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Diffuse Lung Disease |

A Case of Hyper-IgG4 Syndrome Mimicking Löfgren Syndrome: The Importance of Tissue Confirmation in Sarcoidosis FREE TO VIEW

Deepa Bhanot; Mark Hamblin
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Pulmonary and Critical Care Medicine, University of Kansas School of Medicine, Kansas City, Mission, KS


Chest. 2014;146(4_MeetingAbstracts):362A. doi:10.1378/chest.1994276
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Abstract

SESSION TITLE: Interstitial Lung Disease Posters I

SESSION TYPE: Original Investigation Poster

PRESENTED ON: Wednesday, October 29, 2014 at 01:30 PM - 02:30 PM

PURPOSE: ​Hyper-IgG4 syndrome is an increasingly recognized systemic disorder characterized by high serum IgG4 levels with lymphoplasmacytic infiltration of several organ systems. It was Initially described in relation to autoimmune pancreatitis and sialadenitis, but it is now known to involve several other organ systems including the lungs and skin.

METHODS: Our patient is a 41 year old woman who presented to the KU Saroidosis Clinic for suspicion of pulmonary sarcoidosis. She initially presented in 2005 with skin lesions on her bilateral shins, which were thought to be erythema nodosum along with mediastinal lymphadenopathy. Clinical features of Lofgren’s Syndrome lead to a diagnosis of sarcoidosis without biopsy confirmation. The skin lesions would spontaneously resolve and reappeaer but she never required treatment. In 2010 she developed some right periorbital swelling. She underwent a biopsy of this orbital pseudotumor, which revealed a lymphoplasmacytic infiltrate that stained positive for IgG4 plasma cells. Following the diagnosis of Hyper-IgG4 syndrome, she was started on prednisone. Subsequently she underwent skin biopsy from both the hand and leg lesion which showed interstitial granulomatous dermatitis and leukocytoclastic vasculitis respectively, but IgG4 staining was not performed.

RESULTS: She subsequently underwent endobronchial ultrasound with FNA of a subcarinal lymph node, which showed no evidence of granulomatous involvement. Lavage fluid showed 10% lymphocytes on flow cytometry with a low CD4 to CD8 ratio inconsistent with findings in sarcoidosis.

CONCLUSIONS: Hyper-IgG4 syndrome has been reported to involve both the lungs and the skin. The radiologic manifestations of Hyper IgG4 Syndrome are variable including mediastinal and hilar lymphadenopathy, thus mimicking sarcoidosis. Skin involvement with Hyper-IgG 4 related sclerosing disease has typically been shown to involve the head and neck area or chest.There are no case reports of Hyper-IgG4 syndrome presenting as skin lesions involving the extremities, especially in the shin resembling erythema nodosum.

CLINICAL IMPLICATIONS: This presents a further diagnostic challenge to clinicians in a disease, which can mimic sarcoidosis based on mediastinal lymphadenopathy alone.

DISCLOSURE: The following authors have nothing to disclose: Deepa Bhanot, Mark Hamblin

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