Herein, we report a case of Hermansky-Pudlak syndrome (HPS) in which respiratory symptoms improved with pirfenidone treatment. A 43-year-old Japanese woman with oculocutaneous albinism presented with a cough and dyspnea. High-resolution computed tomography revealed areas of reticular and frosted lung opacities. The diagnosis of HPS was confirmed by a prolonged bleeding time and HPS1 gene mutation. Generally, there is no effective treatment for interstitial pneumonia associated with HPS except for lung transplantation. In the present case, the cough and dyspnea improved with pirfenidone administration. Therefore, clinicians should administer pirfenidone in challenging transplantation cases and during the waiting period for transplantation.

We present an unusual case of a geriatric patient with right-sided cardiac displacement and rotation (Pseudo-Dextrocardia) secondary to radiation-induced pulmonary fibrosis (RIPF) after radiation for carcinoma of the right breast. This patient with heart failure with reduced ejection fraction (HFrEF) underwent cardiac resynchronization therapy with a defibrillator (CRT-D) for primary prevention of sudden cardiac death. Cannulization of the coronary sinus ostium was difficult, likely due to the significant cardiac displacement. However, after multiple attempts, it was eventually successful. The clinical manifestations, evaluation, and technical and procedural issues in this patient with an unusual anatomic variant are summarized.

A 75-year-old male was diagnosed with idiopathic pulmonary fibrosis and treated with pirfenidone. He presented with an erythematous thick scaly patch on his face, neck, and both hands and arms. He had a history of significant exposure to sunlight without using sunscreen. All lesions were restricted to sun-exposed areas and appeared one month ago. Histopathological examination revealed necrotic keratinocytes, epidermal spongiosis, liquefaction degeneration of the basal layer, interface dermatitis, solar elastosis, and upper dermal perivascular lympho-histiocytic infiltration. Based on clinical and histopathological findings, the skin lesion could be diagnosed as photosensitive drug eruption induced by pirfenidone. Pirfenidone was discontinued for a month, and the patient was treated with oral and topical corticosteroids. Consequently, the skin lesion almost fully cleared, leaving mild postinflammatory hyperpigmentation. Although there are many reports of photosensitivity reactions to pirfenidone, dermatologists are still not familiar with this drug. Through this case presentation, clinicians should be aware of the potential phototoxic effects of pirfenidone and provide the necessary precautionary information to patients who take pirfenidone.

Idiopathic retroperitoneal fibrosis (iRPF) is a chronic autoimmune disease characterized by fibroinflammatory tissue surrounding the abdominal aorta and iliac arteries and extending into the retroperitoneum to envelop neighboring structures. Hydronephrosis due to obstruction of ureters is the most common complication of iRPF. Glucocorticoid with or without immunosuppressants or tamoxifen, the mainstay of iRPF treatment, usually brings good response. Nevertheless, in some conditions, the obstruction of ureters remains unresolved with the treatment of all these medications. One of the reasons lies in the innate feature of the fibroinflammatory tissue. The proliferation of fibrosis tissue in addition to inflammation in the mass was associated with insufficient response to immunosuppressive therapies. Pirfenidone, an anti-fibrosis agent, has been successful in treating pulmonary fibrosis and renal fibrosis. Therefore, it is rationale to assume the effectiveness of pirfenidone in the treatment of iRPF. In the current article, we report a 61-year-old Chinese man with iRPF who responded well to pirfenidone.

Antifibrotic drugs are the standard treatments for patients with idiopathic pulmonary fibrosis (IPF). This study aims to assess the safety of antifibrotic treatment in IPF patients undergoing lung transplantation.
Patients with a diagnosis of IPF who received a lung transplant between January 2015 and June 2019 at four Spanish hospitals specialized in lung transplantation were retrospectively recruited. Cases were defined as patients receiving antifibrotic treatments at time of transplant. Each case was matched with a control who did not receive antifibrotic treatment.
A total of 164 patients were included in the study cohort (103 cases and 61 controls). There were no statistically significant differences between the cases and controls in any of the items studied related to transplantation except the time until the appearance of chest wall dehiscence: although there were no differences in the incidence of wall dehiscence in either group (12.3% vs. 13.7%; p = 0.318), the patients on antifibrotic drugs experienced it earlier (21 days [IQR = 12.5-41.5] vs. 63 days [IQR = 46.75-152.25]; p = 0.012). There were no differences in overall post-transplant survival between the two groups (p = 0.698) or in conditional survival at 30 days, 90 days, 3 years or 5 years. However, 1 year survival was significantly greater among controls (80.6% vs. 93.3%; p = 0.028).
There was evidence that chest wall dehiscences appeared earlier post-transplant in patients using antifibrotics, even though this factor did not significantly impact survival.

Idiopathic pulmonary fibrosis is a poorly prognosed form of progressive interstitial pneumonia. Patients with IPF have a significantly increased risk of developing lung cancer, which further worsens the course of the disease. The most common histological types of LC among patients with IPF are squamous cell carcinoma and adenocarcinoma. Furthermore, all LC treatment modalities can lead to developing an acute IPF exacerbation. In this report, we present a rare case of coexistence of IPF and small cell lung cancer in a 76-year-old patient with chronic obstructive pulmonary disease, and a former smoker. For over 2 years, the patient was treated with an anti-fibrotic drug-pirfenidone, which slowed down the progression of IPF. Unfortunately, after being diagnosed with an active SCLC, the patient was excluded from further participation in the pirfenidone drug program. SCLC is characterized by high aggressiveness, rapid growth and high metastatic potential; therefore, it is necessary to apply antitumor treatment as soon as possible. The described patient was treated with carboplatin-etoposide chemotherapy. Early treatment tolerance was good and after two cycles of cytotoxic treatment, a partial response was present in CT. The presented case emphasizes the need for further research to determine the treatment regimens in patients with coexisting IPF and LC and the appropriateness of antifibrotic treatment in them. In addition, it can help to choose the treatment method for similar patients, indicating a combination of carboplatin and etoposide as an effective and, at the same time, relatively safes method in terms of the risk of IPF\'s exacerbation.

UNASSIGNED: Pulmonary fibrosis is one of the sequelae of the COVID-19, which seriously affects the quality of life of survivors. Currently, there are no optimal evidence based guidelines targeting this population.
UNASSIGNED: We report a 66-year-old female patient without underlying comorbidities admitted to Changsha Public Health Center because of COVID-19. During hospitalization, she developed co-bacterial infection and acute respiratory distress syndrome, and received broad-spectrum antibacterial therapy, invasive mechanical ventilation and extracorporeal membrane oxygenation. After the acute phase, she developed post-COVID-19 pulmonary fibrosis subsequently treated with pirfenidone. Over 96 weeks after pirfenidone treatment, her modified Medical Research Council Dyspnea level improved to 2 from 4 at discharge. Her 6 minutes walk test distance, total lung capacity, and diffusion capacity for carbon monoxide all increased. Chest CT performed on 2 years after illness onset showed regressing fibrosis. The Hospital Anxiety and Depression Scale, Athens Insomnia Scale, and 36-Item Short Form Health Survey questionnaire all improved.
UNASSIGNED: Post-COVID-19 pulmonary fibrosis is a challenging consequence of COVID-19, and our case suggests that pirfenidone may be an effective treatment option.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and lethal lung disease characterized by progressive dyspnea and irreversible loss of lung function. Pirfenidone is a novel anti-fibrotic and anti-inflammatory drug, which reduces deterioration in the lung function and prolongs progression-free survival in patients with IPF. However, it has adverse effects including gastrointestinal symptoms, hepatic dysfunction or skin photosensitivity, and rash. Lichenoid drug eruption (LDE) refers to lichen planus-like drug eruption usually presenting symmetric eczematous plaques with a purple hue. To date, numerous cases of LDE due to various drugs and pirfenidone-associated photosensitivity have been reported. However, a case of pirfenidone-induced LDE has been very rarely reported to our knowledge. Herein, is a case of pirfenidone-induced LDE so that clinicians can be aware of the possibility of LDE when using pirfenidone.

Anti-melanoma differentiation-associated protein 5 (MDA5)-positive rapidly progressive interstitial lung disease (RP-ILD) is associated with poor prognosis, and the most effective therapeutic intervention has not been established. Herein we report a case of a 45-year-old female patient who presented with myalgia, Gottron\'s papules with ulceration, and dyspnea on exertion which became aggravated within weeks. Laboratory examination and electromyography confirmed myopathy changes, and a survey of myositis-specific antibodies was strongly positive for anti-MDA5 antibody. High-resolution chest tomography suggested organizing pneumonia with rapidly progressive changes within the first month after diagnosis of the disease. Anti-MDA5-associated dermatomyositis with RP-ILD was diagnosed. Following combination therapy with rituximab, tofacitinib and pirfenidone, clinical symptoms, including cutaneous manifestation, respiratory conditions and radiographic changes, showed significant and sustainable improvement. To our knowledge, this is the first reported case of anti-MDA5-associated dermatomyositis with RP-ILD successfully treated with the combination of rituximab, tofacitinib, and pirfenidone.

Interstitial lung disease (ILD) as an initial manifestation of lupus is rare, especially in young children. Here, we report a case of a 3-year-old boy who presented with fever, shortness of breath, and facial erythema. Clinical examination suggested a diagnosis of active systemic lupus erythematosus (SLE) with butterfly rash, anemia, positive antinuclear antibody, positive anti-double-stranded DNA, and hypocomplementemia. On retrospective review of the patient\'s records, multiple chest computed tomography (CT) images showed non-specific interstitial pneumonia + organizing pneumonia pattern, with no further autoimmune work-up during the visit to a respiratory department. In our opinion, persistent interstitial pneumonia may be a clue to connective tissue disease. The patient received steroid treatment for 1 year, and the radiological and immunological resolution was noted. However, he still suffered from cough and dyspnea. After a 1-year follow-up, he was hospitalized again for SLE relapse. While continuing corticosteroid therapy, the patient was given combination therapy consisting of cyclosporine A (CsA) and monthly-pulse cyclophosphamide for 6 months, and decreased proteinuria was noted. However, the patient\'s respiratory symptoms and pulmonary radiologic findings did not improve significantly. With continued steroid therapy, the patient was started on a daily regimen of CsA and pirfenidone. Both drugs were sufficiently effective to allow gradual reduction of steroid dosage. After 2 years of treatment, marked improvements in symptoms, pulmonary function and chest CT images were observed. Our experience with this case emphasizes that prompt work-up for connective tissue disease (CTD) should be considered in young children with ILD, and pirfenidone might be a useful add-on therapy with immunosuppressive agents for refractory CTD-ILD in pediatric patients. Nevertheless, further clinical trials including larger numbers of patients need to assess the efficiency and safety of this combination therapy for refractory CTD-ILD.