Pulmonary-Renal vasculitic disorders: Differential diagnosis and management

Jara, Luis J.; Vera‐Lastra, Olga; Calleja, Maria C.

doi:10.1007/s11926-003-0038-0

Cited by 38 publications

(18 citation statements)

References 51 publications

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“…Currently, the primary therapeutic strategy used to treat GPS is plasma exchange, which may effectively remove the pathogenic antibodies in the blood and alleviate symptoms of the disease (15). Additionally, methylprednisolone serves an important role in inhibiting the formation of immune antibodies.…”

Section: Discussionmentioning

confidence: 99%

Goodpasture's syndrome in early pregnancy: A case report

Qin¹,

Song²,

Liu³

2017

Exp Ther Med

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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 ...

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Section: Discussionmentioning

confidence: 99%

Goodpasture's syndrome in early pregnancy: A case report

Qin¹,

Song²,

Liu³

2017

Exp Ther Med

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“…This may be true in DAH due to other immune causes. One could also make an analogy between DAH and acute glomerulonephritis, in which early treatment is a determinant of renal recovery [1,29,38,39]. For example, in a study of 107 patients with ANCA-associated glomerulonephritis, entry serum creatinine was one of the main predictive factors of chronic renal insufficiency [29].…”

Section: Discussionmentioning

confidence: 99%

Alveolar Haemorrhage in the Immunocompetent Host: A Scale for Early Diagnosis of an Immune Cause

et al. 2010

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Background: Diffuse alveolar haemorrhage (DAH) is a life-threatening condition due to immune and non-immune causes. Early identification of an underlying immune disorder is essential in order to initiate appropriate treatment. Objective: The purpose of this study was to identify early predictive factors of an immune cause of DAH. Methods: We conducted a retrospective study of 76 immunocompetent patients with DAH to identify early predictive factors of immune-related DAH using clinical, radiological and routine biological data available in the first 24 h after hospital admission. Results: Multivariate analysis identified 4 parameters which were independently associated with immune-related DAH: (1) onset of first respiratory symptoms ≧11 days, (2) fatigue and/or weight loss during the month prior to presentation, (3) arthralgias or arthritis and (4) proteinuria ≧1 g/l. A simplified scale was constructed using these variables, with an area under the receiver operating characteristic curve of 0.913, for the diagnosis of immune-related DAH. Conclusions: A simple diagnostic scale can be used to identify an immune-related cause of DAH in immunocompetent patients and may help guide treatment decisions such as initiation of steroid therapy on the day of admission.

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“…17 Diffuse alveolar hemorrhage has been reported in the course of SRC, and Bar et al proposed the term "scleroderma PRS" for this uncommon and fatal complication of SSc. 11 However, the pathogenesis and inciting factors of PRS in SSc remain largely unknown; different pathologies are supposed to be included in the pathogenesis of PRS in SSc from the clinicopathological fi ndings of the reviewed cases.…”

Section: Discussionmentioning

confidence: 99%

“…17 Because effi cacy of early intervention with high-dose corticosteroid has been reported in some scleroderma patients who devel- oped DAH or glomerulonephritis associated with the signs of small vessel vasculitis and DPC-induced glomerulonephritis, 12,26-28 high-dose corticosteroid may be effective for the treatment of scleroderma PRS with vasculitis, especially in MPO-ANCA positive cases, or Goodpasture-like syndrome. On the other hand, high-dose corticosteroid use (PSL ≥ 30 mg/day) has been suspected to be a risk factor or a trigger of SRC, 4 especially normotensive SRC, which often accompanies signs of TMA and DAH, in patients with early diffuse type SSc.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature

et al. 2007

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We describe three cases of acute renal failure with diffuse alveolar hemorrhage, which is designated pulmonary-renal syndrome (PRS), in systemic sclerosis (SSc) and review the literature to better define this rare but severe complication of SSc. The clinical course of three SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage are reported, and the literature published between 1967 and 2005 is reviewed following a PubMed search. Including our cases, a total of 19 SSc patients with acute renal failure and concomitant diffuse alveolar hemorrhage have been reported. Pulmonary-renal syndrome developing in SSc patients can be categorized clinicopathologically into three entities: PRS with thrombotic microangiopathy, PRS with small vessel vasculitides accompanied with SSc, and d-penicillamine-induced Goodpasture-like syndrome. Patients with scleroderma PRS with thrombotic microangiopathy, to which group our all patients belong, often developed diffuse alveolar hemorrhage after receiving high-dose corticosteroid therapy. Pulmonary-renal syndrome is a fatal complication of SSc and results from different pathogenic processes. Prompt differential diagnosis between the subsets is critical, because therapeutic strategy may differ in the use of high-dose corticosteroid and plasma exchange between the subsets of PRS. Clinical courses of the patients with PRS with thrombotic microangiopathy suggest that high-dose corticosteroid therapy is a trigger of diffuse alveolar hemorrhage in patients with diffuse SSc with signs of thrombotic microangiopathy.

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Pulmonary-Renal vasculitic disorders: Differential diagnosis and management

Cited by 38 publications

References 51 publications

Goodpasture's syndrome in early pregnancy: A case report

Goodpasture's syndrome in early pregnancy: A case report

Alveolar Haemorrhage in the Immunocompetent Host: A Scale for Early Diagnosis of an Immune Cause

Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature

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