IgA-dominant post-infectious glomerulonephritis presenting as a fatal pulmonary-renal syndrome

Abstract

Over the last decades, post-infectious glomerulonephritis underwent major changes in its epidemiology, pathophysiology, and outcomes. We are reporting a case of IgA-dominant post-infectious glomerulonephritis (IgA-PIGN) presenting as a fatal pulmonary-renal syndrome. An 86-year-old Filipino man presented with worsening dyspnea, hemoptysis, and decreased urine output over 2 weeks. Past medical history is significant for hypertension, chronic kidney disease stage III, and pneumonia 3 weeks prior treated with intravenous cefazolin for methicillin-sensitive Staphylococcus aureus bacteremia. Physical examination was remarkable for heart rate of 109/min and respiratory rate of 25/min saturating 99% on 3 liters via nasal cannula. There were bibasilar rales in the lungs and bilateral ankle edema. A chest radiograph showed bibasilar opacifications. Blood work was significant for hemoglobin of 8.3 g/dL and creatinine of 9.2 mg/dL (baseline of 1.67). TTE showed EF 55%. Urinalysis revealed large blood and red blood cell casts. Kidney ultrasound showed bilateral echogenicity compatible with renal disease. Pulse methylprednisolone therapy and hemodialysis were initiated with patient’s condition precluding kidney biopsy. Serology workup for rapidly progressive glomerulonephritis was negative. On day 7, the patient required mechanical ventilation; bronchoscopy showed alveolar hemorrhage and plasmapheresis was initiated. Renal biopsy revealed IgA-PIGN with endocapillary and focal extracapillary proliferative and exudative features. IgA-PIGN occurs in diabetic elderly (mean age of 60 years), 0–16 weeks after an infection mainly by Staphylococcus. However, this nondiabetic patient had normal complement IgA-PIGN with fatal pulmonary-renal syndrome. Understanding the pathogenesis and identifying the nephrotoxic bacteria species and the aberrant IgA molecule will open new insights toward prevention and treatment.

Clinicopathological and prognostic study of IgA-dominant postinfectious glomerulonephritis

“…Its pathogenesis was similar to that of active IgA nephropathy, and the clinical and pathological manifestations were highly similar. Marc Saad et al [ 8 ]also reported a case with pulmonary-renal syndrome as the main manifestation. The above study found some difficulty in differentiating IgA-dominant postinfectious glomerulonephritis when the presentation is atypical from other glomerulonephritis, especially primary IgA nephropathy, which can have similar light microscopy and immunofluorescence manifestations, but the treatment options for the two are different, and the prognosis of patients with IgA-dominant postinfectious glomerulonephritis is often reported worse [ 15 – 17 ].…”

Diffuse alveolar hemorrhage in IgA nephropathy: case series and systematic review of the literature

“…Five IgA-mediated syndromes presenting with PRS need to be distinguished from IgAN-related DAH. These include HSP, 58 IgAN-ANCA vasculitis overlap syndrome, IgA-dominant post-infectious glomerulonephritis (IgA-PIGN) 63,64 IgA-variant Goodpasture's syndrome [53][54][55][56][57] and IgA-producing monoclonal gammopathy. 52 HSP is characterized by a tetrad of cutaneous purpura, arthritis, abdominal pain and nephritis.…”

“…66 IgA-PIGN is a recently described entity that can present with pulmonary edema or DAH. 63,64 The older age, prevalence of diabetes, preceding staphylococcal infection as a trigger, low complement levels, acute renal failure at presentation with diffuse proliferative glomerulonephritis and diffuse C3 > IgA on IIF, j > k light chains and subepithelial deposits on IIF help distinguish these entities. 63,69 IgA-variant GPS is defined by the linear pattern on IIF staining of the kidneys and presence of a monoclonal IgA-j circulating antibody.…”

Infection-related immunoglobulin A nephropathy or IgA-dominant postinfectious glomerulonephritis; what is in a name?

“…In 27 (60%) cases, both chains were deposited equally; however, in 16 (35.5%) cases, lambda light chain staining was dominant. EM study showed that sub-epithelial, sub-endothelial, and mesangial areas were the most common sites of Ig deposition (29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41)(42).…”