Unidade Nefrologia Pediátrica, Área da Mulher, da Criança e do Adolescente - CHLC, EPE
- Lisbon Clinical Nephrology Update
- Mayo Nephrology Collaborative Group | Nefrologia Hospital Fernando Fonseca
- 16 e 17 de Junho de 2017, Lisboa
Clinical case: The authors present a case of a 14-year-old Pakistani girl with poorly controlled type 1 diabetes since the age of six (HbA1c greater than 15%), Hashimoto’s thyroiditis with hypothyroidism, severe dyslipidemia and iron deficiency anemia. She was referred to our pediatric nephrology unit with nephrotic-range proteinuria (124mg/m2/hour) without hypoalbuminemia. She had normal blood pressure and renal function. Renal and abdominal ultrasonography and doppler were normal. There was a decreased proteinuria with ACE inhibitors and metabolic control. However, this improvement was very inconsistent by the low adherence to therapeutics. An immunological study was performed after the appearance of self-limited malar rash coincident with sun exposure. Positive anti-dsDNA and anti-nuclear antibodies 1/320 with Anti RNP/Sm and Anti RNP-/A+ were found. These values were not confirmed later. Renal biopsy revealed diabetic nephropathy IIb and type II membranous nephropathy (MN) with a high immunological standard on immunofluorescence (IgG, IgM, K and lambda light chains). No other manifestation of autoimmune disease emerged in the follow-up.
Discussion: Membranous nephropathy is an immune-complex mediated disease, in which antibodies react against endogenous antigens in situ. In childhood, MN is mainly secondary to an underlying pathology. The idiopathic form is a rare cause of asymptomatic proteinuria or nephrotic syndrome and a diagnosis of exclusion. The diagnostic and therapeutic approach to MN is challenging because of its variable manifestation and inconsistent response to treatment. ACE inhibitors, ARBs, corticosteroids and other immunosuppressive therapies such as cyclosporine may be considered. The coexistence of histological findings of diabetic nephropathy and MN with evidence of autoimmune activity raises doubts about whether it is one disease (MN secondary to diabetes) or two diseases (diabetic nephropathy and primary MN). In this case, considering the age, sociocultural context with poor therapeutic adherence and difficult metabolic control, the treatment options are even more limited and under discussion.
Palavras Chave: FSGS, Membranous GN, Mixed GN, Mesangiocapillary GN, Minimal Change GN, Nephrotic Syndrome, SLE