Goodpasture's syndrome in early pregnancy: A case report

Abstract

Abstract. Goodpasture's syndrome (GPS) presenting during pregnancy is extremely rare and patients exhibiting hemoptysis and renal dysfunction in early pregnancy are uncommon. The present study reports the case of a 17-year-old diagnosed with GPS during the thirteenth week of pregnancy. Prompt recognition and immediate treatment (steroids and plasma exchange) was initiated. Renal function normalized following treatment and anti-GBM antibody became negative during hospital stay. However, due to irregular follow-up, the patient eventually succumbed. The case highlights that the current treatment plan for GPS should be revised to improve the outcome of pregnancy. In addition, it determines how important it is for obstetricians to consider whether a pregnancy should be terminated to improve the chances of survival of pregnant patients with GPS. IntroductionGoodpasture's syndrome (GPS) is an immuno disorder characterized by pulmonary hemorrhage, glomerulonephritis and the presence of the antiglomerular basement membrane (anti-GBM) antibody (1,2). The incidence of GPS is <1 case/1,000,000 (3). Combination therapy is recommended for the initial management of the disease, including corticosteroid therapy, cytostatic therapy and plasmapheresis. However, patients have poor prognosis with current treatments and the mortality rate has been estimated as 11% (4). The etiology of Goodpasture's Syndrome remains unknown; however, the development of the disease following environmental exposures including hydrocarbon fumes, metallic dust or tobacco smoke has been reported (5). GPS occurs due to antibody and T-lymphocyte reactivity to the NC1 domain of the α3 chain of type IV collagen (6).Women presenting with GPS in the early period of pregnancy are uncommon and the present case study reports an unusual case of GPS in the early stage of pregnancy. In the present study, the patient received hemodialysis and methylprednisolone, prior to succumbing due to sustained, irreversible renal failure. The majority of pregnancies are terminated prior to term delivery to prevent worsening of conditions (7,8). Thus, current therapeutic strategies used to treat GPS should be revised in order to improve the survival rate of pregnant patients with GPS. Case reportA 17-year-old female was hospitalized at the Traditional Chinese Medicine Hospital of Guizhou (Guiyang, China) on 9th December 2013 due to no urination and hemoptysis for 3 days in the thirteenth week of pregnancy. The patient had history of double lower limb swelling lasting from the onset of pregnancy, which was treated with furosemide in Qianxi County Central hospital. The patient's medical history prior to pregnancy was normal; however, the patient had a 2-week history of cough prior to admission and experienced an episode of brown-colored urine 1 week later. The patient did not experience fever, dysuria or oliguria at that time. The patient visited Qianxi County Central hospital (Quianxi, China) on 21th October 2013 and was diagnosed with acute renal insufficiency. Serum was analysed ...

Pregnancy Outcomes in Systemic Vasculitides

“…About 30–40% of cases with final diagnosis during pregnancy before the third trimester. Onset rarely reported in postpartum Flares reported in 25–50% of patients, mostly with lung infiltration/asthma exacerbation or neuropathy (90%) Cardiac involvement associated with poor prognostic Preeclampsia: rare (< 5%) Fetal loss: 10–15% (up to 50% if disease is active) Therapeutic abortions: 5–10% One maternal death from myocardial infarction and one death from cardiac failure 3 months postpartum Preterm deliveries: 10–40% Cesarean deliveries: 15–40% Rare cases of intrauterine deaths IUGR: 10–25% LBW: 10–30% Immune complex vasculitides Anti-glomerular basement membrane (anti-GBM) disease [ 41 – 44 ] Most cases described disease onset during pregnancy, especially in the second trimester, with some pregnancies resulting in therapeutic abortions Rare case reports described disease onset in postpartum One maternal death (second trimester) is reported, but the patient did not attend follow-up visits after initial improvement with plasmapheresis and glucocorticoids One case report described the recurrence of anti-GBM disease in a subsequent pregnancy Almost all deliveries described resulted in preterm labor Variable outcomes have been described with some patients remaining dialysis-dependent or undergoing renal transplantation and others returning to a normal renal function (less common) None had permanent pulmonary disease Gestational diabetes: 38% (all on glucocorticoids) Preeclampsia: 25% Cesarean deliveries: 50% IUGR with LBW: 75–80% 2 newborns were described as having anti-GBM antibodies without any manifestations 1 fetus had severe complications associated with prematurity (cerebral hemorrhage) Very few cases reported (≤ 20 case reports) Cryoglobulinemic vasculitis [ 2 , 5 , 45 – 47 ] Diagnosis was made before conception in two-thirds of the described cases 2 pregnancies with onset of vasculitis during pregnancy (palpable macular rash and hypertension, glomerulonephritis) 3 vasculitis flares occurred (mononeuritis multiplex, skin involvement, hypertension with proteinuria and arthralgias) (50%) One case noted an improvement in vasculitis symptoms during pregnancy Good maternal outcomes: 4/6 (67%) Preeclampsia described in one pregnancy One miscarriage because of vasculitis flare (1/6) Preterm delivery: 1/5 (31 weeks) (20%) Cesarean deliveries: 3/5 (60%) LBW: 1/5 (premature) One fetus (mother with type 1 cryoglobulinemia) had transient cold-induced cutaneous lesions (resolved with warming and clearance of maternal IgG) Few cases described (6 pregnancies) Avoidance of cold temperatures in neonates and mothers with cryoglobulinemia is advised to avoid precipitation of cryoglobulins IgA...…”

Anti-glomerular Basement Membrane Glomerulonephritis During the First Trimester of Pregnancy

“…There are only a few case reports of anti-GBM GN disease during pregnancy in the literature, especially in the early phase of pregnancy ( 4 ). We summarized the reports of anti-GBM GN in pregnancy in Table 2 ( 5 - 13 ). There have been some reports of a severe renal outcome and prognosis in pregnancy ( 5 - 7 , 9 ).…”

“…We summarized the reports of anti-GBM GN in pregnancy in Table 2 ( 5 - 13 ). There have been some reports of a severe renal outcome and prognosis in pregnancy ( 5 - 7 , 9 ). For example, Qin et al reported both maternal and fetal death in a case of Goodpasture's syndrome during pregnancy ( 5 ).…”

Goodpasture syndrome and anti-glomerular basement membrane disease

“…De novo anti-GBM disease in pregnancy is rare and just few cases have been described (93)(94)(95)(96)(97)(98)(99). Most patients presented after the first trimester with acute kidney injury, hematuria, proteinuria and anemia (98).…”