Introduction:
Nephritic syndrome is a clinical condition characterized by hematuria, elevated blood pressure, reduced urine output, and edema. Thrombotic microangiopathy (TMA) is a pathological condition that presents with thrombocytopenia, microangiopathic hemolytic anemia (MAHA), and organ damage, often appearing as nephritic syndrome. TMA is frequently linked to renal failure. Timely and aggressive treatment can help in improving renal function. In cases where kidney biopsy is not feasible due to shrunken kidneys, diagnosing TMA conclusively may be challenging. However, estimating ADAMTS13 activity can aid in TMA diagnosis for further management in such situations.
Methods:
This was a retrospective observational study in patients who had presented with nephritic syndrome and hypocomplementemia in the Department of Nephrology, Dr. D. Y. Patil Medical College and Hospital. ADAMTS13 activity and patients' levels, complement level, and renal biopsy were retrieved from medical records and evaluated.
Results:
A total of seventeen cohorts were enrolled. Serum complement levels were low in thirteen (76.4%) out of seventeen patients; C3 level (Mean ± SD) was 71.5 ± 26.9 mg/ dL (Ref. range 80-120 mg/dL) and C4 levels of all patients were within normal limits [28.1± 11.7 mg/ dL (Ref. range 10-40 mg/dL)]. Ten (58.8%) patients had low ADAMTS 13 levels of 0.4±0.31 (Ref. Range 0.45 – 1.3 IU/ mL). Five (29.1%) out of seventeen were biopsied. Interstitial fibrosis and tubular atrophy was seen, 30-45% (in 60% of samples) and >50% (in 40%) of five biopsied samples respectively. Medial thickening of arteries and changes of TMA were seen in all samples.
Conclusions:
In nephritic syndrome, if complement levels are observed to be low, then further assessment of complement pathway abnormalities should be piloted. This is crucial as it can have a significant impact on the management of these patients who are prospective kidney transplant recipients or having post-renal transplant status.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.