Idiopathic pulmonary haemosiderosis treated by plasmapheresis.

Pozo-Rodrı́guez, Francisco; Freire-Campo, J M; Gutierrez‐Millet, Victor; C, Barbosa-Ayucar; Atauri, J Diaz de; Martı́n-Escribano, Pedro

doi:10.1136/thx.35.5.399

Cited by 17 publications

(5 citation statements)

References 5 publications

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“…There are several reports of temporary improvement after administration of azathioprine [4,10,161 whereas in the hands of others, it was without benefit [3]. Plasmapheresis has been also used in one reported case [11]. The weak points of the above studies were the short observation period, the small number of patients, and the lack of control by any means.…”

Section: Discussionmentioning

confidence: 92%

Prognostic criteria in idiopathic pulmonary hemosiderosis in children

1983

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An epidemiologic survey was undertaken of 30 children in whom idiopathic pulmonary hemosiderosis (IPH) had been identified. In determining the prognostic significance of various parameters, a clinical severity score was used. This included the year of onset of IPH, fever, difficulty in breathing, the severity of the anemia, the roentgenographic findings and therapeutic modalities such as transfusions, administration of steroids, antibiotic treatment and splenectomy. Eighteen patients had died. The results of this investigation suggest that: (1) The severity of the disease at its onset does not determine the survival; (2) Females survived longer; (3) Young age of the patients at the onset of IPH seems to carry a less favorable prognosis; (4) The common therapeutic modalities in use have not proved to be beneficial. We believe that some environmental insult to the developing lung in genetically predisposed persons may contribute to a higher morbidity in the younger patients.

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Section: Discussionmentioning

confidence: 92%

Prognostic criteria in idiopathic pulmonary hemosiderosis in children

1983

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“…In contrast, use of plasmapheresis has not been established in IPH. Although, F Pozo-Rodriguez et al reported a case of IPH in 1980 which was successfully treated with plasmapheresis suggesting a link to immunological pathogenesis of this disorder [ 12 ]. Thus, in the above clinical setting a single definitive diagnosis was difficult to establish.…”

Section: Discussionmentioning

confidence: 99%

Recurrent alveolar hemorrhage: How do you treat that which you cannot see?

Philipose

Siddiqui

Jiyoung

et al. 2018

Respiratory Medicine Case Reports

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Diffuse alveolar hemorrhage (DAH) is a rare and life-threatening event which is characterized by bleeding into the alveolar spaces of the lung. Etiology of DAH can be broadly divided into immune and non-immune mediated disease. In the absence of infection or malignancy, an immunological workup is required to find the cause of alveolar bleed. Rarely, there is a failure to establish a definitive etiology in patients with DAH. In those scenarios, patients who do not respond to steroids, plasmapheresis should be considered as a rescue treatment to prevent catastrophic outcomes. Herein, we present a unique case of a 48-year-old male admitted with DAH of unknown etiology completely recovered after empirical plasmapheresis.

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“…Following the diagnosis of IPH, immunosuppressive medications were used in 37/46 (80.4%) patients in cohort A. Corticosteroid (CS) was the most popular first-line medication 35/37 (94.6%). The other two patients were started on azathioprine (AZA) as first line therapy [ 17 , 20 ]. Ten out of 35 (28.6%) patients who initially received CS required a second-line medication.…”

Section: Reviewmentioning

confidence: 99%

Comparative Analysis of Adult Patients With Idiopathic Pulmonary Hemosiderosis and Lane-Hamilton Syndrome: A Systematic Review of the Literature in the Period 1971-2022

Saha¹,

Datar²,

Aiman³

et al. 2022

Cureus

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Idiopathic pulmonary hemosiderosis (IPH) causes diffuse alveolar hemorrhage (DAH) by a yet unknown mechanism. The coexistence of IPH and celiac disease (CD), also known as Lane-Hamilton syndrome (LHS), has been reported in both pediatric and adult patients. The objective of this study was to compare demographics, clinical and radiologic findings, treatment, and outcomes between adult patients with IPH and LHS. This is a systematic review of the literature. Multiple databases were searched using appropriate formulas to identify relevant articles. A total of 60 studies reporting 65 patients were included in the review. Forty-nine of these patients had IPH and 16 had LHS. The prevalence of anti-CD antibodies among tested patients was 13/22 (59%). The symptom onset and diagnosis of IPH occurred earlier in patients with LHS. The median delay in diagnosis was the same between the two groups (52 weeks). The classic triad was more likely to be present in patients with LHS. Only 20% of patients in the LHS cohort had any significant gastrointestinal (GI) symptoms at the time of IPH diagnosis. A gluten-free diet alone was effective in the majority of patients. Fewer patients in the LHS cohort received systemic corticosteroid than the IPH cohort. The recurrence and mortality in patients with LHS appear to be less than in the IPH cohort. The prevalence of CD is 25% in adult patients with IPH. Patients with LHS may have a milder course than patients without CD. Serologic testing for CD should be performed in all patients diagnosed with IPH.

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Idiopathic pulmonary haemosiderosis treated by plasmapheresis.

Cited by 17 publications

References 5 publications

Prognostic criteria in idiopathic pulmonary hemosiderosis in children

Prognostic criteria in idiopathic pulmonary hemosiderosis in children

Recurrent alveolar hemorrhage: How do you treat that which you cannot see?

Comparative Analysis of Adult Patients With Idiopathic Pulmonary Hemosiderosis and Lane-Hamilton Syndrome: A Systematic Review of the Literature in the Period 1971-2022

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