Idiopathic pulmonary hemosiderosis is a rare
disease characterized by recurrent episodes of hemoptysis, pulmonary
infiltration and iron deficiency anemia [1-4]. Celiac disease is an
enteropathy characterized by life-long intolerance to ingested
gluten in genetically susceptible people [5]. The simultaneous
occurrence of these conditions is rare and is called Lane-Hamilton
syndrome [6]. Significant improvement can be obtained with
gluten-free diet, not only in intestinal but also in pulmonary
symptoms [1,4,7].
Case Report
This four and half years old girl was admitted
with complaints of cough, difficulty in breathing and pallor. She
had intermittent episodes of abdominal pain and diarrhea. The body
weight and height were 16 kg and 105 cm (both at 3rd percentile).
The heart rate, respiratory rate, blood pressure and oxygen
saturation were 168 per min, 54 per min, 80/40 mm Hg and 88%
respectively. The patient showed severe pallor. Fine crepitations
were heard and liver was palpable 2 cm below the right subcostal
margin. Investigations showed hemoglobin level of 2.5 g/dL,
leukocytes 8300/cu mm and platelets 296000/cu mm. The mean
corpuscular volume was 59 fl, reticulocyte count 10%, plasma iron 13
µg/dL, iron biding capacity 296 µg/dL and ferritine 74 µg/L. The
peripheral smear examination showed hypochromic microcytic anemia.
The chest radiograph showed bilateral infiltrates; echocardiography
was normal. The findings improved after blood transfusion and the
pathologic radiological findings disappeared within a few days.
On follow-up, the patient showed recurrence of
anemia, steatorrhea, abdominal pain and diarrhea. The levels of
serological markers of celiac disease (anti-gliadin antibody IgA,
IgG and antiendomysial antibody IgA) were >12 U/mL (normal range
0-12 U/mL). She was diagnosed as celiac disease based on the
presence of crypt hyperplasia and partial villous atrophy and marked
lymphocytic infiltration of the villous epithelium. The patient
received gluten-free diet with improvement in intestinal symptoms.
The patient was admitted again with anemia and respiratory distress.
The hemoglobin level was 3 g/dL; chest radiograph showed bilateral
patchy infiltrates. Hemosiderin laden macrophages were seen on
gastric aspirate. Histologic examination of lung biopsy specimen
revealed hemosiderin-laden intraalveolar macrophages, but there was
no evidence of pulmonary vasculitis or granulomatous inflammation.
Coagulation studies, levels of vitamin B12, folic acid,
00-1 antitrypsin,
immunoglobulin and complement were normal; serology for systemic
lupus and other vasculitis were negative. RAST test against cow milk
was negative. The patient was treated with oral methylprednisolone,
at a dose of 2 mg/kg/day and gluten-free diet, with recovery of
gastrointestinal and pulmonary symptoms. At follow up, the disease
was controlled with low dose steroids and a gluten-free diet. The
height was within 10 to 25 percentile.
Discussion
Idiopathic pulmonary hemosiderosis with
concomitant celiac disease is a rare condition [3,4,7,8]. Both
conditions are considered to be immune-mediated, although the causal
relationship is not clear [2-4,6,7]. Accumulation of immunocomplexes
(including food allergens) in the alveolar capillary basement
membrane, and cross reaction between antireticulin antibodies and
alveolar basal membrane antigens are presumed to be pathogenic [3].
Use of gluten-free diet results in cessation of
lung hemorrhage, reduced need for blood transfusions [2-4,7] and
improved respiratory functions in Lane Hamilton syndrome.
Gluten-free diet alone may not be sufficient, and patients require
immunosuppressive therapy [1].
In two studies that screened patients with
idiopathic pulmonary hemosiderosis for celiac disease [1,9] an
accompaniment rate of 3/7 and 3/10, respectively was found . Two
other studies showed that the two conditions may be associated in
6.6-8.7% cases [8,10]. Some researchers suggest that all patient
with IPH should be screened routinely for CD, even in the absence of
gastrointestinal symptoms [1-4,7,10]. Our patient had growth
retardation, chronic anemia, recurrent abdominal pain and diarrhea.
The diagnosis of celiac disease was based on positive serologic
test, intestinal biopsy, and clinical and serological remission with
gluten-free diet.
However, patients who have the Lane Hamilton
syndrome show satisfactory improvement of pulmonary and
gastrointestinal symptoms on a gluten-free diet. We propose that
patient with idiopathic prolonged hemosiderosis should be screened
for celiac disease, even in absence of gastrointestinal symptoms.
Contributors: All the authors
contributed to the case management, reviewing the literature and
drafting the manuscript. All authors approved the final manuscript.
Funding: None; Competing interests:
None stated.
References
1. Khemiri M, Ouederni M, Khaldi F, Barsaoui S.
Screening for celiac disease in idiopathic pulmonary hemosiderosis.
Gastroenterol Clin Biol. 2008;32:745-8.
2. Hammami S, Ghedira Besbes L, Hadded S,
Chouchane S, Ben Meriem C, Gueddiche MN. Co-occurrence of pulmonary
haemosiderosis with coeliac disease in a child. Respir Med.
2008;102:935-6.
3. Hoca NT, Dayioglu D, Ogretensoy M. Pulmonary
hemosiderosis in association with celiac disease. Lung.
2006;184:297-300.
4. Sethi GR, Singhal KK, Puri AS, Mantan M.
Benefit of gluten-free diet in idiopathic pulmonary hemosiderosis in
association with celiac disease. Pediatr Pulmonol. 2010 Oct 21.
5. Polanco I. Celiac disease. J Pediatr
Gastroenterol Nutr. 2008;47:S3-6.
6. Lane DJ, Hamilton WS. Idiopathic steatorrhoea
and idiopathic pulmonary haemosiderosis. Br Med J. 1971 10;2:89-90.
7. Agarwal R, Aggarwal AN, Gupta D. Lane-Hamilton
syndrome: simultaneous occurrence of coeliac disease and idiopathic
pulmonary haemosiderosis. Intern Med J. 2007;37:65-7.
8. Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol
D. Long-term clinical course of patients with idiopathic pulmonary
hemosiderosis (1979-1994): prolonged survival with low-dose
corticosteroid therapy. Pediatr Pulmonol. 1999;27:180-4.
9. Wright PH, Menzies IS, Pounder RE, Keeling PW.
Adult idiopathic pulmonary haemosiderosis and coeliac disease. Q J
Med. 1981;50:95-102.
10. Le Clainche L, Le Bourgeois M, Fauroux B,
Forenza N, Dommergues JP, Desbois JC, et al. Long-term
outcome of idiopathic pulmonary hemosiderosis in children. Medicine
(Baltimore). 2000;79:318-26.
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