SESSION TYPE: ILD Global Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a potentially life threatening condition, and requires prompt management. Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of DAH. Here we describe a case of IPH with rare associations in a young adult.
CASE PRESENTATION: 19 years old male from Northern India presented with recurrent haemoptysis (2 tsf/day, for 3 years) with generalized weakness, and progressive dyspnea (MRC grade II). He had previously been diagnosed as a case of iron deficiency anemia (Hb 2 g/dl), and received blood transfusion and hematinics. There was no history of joint pains, rash, photosensitivity, alopecia, oral ulcers, Raynauds phenomenon, dysuria, bleeding from other sites , recurrent chest infections, or tuberculosis. On examination he was pale with pulse rate 110/min; BMI was 18 kg/m2. Systemic examination was within normal limits . His X Ray Chest showed cardiomegaly. ECG showed sinus tachycardia with nonspecific interventricular conduction delays, with T wave inversion (I, aVL, V4-V6). Troponins and CKMB were not raised. His hemoglobin was 7.8 g/dl; peripheral smear was suggestive of iron deficiency. Liver, renal functions and ABG (PO2 89 mm Hg, FiO2 0.21) were normal. CECT Chest revealed bilateral ground glass opacities. Pulmonary function testing showed mild restriction (FVC 3.05 L, 68 % predicted), with increased diffusion capacity for carbon monoxide (DLCO). Bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) revealed hemosiderin laden macrophages, suggestive of IPH. Bacterial culture, AFB, fungal cultures and other staining were negative. Work up for connective tissue including RF, ANA, ANCA were normal . Echocardiography revealed dilated cardiomyopathy (DCMP) with severe LV dysfunction (LVEF 25%) . On further evaluation he gave history of recurrent diarrhea in childhood, not foul smelling, without any blood or mucus, though was asymptomatic since last 5 years. Serum Anti Tissue Transglutaminase antibody titres were strongly positive (291.66 IU/ml, normal range <30 IU/ml). Duodenal biopsy showed subtotal villous atrophy (Marsh Grade IIIB), confirming the diagnosis of celiac disease (CD). A final diagnosis of IPH with CD (Lane Hamilton Syndrome) with DCMP was made. Patient was started on gluten free diet, beta blockers & diuretics. In a short follow up of two months, patient has shown symptomatic improvement. Hemoptysis has decreased, hemoglobin has improved to 10.2 g/dl. A repeat ECHO has shown LVEF of 35%.
DISCUSSION: DAH is bleeding from pulmonary microvasculature. A prompt work up for vasculitides, connective tissue disorders and other causes with symptomatic treatment and immunosuppression is required.1 IPH is a rare cause of DAH, with an incidence of 0.2-2.0/million per year. The association between IPH and CD was first described by Lane and Hamilton in 1971.1 28 cases have been reported so far, with good response to gluten free diet, and few requiring immunosuppression. CD has been reported in 4-5.8% patients with DCMP, with improvement following gluten free diet. Selenium and carnitine deficiency may also be contributory. Screening for CD has been recommended in patients with DCMP.2 This is the first case report of Lane Hamilton syndrome with DCMP in adults. Two other cases have been reported in pediatric age group. Both cases required gluten free diet alongwith immunosuppression. 3
CONCLUSIONS: Screening for CD is important in a case of IPH. DCMP is a rare third association. It is potentially reversible with gluten free diet.
1) R. Lazor: Chapter 2. Alveolar haemorrhage syndromes. European Respiratory Society Monograph 54: 15-31, 2011
2) Lodha A, Haran M, Hollander G, et al: Celiac disease associated with dilated cardiomyopathy. South Med J 102:1052-4, 2009
3) Narula N, Rawal P, Kumar RM, Ram Thapa B. Association of celiac disease with cardiomyopathy and pulmonary hemosiderosis. J Trop Pediatr 56: 201-3, 2010
DISCLOSURE: The following authors have nothing to disclose: Gopi Khilnani, Pawan Tiwari, Alpesh Goyal, Lavleen Singh, Arun Arora, Randeep Guleria
No Product/Research Disclosure InformationAll India Institute of Medical Sciences, New-Delhi, India