DIFFUSE ALVEOLAR HEMORRHAGE AS AN ATYPICAL PRESENTATION FOR IGA NEPHROPATHY: CASE REPORT

7 Feb 2025 12 a.m. 12 a.m.

WCN25-AB-1387, Poster Board= FRI-151

Introduction:

IgA nephropathy (IgAN) is the most common cause of primary glomerulonephritis in most developing countries. Most cases of IgAN are clinically restricted to the kidney, however diffuse alveolar hemorrhage as initial presentation is extremely rare but possible. Kidney-lung syndrome involving a combination of alveolar hemorrhage and rapidly progressive glomerulonephritis is the typical presentation of ANCA-positive vasculitis, but some other rare causes may present it as well, such as ANCA-negative vasculitis (e.g. IgAN). We present a case report of alveolar hemorrhage as initial presentation of IgAN.

Methods:

A 16 y-o, female, with no prior medical history. She started 5 days earlier with dyspnea and hemoptysis, receiving outpatient antibiotic therapy without improvement. She came to the emergency room with cough, dyspnea, and bloody expectoration, having tomographic findings compatible with diffuse alveolar hemorrhage (DAH) (Figure 1). Key laboratories included SCr 19.6 mg/dl, GFR 2.3 ml/min/1.73m2 (CKD-EPI), BUN 160 mg/dl, Na 138 mmol/L, K 7 mmol/L, C3 106 mg/dl, C4 24.1 mg/dl, Anti-MBG negative, Anti-DNA negative, P-ANCA negative, C-ANCA negative, urinalysis shows 30 erythrocytes per field and proteins 30mg/dl, and blood gas analysis with elevated anion gap metabolic acidosis. Due to suspicion of vasculitis, plasma exchange was prescribed and performed with a total plasma volume of 1797ml and 5 exchanges and the hemorrhage remitted. Renal biopsy showed IgA nephropathy with fibrocellular crescents, interstitial fibrosis grade III (>50%), tubulointerstitial nephritis and chronic arteriopathy (Figure 2 & 3). Patient showed no improvement on kidney function and was transferred to peritoneal dialysis as chronic therapy.

Figure 1 Presents computed axial tomography with a pattern compatible with diffuse alveolar hemorrhage

Figure 2. Presents a section stained with Jones methamine silver stain identifying a glomerulus with a fibrocellular crescents.

Results:

IgAN has a variety of renal and extrarenal manifestations. We presented a case of a female patient with IgAN and DAH, evidence regarding this combination is scarce. Simultaneous and sequential involvement have been described and kidney biopsy patterns include crescentic glomerulonephritis (GN), mesangio-proliferative GN, membranous GN and fibrillary GN.

Conclusions:

Treatment with systemic steroids, immunosuppression and plasma exchange have been used in similar cases.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.