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

Abstract

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.

Lane-Hamilton Syndrome in an Adult With Down Syndrome: A Case Report

“…Since there is an established association between IPH and CD, it is recommended to screen for CD even in patients without gastrointestinal symptoms [ 14 ].…”

“…It’s difficult to assess the prognosis due to the small series of patients and inadequate follow-up. The early recognition of this entity is important because a gluten-free diet has a positive effect on the regression of both CD and IPH [ 14 ].…”

Idiopathic pulmonary haemosiderosis misdiagnosed as haemolytic anaemia: a case report

“…A review of the paediatric idiopathic pulmonary haemosiderosis literature demonstrates that patients who have been administered corticosteroids and other immunosuppressants have experienced a considerable increase in their survival rate. 12 It is essential to consider the possibility of idiopathic pulmonary haemosiderosis in cases of recurrent pulmonary haemorrhage of unknown origin, as early diagnosis and treatment are crucial to reducing mortality. Delayed diagnosis can lead to hypoxia and worsened lung function, negatively impacting on the patient's prognosis.…”

Juvenile idiopathic arthritis presenting as diffuse alveolar hemorrhage at onset: A case series

“…In severe cases, patients may experience life‐threatening alveolar hemorrhage and develop acute respiratory failure. The etiology of DAH is often unknown in childhood, but most children have a background of autoimmune disease or gradually develop rheumatic disease manifestations with age 1,2 . Juvenile idiopathic arthritis (JIA) is a chronic idiopathic inflammation that mainly affects the joints.…”

“…The etiology of DAH is often unknown in childhood, but most children have a background of autoimmune disease or gradually develop rheumatic disease manifestations with age. 1,2 Juvenile idiopathic arthritis (JIA) is a chronic idiopathic inflammation that mainly affects the joints. Rheumatoid factor (RF)-positive JIA is considered to be the equivalent of adult RFpositive rheumatoid arthritis (RA).…”