Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature

Saha, Biplab K.; Bonnier, Alyssa; Saha, Santu; Saha, Baidya Nath; Shkolnik, Boris

doi:10.1007/s10067-022-06104-3

Cited by 10 publications

(32 citation statements)

References 109 publications

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“…Although hemoptysis is common in adult patients, pediatric patients may not have hemoptysis due to inadvertent swallowing of the sputum. Systemic symptoms affect approximately half of the patients [ 86 ]. The classic triad consists of hemoptysis, radiologic chest abnormalities, and anemia.…”

Section: Reviewmentioning

confidence: 99%

“…Systemic CS represents the first-line therapy during active disease as well as for maintenance therapy [ 99 ]. Approximately one-third of reported adult patients have required a second-line immunosuppressive agent to control their disease [ 86 ]. Unconventional therapies, such as liposteroid, leflunomide, and mesenchymal stem cell transplant, have also been tried [ 100 - 102 ].…”

Section: Reviewmentioning

confidence: 99%

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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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Section: Reviewmentioning

confidence: 99%

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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“…Patients of all ages can suffer from IPH 3,38 . In the pediatric population, the disease generally occurs before 10 years.…”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

“…The classic triad is present in approximately half of the pediatric patients 27 . In contrast, nearly 80% of adult patients demonstrate all three manifestations 3 . The classic triad is present in 93.8% of adult LHS patients 42 …”

Section: Clinical Aspects In Iphmentioning

confidence: 99%

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

Saha

Aiman

Chong

et al. 2022

Pediatric Pulmonology

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This manuscript reports the recent advances in idiopathic pulmonary hemosiderosis (IPH), a rare cause of diffuse alveolar hemorrhage in children and adults. This narrative review of the literature summarizes different aspects of IPH, including proposed pathogenesis, patient demographics, clinical and radiological characteristics, treatment, and prognosis. Additionally, the association between Celiac Disease (CD) and IPH is carefully evaluated. IPH is a frequently misdiagnosed disease. The delay in the diagnosis of IPH is often significant but fortunately, appears to have decreased in recent years. IPH in adults and children have distinct demographic preferences. The autoantibodies are common in IPH but with a definite difference between the adult and pediatric populations. The definitive diagnosis of IPH requires lung biopsy and careful exclusion of all competing diagnoses, even with lung biopsy showing bland pulmonary hemorrhage. The presence of nonspecific inflammatory cells or lymphoid aggregates may suggest a secondary immunologic phenomenon and needs careful evaluation and follow-up. A substantial number of patients suffer from coexisting CD, also known as Lane−Hamilton syndrome (LHS), and all patients with IPH need to be evaluated for LHS by serology. Although strict gluten free diet can manage the majority of patients with LHS, other patients generally require immunosuppressive therapy. The corticosteroids are the backbone of IPH therapy. Recently utilized experimental treatment options include mesenchymal stem cell transplant, liposteroid and bronchial artery embolization. The immunosuppression should be adjusted to achieve optimal disease control. Patients may progress to endstage lung disease despite all measures, and lung transplantation may be the only viable option.

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“…Although autoantibodies have been reported in pediatric IPH patients consistently [ 12 ], the true prevalence of autoantibodies in adults has been largely unknown until recently. We have recently discussed the prevalence of autoantibodies in a cohort of adult IPH patients as reported in the literature [ 13 ]. Similar to pediatric patients, a majority of these patients had antibodies suggestive of celiac disease (CD), a combination known as Lane Hamilton syndrome (LHS) [ 12 , 14 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

The Spectrum of Autoantibodies in Adult Patients With Idiopathic Pulmonary Hemosiderosis: A Brief Review of the Literature

Saha¹,

Bonnier²,

Saha³

et al. 2022

Cureus

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While autoimmune antibodies or autoantibodies have been reported sporadically in adult patients with idiopathic pulmonary hemosiderosis (IPH), their true prevalence is unknown. The question as to whether any difference exists between antibody-positive and negative patients has not been explored. The primary objective of this paper was to assess the spectrum of autoantibody testing and its positivity rate. The other objectives included a comparative analysis of demographics, symptom onset, clinical manifestations, and differences in clinical outcomes between antibody-positive (cohort A) and negative (cohort B) patients. To that end, we conducted a retrospective review of the relevant published literature. Multiple databases were searched to retrieve studies published between 1990 and 2022. A total of 35 studies, involving 38 patients, were identified. Five of these patients had a positive autoantibody. Patients in cohort A were older and more likely to be male. The frequencies of testing for these antibodies were as follows: antineutrophil cytoplasmic antibody (ANCA): 37/38 (97.4%), antinuclear antibody (ANA): 31/38 (81.6%), and anti-glomerular basement membrane antibody (anti-GBM): 30/38 (78.9%); 5/38 (13.2%) patients tested positive for an autoantibody, and two of these patients were positive for ANA, two for antithyroid antibody, and one patient tested positive for ANCA, rheumatoid factor (RF), and granulocyte monocyte-colony stimulating factor (GM-CSF) antibody. There was no difference between the cohorts regarding their clinical presentations, recurrence risks, and survival. The occurrence of autoantibodies is uncommon in adult IPH patients. This is in contrast with the pediatric IPH patient population, where the prevalence is much higher (26.4% vs. 13.2%), and the antibodies are more diverse. Unlike pediatric patients, adult patients with autoantibodies do not necessarily have worse outcomes.

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Adult patients with idiopathic pulmonary hemosiderosis: a comprehensive review of the literature

Cited by 10 publications

References 109 publications

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

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

Updates in idiopathic pulmonary hemosiderosis in 2022: A state of the art review

The Spectrum of Autoantibodies in Adult Patients With Idiopathic Pulmonary Hemosiderosis: A Brief Review of the Literature

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