Sudden Vision Loss Due to Optic Neuritis-An Uncommon Presentation of Neurosarcoidosis.

Sarcoidosis is a systemic, granulomatous disease of unknown etiology, most often manifested by mediastinal and hilar lymph node enlargement and parenchymal nodules in the lungs. However, it may involve any other organ. Neuro-sarcoidosis, a condition that affects up to 20% of sarcoidosis patients, can be found in any part of the central or peripheral nervous system and has important ophthalmic and neuro-ophthalmic manifestations. We present two patients with sudden vision loss due to neurosarcoidosis. In both cases, biopsy of the mediastinal lymph node showed non-caseating granulomas consistent with sarcoidosis. Treatment involved high doses of methylprednisolone intravenously, followed by topical dexamethasone eye drops in the first case and a systemic steroid treatment in the second, resulting in symptom relief. Those two cases demonstrate that sarcoidosis should be considered as a differential diagnosis in cases of optic neuritis.

The Presence of T Allele (rs35705950) of the MUC5B Gene Predicts Lower Baseline Forced Vital Capacity and Its Subsequent Decline in Patients with Hypersensitivity Pneumonitis.

Hypersensitivity pneumonitis (HP) is an exposure-related interstitial lung disease with two phenotypes-fibrotic and non-fibrotic. Genetic predisposition is an important factor in the disease pathogenesis and fibrosis development. Several genes are supposed to be associated with the fibrosing cascade in the lungs. One of the best-recognized and most prevalent is the common MUC5B gene promoter region polymorphism variant rs35705950. The aim of our study was to establish the frequency of the minor allele of the MUC5B gene in the population of patients with HP and to find the relationship between the MUC5B promoter region polymorphism and the development of lung fibrosis, the severity of the disease course, and the response to the treatment in patients with HP. Eighty-six consecutive patients with HP were tested for the genetic variant rs35705950 of the MUC-5B gene. Demographic, radiological, and functional parameters were collected. The relationship between the presence of the T allele and lung fibrosis, pulmonary function test parameters, and the treatment response were analyzed. The minor allele frequency in the study group was 17%, with the distribution of the genotypes GG in 69.8% of subjects and GT/TT in 30.2%. Patients with the GT/TT phenotype had significantly lower baseline forced vital capacity (FVC) and significantly more frequently had a decline in FVC with time. The prevalence of lung fibrosis in high-resolution computed tomography (HRCT) was not significantly increased in GT/TT variant carriers compared to GG ones. The patients with the T allele tended to respond worse to immunomodulatory treatment and more frequently received antifibrotic drugs. In conclusions: The frequency of MUC5B polymorphism in HP patients is high. The T allele may indicate a worse disease course, worse immunomodulatory treatment response, and earlier need for antifibrotic treatment.

An Unfavorable Outcome of M. chimaera Infection in Patient with Silicosis.

Mycobacterium chimaera is a slow-growing, nontuberculous mycobacterium (NTM) belonging to the Mycobacterium avium complex (MAC). It was identified as a unique species in 2004. Since 2013 it has been reported as a cause of disseminated infection in patients after cardiac surgeries. Only a few cases associated with underlying lung diseases have been noted. M. chimaera infection is characterized by ambiguous symptoms. There is no treatment with proven effectiveness, and it has a poor prognosis. Silicosis is a disease that can predispose to mycobacterial infection. Silica damages pulmonary macrophages, inhibiting their ability to kill mycobacteria. We present a case of M. chimaera infection in a patient with silicosis and without other comorbidities. To our knowledge, it is the first case of silicosis associated with M. chimaera disease. A 45-year-old man presented with a persistent low-grade fever. Based on the clinical and radiological picture, positive cultures, and histological examination, the nontuberculous mycobacterial disease was diagnosed. First, multidrug therapy according to the treatment guidelines for MAC was implemented, then antibiotics were administrated, based on drug sensitivity. Despite the treatment, eradication was not achieved and the patient died. The analysis of M. chimaera infection cases could contribute to developing recommendations and thus improve the prognosis.

Exogenous lipoid pneumonia induced by nasal instillation of paraffin oil.

Lipoid pneumonia is a rare pulmonary disease, classified in terms of the source of lipid exposure into two variants: exogenous and endogenous. We present a patient with exogenous lipoid pneumonia, acquired after chronic exposure to paraffin oil-containing nasal drops. The diagnosis was established by demonstration of lipid-laden macrophages in bronchoalveolar lavage, chest computed tomography results and a history of lipid exposure.

[Pulmonary involvement in connective tissue disease]. / Zmiany w ukldzie oddechowym w przebiegu ukladowych chorób tkanki lacznej.

The connective tissue diseases are a variable group of autoimmune mediated disorders characterized by multiorgan damage. Pulmonary complications are common, usually occur after the onset of joint symptoms, but can also be initially presenting complaint. The respiratory system may be involved in all its component: airways, vessels, parenchyma, pleura and respiratory muscles. Lung involvement is an increasing cause of morbidity and mortality in the connective tissue diseases. Clinical course is highly variable - can range from mild to rapidly progressive, some processes are reversible, while others are irreversible. Thus, the identification of reversible disease , and separately progressive disease, are important clinical issues. The frequency, clinical presentation, prognosis and responce to therapy are different, depending on the pattern of involvement as well as on specyfic diagnostic method used to identify it. High- resolution computed tompography plays an important role in identifying patients with respiratory involvement. Pulmonary function tests are a sensitive tool detecting interstitial lung disease. In this article, pulmonary lung involvement accompanying most frequently apperaing connective tissue diseases - rheumatoid arthritis, systemic sclerosis, lupus erythematosus, polymyositis/dermatomyositis, Sjögrens syndrome and mixed connective tissue disaese are reviewed.

[Acute interstitial pneumonia in patient with rheumatoid arthritis treated with leflunomide]. / Ostre sródmiazszowe zapalenie pluc u chorego na reumatoidalne zapalenie stawów leczonego leflunomidem.

Leflunomide (LEF) is an isoxazole derivative used as disease-modifying anti-rheumatic drug (DMARD) in the treatment of rheumatoid arthritis (RA). It is effective and safe in patients with active RA, in whom standard treatment is insufficient or contraindicated, but it can cause interstitial lung disease (ILD). Identified risk factors for LEF-induced ILD include pre-existing ILD, cigarette smoking, low body weight, and use of loading dose. LEF should be avoided in patients with pre-existing ILD. We present a case of 59-year-old male with RA and a history of smoking and methotrexate (MTX) treatment, who developed dyspnoea, non-productive cough, and fever about two months after the administration of LEF. The clinical and radiological presentation was of acute pneumonia. The patient was treated with methylprednisolone pulse, prednisone, and cyclophosphamide, but he died of respiratory failure.

[A surprising diagnosis in a male with a tumour of the chest wall--not always lung cancer]. / Zaskakujace rozpoznanie u mezczyzny z guzem sciany klatki piersiowej--nie wszyscy choruja na raka pluca.

Lung cancer is the most frequent malignant tumour in men. Advanced disease may produce metastatic tumours in subcutaneous tissue and also infiltrate the chest wall. We present a history of a man referred to our department suspected of lung tumour infiltrating the chest wall. Additionally, bone metastatic disease was diagnosed (ribs, vertebral bodies and skull). Thanks to a wide diagnostic approach, ductal cancer of the breast was finally diagnosed, a neoplasm that is extremely rare in male patients, usually presenting as a definite nodule in the nipple area of the breast. This case shows the importance of careful histological evaluation of the chest wall tumour.

[Interstitial lung disease in patients with polymyositis and dermatomyositis--report of three cases]. / Zmiany sródmiazszowe w plucach u chorych na zapalenie wielomiesniowe i skórno-miesniowe--prezentacja trzech przypadków.

Polymyositis (PM) and dermatomyositis (DM) are connective tissue diseases (CTD) characterized by proximal muscle weakness along with changes in various internal organs, with the lungs most frequently involved. Presentation of the disease in the lungs comprises diffuse alveolar haemorrhage due to vasculitis and interstitial lung disease (ILD), which is the most frequent manifestation of CTD in the lungs and worsens the outcome and prognosis. The mechanisms involved in the ILD are not fully known, but the role of autoimmune response is unquestioned. No relationship between the severity of CTD and the changes in the lungs was observed. ILD may present at any time in the course of CTD, sometimes before the signs and symptoms of myositis occur. The more accurate imaging methods are, the more frequently changes in the lungs are detected. High resolution computed tomography (HRCT) is a gold standard in ILD imaging. Treatment of PM/DM-related ILD relays on systemic glucocorticosteroids as the first choice drugs. We present three cases of PM/DM-related ILD in middle-aged men, with a different clinical and radiological presentation. In all cases, apart from imaging (plain X-ray and HRCT of the chest) and pulmonary function tests, histological evaluation of lung changes was performed. In two cases non-specific interstitial pneumonitis (NSIP) was diagnosed, and in the third--organizing pneumonia along with sarcoid changes in the lymph nodes. Because of decreased pulmonary function all patients were treated with systemic corticosteroids and two of them additionally with azathioprine or cyclophosphamide, and the outcome was good in all of them.

[Interstitial lung disease in patients with primary biliary cirrhosis]. / Zmiany sródmiazszowe w plucach u chorych na pierwotna zólciowa marskosc watroby.

Primary biliary cirrhosis (PBC) is a chronic autoimmune disorder of unknown etiology. The disease affects middle-aged women and is characterized by the destruction of the intralobular bile ducts that causes consequent cholestasis. AMA is a hallmark of PBC, composed mostly of IgG and IgM class. The M2 antibody is the most specific one, with sensitivity range of 54-98% depending on type of test used. PBC is often accompanied by other autoimmune diseases, such as Sjögrens syndrome, thyroiditis, rheumatoid arthritis, dermatomyositis, polymyositis. Interstitial lung disease (ILD) has been reported in patients with primary biliary cirrhosis but its frequency and nature are poorly understood. We report pulmonary involvement in the course of PBC in 4 middle-aged women. Histopatological examination of lung specimens was available in three patients: two presented with sarcoid - like granulomas, one with lymphocytic interstitial pneumonia (LIP). In one patient the diagnosis of pulmonary fibrosis was based on clinical and radiological features. Because of abnormal pulmonary function tests (PFT) results all the patients were treated with prednisone, one, additionally with azathioprine. The treatment was successful in all of the patients.

[Mycobacterial infection caused by Mycobacterium avium in allogenic bone marrow transplant recipient with concomittant bronchiolitis obliterans as a manifestation of graft versus host disease - case report and review of the literature]. / Mikobakterioza wywolana Mycobacterium avium u chorej po przeszczepieniu szpiku kostnego ze wspolistniejacym zarostowym zapaleniem oskrzelikow jako wyraz choroby przeszczep przeciw gospodarzowi -- opis przypadku oraz przeglad pismiennictwa.

Patients after organ transplantations are at risk for mycobacteriosis development. Frequency of the mycobacterial infection after bone marrow transplantation (BMT) is not as high as one could expect. It ranges from 0.4 to 4.9%. We present a case of a female patient after allogenic BMT as a treatment of chronic myelogenous leucaemia, with bronchiolitis obliterans as a symptom of graft versus host disease (GvHD), treated with corticosteroids and infected with Mycobacterium avium. She was admitted to the hospital with dyspnoea, cough with large amount of sputum production and subfebrile status. She had partial respiratory insufficiency and obturative disturbances of respiration (FEV(1) 0.67 l i.e. 22% of normal) with decline of VC (2.23 l i.e. 64% of normal). The high-resolution computed tomography (HRCT) revealed multifocal infiltrations and bronchiectases in the upper and middle pulmonary fields, which were absent in the previous HRCT taken 3 years earlier. In the bronchial secretion acid-fast bacilli were found by smear and culture. The isolate was classified as Mycobacterium avium complex (MAC) by high performance liquid chromatography (HPLC). The patient was treated with clarithromycin, ciprofloxacin, isoniazide (INH), ethambutol (EMB), amikacin, but M. avium was still present in the sputum after 3 months. Treatment was continued in her parent hospital, where after a few months her sputum became negative for M. avium. But she died over a year later from progressive respiratory insufficiency in the course of bronchiolitis obliterans. The patient was in the group of high risk for mycobacterial infection development and the course of her illness was typical. We decided however to present the case as the topic seems to be quite neglected in the literature.

[Exogenous lipid pneumonia--a report of four cases]. / Egzogenne lipidowe zapalenie pluc--róznorodnosc obrazu klinicznego i radiologicznego.

Exogenous lipoid pneumonia (ELP) is a rare interstitial pulmonary disease caused by aspiration of the oily material. It is known to mimic many pulmonary diseases and causes many diagnostic difficulties. We present four patients with ELP. The clinical picture was very heterogenous. We described these cases to show that the use of the mineral oils may increase the risk of lipoid pnemonia.

[Analysis of pulmonary function tests and cellular composition of bronchoalveolar lavage fluid in patients with sarcoidosis]. / Analiza badan czynnosciowych pluc i skladu komórkowego plynu z plukania oskrzelowo-pecherzykowego u chorych na sarkoidoze.

We investigated cellular composition of bronchoalveolar lavage fluid and changes in pulmonary function tests in patients with histopathologically proven sarcoidosis. We analyzed BAL, lung volumes, lung compliance, and diffusing capacity in 33 nonsmoking patients (18 males, 15 females, age 22-60 years, mean 40 years). In 39% of the patients we observed decreased static compliance and in 18% of patients reduction in diffusing capacity. The total cell count, percentage of lymphocytes and CD4/CD8 ratio and pulmonary function parameters in I, II and III radiological stages of sarcoidosis were similar. We found a moderate negative correlation between the carbon monoxide diffusing capacity (DLco% of pred.) and CD4/CD8 ratio (r = -0.40, p < 0.05). No correlation was observed between other pulmonary function indices and total cell count in BAL, percentage of lymphocytes and CD4/CD8 ratio. Our results suggest, that reduction in diffusing capacity may reflect an insensitivity of alveolitis, but correlation is moderate and it usefulness for making therapeutic decision is not clear.