ABSTRACT
IgA nephropathy (IgAN), characterized as immune complex-mediated glomerulonephritis, can occasionally manifest alongside the pulmonary-renal syndrome. Henoch-Schönlein purpura (HSP), an inflammatory condition affecting small vessels through leukocytoclastic vasculitis, exhibits a close association with IgA nephropathy. Nonetheless, HSP's infrequent complications encompass pulmonary hemorrhage. Notably, the onset of pulmonary hemorrhage can rapidly precipitate a grave decline in the patient's health status, carrying a potentially fatal outcome for both disorders. Moreover, the existing literature regarding this specific complication and its management, particularly among adults, remains relatively limited. We report a rare case of a 43-year-old male with acute renal failure secondary to IgA nephropathy associated with HSP, whose condition was further complicated by pulmonary hemorrhage. He was treated with extensive plasmapheresis, pulse steroids, rituximab, and cyclophosphamide, which led to the successful recovery of his kidney function. Recognizing the potential of various presentations can significantly contribute to early diagnosis and prompt treatment, potentially leading to an improved prognosis for these patients.
ABSTRACT
Pulmonary renal syndrome (PRS) is a combination of rapid progressive glomerulonephritis (RPGN) and diffuse alveolar hemorrhage (DAH) caused by a variety of immunological and non-immunological etiologies. The difficulty in identifying and reporting seronegative PRS cases could be attributed to the lack of specific immunological markers. Thus, we report a rare case of a 13-year-old boy who was initially diagnosed with idiopathic pauci-immune pulmonary capillaritis (IPIPC). A year later, his condition became complicated, and was referred for further workup. During his hospital stay, he underwent a renal biopsy that showed stage II membranous nephropathy (MN). He tested negative for immunological markers and a diagnosis of seronegative PRS was established. He responded well to the immunosuppression therapy with monthly follow-ups. As in our patient, PRS may manifest as acute renal failure symptoms and non-specific respiratory symptoms that require extensive workup. The severity of the disease is inferred from the renal function at the time of presentation. Management involves immunosuppression and treatment of the underlying condition, with dialysis dependency occurring in a significant percentage of patients and a high mortality rate, especially in critically ill and older patients. In conclusion, timely diagnosis and treatment are essential given the condition's rapid progression and high mortality rate.
ABSTRACT
INTRODUCTION: Patients with end-stage kidney disease (ESKD) suffer from functional iron deficiency where despite the presence of sufficient iron stores in the body, adequate iron is unavailable for heme synthesis. This study hypothesis was that in patients undergoing hemodialysis (HD), administration of intravenous (IV) ascorbic acid (AA) exerts a good effect on the management of anemia, either by increasing the mobilization of iron from tissue stores or acting as an antioxidant to overcome the inflammatory block and increase the erythropoietin sensitivity. METHODS: Fifty patients with ESRD who were on regular HD were included in the study. Patients' ferritin levels ranged from 500 to 1200 ng/mL with transferrin saturation of 30% or more. However, all patients were anemic and received erythropoietin therapy. Iron therapy was discontinued in the first group, whereas it was continued in the second group that received IV AA. RESULTS: A significant increase in the levels of Hb was observed in the second group after 6 months despite the decrease in ferritin levels in both the groups. Transferrin saturation decreased in both groups, the decrease being more in the first group. The levels of C-reactive protein (CRP) decreased in the second group, whereas these increased in the first group. CONCLUSIONS: Intravenous AA as an adjuvant therapy with iron exerts a favorable and significant effect on the Hb, serum ferritin, and CRP levels in patients with ESKD having anemia. The discontinuation of iron therapy only decreases the serum ferritin levels and does not improve the Hb or CRP levels.
