Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology

Walsh, Laura; McCarthy, Cormac; Henry, Michael T.

doi:10.1136/bcr-2020-241048

Cited by 4 publications

(7 citation statements)

References 26 publications

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“…A total of 60 studies that fulfilled the inclusion criteria were identified [ 2 , 11 , 14 - 69 ]. Figure 1 illustrates the study selection process.…”

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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“…A total of 60 studies that fulfilled the inclusion criteria were identified [ 2 , 11 , 14 - 69 ]. Figure 1 illustrates the study selection process.…”

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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“…Five patients were positive for autoantibodies (cohort A) [ 18 - 22 ], and the rest were tested but turned out to be negative for any antibodies (cohort B). The mean (SD) and median (IQR) ages at diagnosis of IPH for cohort A were 58.2 (19.9) years and 50 (36.5) years, respectively.…”

Section: Reviewmentioning

confidence: 99%

“…Of note, 5/38 (13.2%) patients tested positive for an autoantibody. Two of these patients were positive for ANA [ 20 , 21 ], two for antithyroid antibody [ 19 , 21 ], and one patient each tested positive for ANCA [ 21 ], RF [ 18 ], and GM-CSF antibody [ 22 ]. Two of these five patients also tested positive for CD antibodies [ 20 , 21 ].…”

Section: Reviewmentioning

confidence: 99%

“…Other autoantibodies identified in our review were ANA [ 20 , 21 ], RF [ 18 ], anti-thyroid [ 19 , 21 ], and anti-GM-CSF antibodies [ 22 ]. In addition, we have identified several additional patients with autoantibodies reported as adults after being diagnosed with IPH as children (Table 2 ) [ 28 - 31 ].…”

Section: Reviewmentioning

confidence: 99%

“…Therefore, the coexistence of IPH and RA could suggest immune dysregulation in patients with IPH rather than causation. Similarly, the anti-thyroid antibodies [ 19 ] and coexistence of IPH with pulmonary alveolar proteinosis [ 22 ] are likely to represent the same phenomenon.…”

Section: Reviewmentioning

confidence: 99%

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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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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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Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology

Cited by 4 publications

References 26 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

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

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

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