BAckgRoundFor over half a century, clinicians and pathologists have recognized a group of pulmonary diseases associated with an accumulation of lymphoid follicles in the walls of bronchi and bronchioles. This entity was first described in patients with bronchiectasis, a disease characterized by extensive airway mural inflammation, where lymphocytic hyperplasia in the walls of small airways formed part of the inflammatory picture. However, in a group of patients, pathologists were able to confirm that sub-epithelial accumulation of lymphoid follicles was the primary pathology affecting the bronchioles [1]. These enlarged follicles would often distort the architecture of the bronchial tree, merely due to their size, projecting into the bronchial lumen and causing partial bronchial and bronchiolar obstruction.In 1947, the first reference to this entity was made by Engel et al., who coined the term "nodal bronchiolitis", describing thickened bronchioles, with well formed lymphoid follicles in their walls in the absence of a diffuse inflammatory infiltrate [2].In 1952, Whitewall studied 200 consecutive lung specimens mainly from lobectomies and pneumonectomies of patients with advanced bronchiectasis. He described this feature as "follicular bronchiectasis", where the most prominent microscopic finding was an extensive formation of lymphoid follicles and lymph nodes in the walls of affected bronchi and bronchioles [1].In 1979, Epler et al., described an association between bronchiolitis and the administration of D-penicillamine in 2 patients with rheumatoid arthritis and eosinophilic fasciitis. The chronic inflammatory bronchiolar disorder seen was characterized by extensive proliferation of lymphoid tissue, occurring as follicles in the bronchiolar walls and was given the name "follicular bronchiolitis" (FB) [3].Currently, FB is classified as one of the non-neoplastic (reactive) pulmonary lymphoid disorders in a group known as the lymphoproliferative pulmonary diseases (LPDs) [ ABstRActFollicular bronchiolitis (FB) also known as hyperplasia of the bronchial associated lymphoid tissue (BALT), or bronchiolar nodular lymphoid hyperplasia, is an entity characterized by the development of lymphoid follicles with germinal centers in the walls of small airways. FB is thought to be caused by antigenic stimulation of BALT, followed by a polyclonal lymphoid hyperplasia. It is currently classified as one of the reactive pulmonary lymphoid disorders in a group known as the lymphoproliferative pulmonary diseases (LPDs).FB is a pathological diagnosis that can be seen in several clinical settings, including connective tissue diseases, immunodeficiency states, autoimmune diseases, infections, obstructive airway diseases, as well as several types of interstitial lung diseases (ILDs).Its characteristics need to be carefully identified and differentiated from other closely related diseases in the group of LPDs due to significant differences in treatment and prognosis. Lymphoproliferative Pulmonary diseases (LPds)Reactive / of lymphoi...

Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.

Common variable immunodeficiency (CVID) is one of the most common primary immunodeficiencies, which is characterized by reduced serum immunoglobulin levels and B-lymphocyte dysfunction. There are many clinical manifestations of this disease, the most common of which are recurrent respiratory tract infections. Among the most recently recognized autoimmune manifestation of CVID is a disease described as granulomatous-lymphocytic interstitial lung disease (GLILD), where CVID coexists with a small airway lymphoproliferative disorder, mimicking follicular bronchiolitis, or lymphocytic interstitial pneumonitis (LIP) on histology specimens. We herein describe the clinical and radiological features of GLILD in a 55-year-old woman where the diagnosis of CVID was actively pursued and eventually confirmed after her lung biopsy showed characteristic features of GLILD. The patient had dramatic response to treatment with IVIG and corticosteroids for 3 months followed by Mycophenolate mofetil for maintenance therapy. K E Y W O R D Scommon variable immunodeficiency, follicular bronchiolitis, granulomatous-lymphocytic interstitial lung disease, lymphoproliferative disorder, lymphocytic interstitial pneumonitis | I NTR ODU CTI ONCommon variable immunodeficiency (CVID) is estimated to affect 1/25,000 to 1/50,000 individuals worldwide. Approximately, 20% of CVID patients have associated autoimmune diseases, including hemolytic anemia, thrombocytopenia, neutropenia, rheumatoid arthritis, vitiligo, pyoderma gangrenosum, and others. [1][2][3][4][5][6][7] Granulomatous-lymphocytic interstitial lung disease (GLILD) is a pathologic diagnosis characterized by granulomas with benign lymphoproliferative infiltrates involving diffuse areas of the pulmonary parenchyma. [8][9][10] It is the most common cause of diffuse parenchymal lung disease in patients with CVID, occurring in approximately 10%-30% of all patients. 8-13 GLILD can be detected on lung biopsies many years prior to the diagnosis of CVID, 14 and is often associated with other organ granulomas, splenomegaly, adenopathy, and autoimmune cytopenias. 12,15 | C AS E PR ES ENTATI ONA 55-year-old woman was evaluated in the pulmonary clinic for progressively worsening dyspnea on exertion for 3 months. Her symptoms began with a dry cough, which failed to respond to two courses of antibiotics that were prescribed by her primary care physician for a possible respiratory tract infection. Her medical history was significant for Hashimoto's thyroiditis, Type I diabetes mellitus and Morphea-form skin rash. She had no history of smoking, recurrent infections, or chronic respiratory diseases, with no significant occupational or Figure 1) showed bilateral lower lung zone patchy and nodular opacities. Her pulmonary function test showed mild restriction with a moderately reduced diffusing capacity of the lung for carbon monoxide (DLCO). On chest computed tomography (CT) scan (Figure 2) diffuse bilateral ground glass opacities with bulky mediastinal and hilar lymphadenopathy was noted. Other ...

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

Exogenous lipoid pneumonia (ELP) is a rare type of inflammatory lung disease caused by aspiration and/or inhalation of fatty substances and characterized by a chronic foreign body-type reaction to intra-alveolar lipid deposits. The usual clinical presentation occurs with insidious onset of nonspecific respiratory symptoms and radiographic findings that can mimic other pulmonary diseases. Diagnosis of ELP is often missed or delayed as it requires a high index of suspicion and familiarity with the constellation of appropriate history and radiologic and pathologic features. We herein report a case of occupational exposure to tabletop “Teppanyaki” entertainment cooking as a cause of ELP, confirmed by surgical lung biopsies in a 63-year-old Asian woman who worked as a Hibachi-Teppanyaki chef for 25 years.