Coexisting nutcracker phenomenon and superior mesenteric artery syndrome in a patient with IgA nephropathy

Abstract

Rationale: Nutcracker and superior mesenteric artery (SMA) syndrome share the same pathogenesis, but the simultaneous occurrence of both diseases is quite rare. A combination of the nutcracker syndrome and IgA nephropathy has previously been reported. Herein, we report what we believe is the first case of coexisting nutcracker and SMA syndrome in a patient with IgA nephropathy. Patient concerns: A 15-year-old Chinese boy who was diagnosed with IgA nephropathy at 8 years of age presented with gross hematuria, fatigue, anorexia, nausea, and recurrent abdominal distension for 1 week without any obvious evidence of preceding infection. Laboratory data showed macroscopic hematuria, heavy proteinuria, and relatively normal renal function. Doppler ultrasonography and upper gastrointestinal gastrografin study were performed, respectively. Since his renal function deteriorated after admission, repeated renal biopsy was performed. Diagnoses: IgA nephropathy with nutcracker phenomenon and SMA syndrome. Intervention: Immunosuppressive therapy combined with conservative therapy for superior mesenteric artery syndrome. Outcomes: One month later, his abdomen symptoms such as anorexia and abdominal distension eased a lot with body weight increase of about 3 kg. After 6 months of follow-up, his body weight increased to 57 kg, serum creatinine decreased to 63 μmol/L, and urine microscopy showed 75.5 RBC/high-power field with 0.3 g urine protein per day. Lessons: Although the association between vascular compression and IgA nephropathy (IgAN) has not been elucidated yet, combination of nutcracker syndrome and SMA syndrome should be considered in patients with IgAN. The combination may increase the complexity of the disease, and renal biopsy should not be hesitated for differential diagnosis.

Associations of left renal vein entrapment with IgA nephropathy and Henoch–Schönlein purpura nephritis

“…have shown that the prevalence of LRV entrapment in IgAN was 6.8% (10/146) in Japanese patients [ 12 ]. Recently, we have reported a rare case of concurrent nutcracker and SMA syndrome in a 15-year-old Chinese male juvenile diagnosed with IgAN [ 13 ]. Considering the relatively common combination of LRV entrapment and IgAN, a possible causal relationship between them has been raised [ 6 , 12 ].…”

Case Report: An Unusual Case of Nutcracker Syndrome and Literature Review

“…Therefore, the terminology NCS should be restricted to patients with typical clinical symptoms and signs, especially hematuria, low back pain, proteinuria, male varicocele, and female pelvic congestion (3). NCS is an important diagnosis of unexplained hematuria due to the lack of clear diagnostic procedures and standards and the possibility of the coexistence of other glomerular diseases (5)(6)(7)(8), and its diagnosis is often delayed. The diagnosis of NCS is mainly based on imaging examinations of patients with typical symptoms and signs, including US, CTA, magnetic resonance angiography (MRA), and intravascular ultrasound (IVUS) (9).…”

Síndrome de Wilkie y del Cascanueces superpuestos a un caso de dispepsia funcional

“…(7,8) The concurrence of both syndromes is rare. (9,10,11) It has been documented in the scientific literature the association with other compressive syndromes of different vascular or gastrointestinal structures such as the arcuate ligament or the left iliac vein (May-Thurner syndrome) (12) as well as with IgA nephropathy. (9) In our case, the patient did not display any symptoms of the Nutcracker syndrome, it being an incidental finding in the imaging study.…”