphilis-associated secondary membranous nephropathy (MN). The patient showed nephrotic syndrome remission following antibiotic treatment for syphilis alone. Pathologically, the target antigen of immune complexes accumulated on glomerular basement membranes (GBM) in secondary MN caused by syphilis has been reported to be neuron-derived neurotrophic factor (NDNF).
View Article and Find Full Text PDFLupus
May 2024
Laboratory of Renal Pathophysiology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
Background And Hypothesis: Brazil has the largest number of individuals of African descent outside Africa and a very admixed population. Among cases of lupus nephritis (LN) in the country, there are differences in incidence, and even in severity, depending on the location and characteristics of the population studied. The aim of this study was to describe the clinical and epidemiological characteristics of LN in Brazil, as well as to determine which of those characteristics would be risk factors for a poor renal prognosis.
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