United States

Background
Antiphospholipid syndrome (APS), a thrombo-inflammatory systemic autoimmune disease, refers to the presence of persistent procoagulatory antiphospholipid antibodies (APLA) in the setting of arterial/venous thrombosis and/or recurrent obstetric morbidity. Primary APS refers to disease manifestation in the absence of clinical or laboratory evidence of another disease. Secondary APS occurs in the presence of another autoimmune disorder, infection, drugs, or malignancy. APS is known to induce a hypercoagulable state which, in rare circumstances following surgical procedures, could present as acute upper limb ischemia. Successful limb salvage and revascularization requires a timely diagnosis and localization of the arterial occlusion.
Case Presentation
We present a case of a 40-year-old female, 10 days post breast implant surgery and reduction abdominoplasty, who developed arterial thrombosis of the right upper extremity and subsequently the right lower extremity. The patient has a 25-pack year smoking history. On her physical exam she had a cold, mottled, cyanotic right hand, thenar area, and thumb. The patient underwent multiple surgical procedures, including a thrombectomy with thrombolytic therapy for a right subclavian clot, subsequent arteriotomy for radial artery occlusion, a femoral and popliteal thrombectomy, and eventually multiple digital amputations, secondary to digital necrosis, with application of a groin flap. Following this procedure, she fell dehiscing the groin flap requiring three additional debridement procedures. Additional testing revealed positive ANA and lupus anticoagulants. She was subsequently diagnosed with antiphospholipid syndrome, explaining the cascade of thrombotic events.
Discussion
Taking into account patients&#39; risk factors is essential in considering the diagnosis of APS. Timely recognition and diagnosis of APS is not only important to guide treatment and management, but also contributes to physicians’ understanding of the psychosocial impact of APS. The patient’s complications were not limited to physical manifestations, as there is a demonstrated psychosocial impact to the diagnosis of APS. This sequence of events contributed to her feelings of depression, which led to her pursuing psychiatric treatment. The large delay in finding the underlying cause of her symptoms certainly added to her anxiety and stress. Further demonstrating the need for consideration and further research investigating the psychiatric manifestations of APS, with an emphasis on depression. Additional considerations should be made regarding the timely diagnosis of APS in order to more aptly care for patients and limit the patients’ psychological toll.

2023 AMA Research Challenge

A Case of Digital Gangrene in a Patient with Antiphospholipid Syndrome

Background
Antiphospholipid syndrome (APS), a thrombo-inflammatory systemic autoimmune disease, refers to the presence of persistent procoagulatory antiphospholipid antibodies (APLA) in the setting of arterial/venous thrombosis and/or recurrent obstetric morbidity. Primary APS refers to disease manifestation in the absence of clinical or laboratory evidence of another disease. Secondary APS occurs in the presence of another autoimmune disorder, infection, drugs, or malignancy. APS is known to induce a hypercoagulable state which, in rare circumstances following surgical procedures, could present as acute upper limb ischemia. Successful limb salvage and revascularization requires a timely diagnosis and localization of the arterial occlusion.
Case Presentation
We present a case of a 40-year-old female, 10 days post breast implant surgery and reduction abdominoplasty, who developed arterial thrombosis of the right upper extremity and subsequently the right lower extremity. The patient has a 25-pack year smoking history. On her physical exam she had a cold, mottled, cyanotic right hand, thenar area, and thumb. The patient underwent multiple surgical procedures, including a thrombectomy with thrombolytic therapy for a right subclavian clot, subsequent arteriotomy for radial artery occlusion, a femoral and popliteal thrombectomy, and eventually multiple digital amputations, secondary to digital necrosis, with application of a groin flap. Following this procedure, she fell dehiscing the groin flap requiring three additional debridement procedures. Additional testing revealed positive ANA and lupus anticoagulants. She was subsequently diagnosed with antiphospholipid syndrome, explaining the cascade of thrombotic events.
Discussion
Taking into account patients' risk factors is essential in considering the diagnosis of APS. Timely recognition and diagnosis of APS is not only important to guide treatment and management, but also contributes to physicians’ understanding of the psychosocial impact of APS. The patient’s complications were not limited to physical manifestations, as there is a demonstrated psychosocial impact to the diagnosis of APS. This sequence of events contributed to her feelings of depression, which led to her pursuing psychiatric treatment. The large delay in finding the underlying cause of her symptoms certainly added to her anxiety and stress. Further demonstrating the need for consideration and further research investigating the psychiatric manifestations of APS, with an emphasis on depression. Additional considerations should be made regarding the timely diagnosis of APS in order to more aptly care for patients and limit the patients’ psychological toll.

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Background: Wound dehiscence is a known postoperative complication but, in cervical spine surgery, it is rare and there is a lack of documented literature. This clinical vignette reports novel complications of wound dehiscence in a patient treated with spinous process ostectomies after posterior cervical surgeries.

Case Presentation
A 41-year-old male initially presented to the emergency department (ED) with a chief complaint of middle thoracic back pain, numbness and tingling in the T4 dermatome and bilateral lower extremity motor deficits. MRI showed T2-T3 discitis, osteomyelitis and a thoracic epidural abscess, central cord remodeling, and a paraspinal soft tissue abscess that measured 2.2 x 1.2 x 2.3 cm. The patient ultimately underwent a T2-T3 decompression with left pedicle subtraction, irrigation, and debridement (I&D) of the T2-T3 disc space, and C7-T5 posterior spinal fusion.
Following an uneventful postoperative course, at five months postoperatively the patient felt a tear over the incision and was found to have a full thickness wound dehiscence with protrusion of the spinous processes. 
The patient underwent operative intervention for resection of the spinous process, I&D, and approximation of the muscle tissue and fascia. The spinous processes of T4 and T5 that were visible were removed. Flaps were raised to allow for adequate coverage of scar and fascia. At the two-week follow-up, the incision showed positive signs of healing overall, although there was some blistering noted; the sutures remained in place for another week. The spinous process at the level above the incision (C6, C7, and T1) was more prominent postoperatively which were not prominent prior to surgery. One week later, sutures were removed, and the wound looked well healed overall despite some erythema.
Six weeks following the second surgery, the spinous process at C6, C7, and T1 had eroded through the skin. The patient was scheduled for resection of those spinous processes and a large paraspinal flap for wound coverage. At the two-week, one-month, and six-month follow-up visits, the incision looked well healed with no signs of dehiscence

Discussion
This clinical vignette documents the successful treatment of cervical wound dehiscence which is a rare postoperative complication of cervical spinal surgery. We believe that this clinical vignette demonstrates successful treatment of cervical wound dehiscence with notable prominence of spinous processes, a topic that is not well documented in the literature. This information is valuable as treatment strategies and research into cervical wound dehiscence are limited to a single case report.




Cervicothoracic Wound Dehiscence Following Internal Fixation of the Cervical and Thoracic Spine

Background: Stress fractures are a type of bone injury resulting from repetitive microtrauma and abnormal bone stress. There are two main types of stress fractures: fatigue fractures (repetitive stress on normal bones) and insufficiency fractures (any injury to weakened bones). This case report highlights a rare and challenging diagnosis of bilateral synchronous fatigue fractures of the proximal tibial metaphysis, accompanied by sprains of the medial menisci and anterior cruciate ligaments.

Case presentation: A 36-year-old male with a BMI of 33 and a sedentary lifestyle for over a decade started jogging and playing cricket matches. He developed pain and swelling in both knees within a week of becoming physically active. There was no history of trauma. Weight-bearing increased the pain. Point tenderness was present along the medial side of the knee joint lines. Tibial traction tests, varus, and valgus stress tests were negative on both legs. McMurray test was negative on the left side and positive on valgus stress with external rotation on the right leg, suggesting a tear on the medial meniscus on the right side. 

He was clinically diagnosed as having a medial meniscal tear on the right knee and left infrapatellar bursitis on the left knee. X-rays of both knees were normal. Initial management with the RICE algorithm (Rest, Ice, Compression, and elevation) and NSAIDs did not improve clinical status. MRI taken after two weeks showed bilateral stress fractures of the proximal tibial metaphysis, bilateral partial tears of the medial menisci, and anterior cruciate ligaments. Lab tests showed elevated ESR, normal Vitamin D and calcium levels, and negative for autoantibodies. Bone densitometry was normal. With the new diagnosis, he was treated with partial immobilization by knee splints with bathroom privileges for three weeks and physiotherapy for 12 weeks. He started walking with support after three months and total weight bearing after six months of initial diagnosis.

Discussion: The diagnosis was initially missed, leading to mistreatment and significant physical, mental, and financial consequences to the patient. This case emphasizes the importance of considering stress fractures and ligament injuries in individuals with recent intense exercise history, particularly in obese patients and obtaining imaging studies earlier. It also underscores the need for evidence-based practices and encourages healthcare professionals to stay updated with current research to improve patient care.

Bilateral Synchronous Proximal Tibial Metaphyseal Stress Fractures with Sprains of Anterior Cruciate Ligaments and Medial Menisci – A Case Report

Background

Parotitis presents in many forms, and the symptoms vary from modest to debilitating. It may
present a diagnostic challenge because of its extensive etiologies: bacterial, pure viral,
autoimmune or a combination of these. Before the development of the mumps vaccine, parotitis
caused by the mumps virus was extremely common, particularly in school-aged children.
However, in recent years, the influenza (type A most commonly) virus has emerged as another
viral etiology associated with parotitis. Given this emerging clinical knowledge, healthcare
workers should start considering influenza as a cause of parotitis, especially during flu seasons.
We present a case of a pediatric patient with a recent positive influenza test who returned to the
Emergency Room with swollen bilateral parotid glands. The patient was diagnosed with parotitis
clinically and it was associated with the influenza virus.

Case presentation

A three-year-old boy with prior medical history of hypoparathyroidism presented to the ER with
swollen bilateral facial edema a day after presenting shortness of breath, runny nose, fever and
cough consistent with influenza. Patient vaccination status was up-to-date, except for the annual
influenza one. Physical examination revealed a well developed and nourished child with nasal
secretions and bilateral facial swelling. He had clear lungs, normal breath sounds and no
respiratory distress. Pertinent labs revealed leukocytosis (12.65), increased CRP (1.8) and
increased amylase levels (241), suggesting inflammation of the parotid glands. Patient underwent
CT of facial bones with IV contrast, which revealed bilateral parotid glands swelling. Given his
upper respiratory symptoms, the patient was diagnosed with bilateral parotitis secondary to
influenza virus infection. He was treated with supportive care, acetaminophen and catch-up
influenza vaccine.

Discussion

It can be challenging to determine whether the temporal association between influenza and rare
complications indicates that the virus is causally associated with the syndrome. However,
physicians should consider the possibility of influenza infection when a patient presents with
parotitis, especially if a temporal relation exists. Patients may benefit because widespread
epidemics of influenza occur in the United States each year and such a public health response
would generally be unnecessary if a case of parotitis were found to be due to influenza rather
than mumps, the most common cause of parotitis.

Is there a link between parotitis and influenza? Physicians should consider influenza virus as an etiology for patients​

Background: 
Total hip arthroplasty (THA) is an effective procedure to improve pain, range of motion, and function for a variety of conditions, including osteoarthritis and post-traumatic arthritis. In the absence of surgical complications, some patients have unexplained persistent pain or weakness at the operative joint. It has been shown that up to 28% of patients had persistent pain at the surgical site 12 to 18 months following THA. Importantly, more than 12% of patients had pain that limited their activities of daily living to a moderate or severe degree. Currently, there are no widely accepted non-pharmacological treatments for persistent postoperative pain for THA. The purpose of this case report is to detail a successful reduction of chronic postoperative pain and weakness with implanted peripheral nerve stimulation (PNS) and physical therapy (PT) in a patient with post-traumatic THA. 

Case Presentation: 
The patient is a 53-year-old white male pilot who presented to our pain management clinic in May 2022. In February 2021 he was ejected from an airplane and had polytrauma with multiple facial fractures, an L4 vertebral compression fracture, and a right hip fracture. He had an immediate uncomplicated THA surgery followed by standard physical therapy treatment. Approximately five months following THA, he developed right-sided thigh pain, numbness, and tingling in the femoral nerve distribution, which was recalcitrant to medication and standard physical therapy. 
SPR Therapeutics single-lead percutaneous peripheral nerve stimulator was implanted near the right femoral nerve on 3/10/23. During four months of treatment, the patient attended 11 visits of physical therapy. 

Discussion: 
This case report demonstrates the effectiveness of implanted PNS in a patient with chronic pain and weakness following post-traumatic THA. This treatment improved his hip flexion strength, measured by handheld dynamometer, from 22.83lb to 50.8lb in three weeks. Limb symmetry index improved from 58.19% to 91.87%. His Lower Extremity Functional Scale improved from 35/80 (43.75%) to 54/80 (67.5%) within five weeks. 

Although peripheral nerve field stimulation has been used previously for this indication, single lead implanted PNS has not been studied. This case’s success demonstrates PNS may help eliminate post-operative persistent pain and weakness in many patients, including those with post-traumatic arthroplasty. Future randomized control trials using implanted single-lead PNS are warranted to provide robust results to guide physician clinical practice.

Implanted Peripheral Nerve Stimulation Improves Chronic Pain and Weakness Following Total Hip Arthroplasty

Background
Delirium, defined as a disturbance in attention and awareness that cannot be better explained by a medical condition1, is a serious complication of hospitalization for the elderly population, impacting 8-17% of older adults in the emergency room and up to 40% of nursing home residents. Development of delirium is associated with long term poor health outcomes, including dementia, institutionalization, and death. While the presentation of delirium can sometimes mimic a catatonic state with psychomotor retardation, echolalia, the inappropriate repetition of words spoken by others, is not common. More frequently, echolalia is cited as a symptom of catatonia. This case report describes a patient with minimal psychiatric history who experienced oxycodone-induced delirium, with unique primary presenting symptoms of echolalia and narration. A thorough search of existing literature has found no similar examples.

Case Presentation
We present a case report of an 80-year-old female who presented with altered mental status and mental status exam significant for overproduction of speech and echolalia. The patient was found to have a recent history of spinal surgery performed at an outside hospital and was discharged to home on baclofen and oxycodone four days prior to admission. Extensive diagnostic work-up that included routine blood work, blood and CSF cultures, EEG, and neuroradiological studies were all within normal limits. The patient’s echolalia and narration extinguished and her mental status improved by day three of admission after discontinuing oxycodone and empiric treatment with acyclovir and thiamine.

Discussion
Narcotic analgesics have long been explained as a cause of delirium in elderly patients. This patient showed acute emergence of symptoms following onset of treatment with opioid analgesic, oxycodone. Although this presentation is complicated by the patient’s recent operation, lack of symptoms post-op and the disappearance of symptoms following cessation of the drug suggest that oxycodone was the cause of her delirium. Notably, this patient presented to the hospital exhibiting echolalia, the inappropriate repetition of words spoken by others. These behaviors are more commonly associated with catatonia, and have yet to be described as a primary presenting feature of delirium.

In conclusion, physicians should be aware of the possible deliriogenic properties of all narcotic substances, including oxycodone. Furthermore, atypical presenting symptoms of delirium including echolalia and narration should be considered when establishing a diagnosis. Additional research is warranted to examine whether such symptoms are more commonly associated with delirium in general or that associated with narcotics than is currently suspected. 



Echolalia as the Presenting Symptom of Opioid-Induced Delirium: A Case Report

Background
A paucity of literature delineates psychiatric symptoms in osmotic demyelination syndrome (ODS). Though clinical manifestations of ODS are canonically limited to the neurologic, some existing literature has identified psychiatric sequelae of ODS including: psychosis, catatonia, mania, behavioral changes, and cognitive disturbances. This case report discusses psychiatric sequelae of ODS, specifically manic-like symptoms, and the importance of judicious medical work-up for new onset psychiatric symptoms. This neuropsychiatric case highlights interdisciplinary collaboration between inpatient psychiatry and neurology in promptly recognizing and treating a neurologic emergency. 
Case Introduction
A 37-year-old female with a past psychiatric history of generalized anxiety disorder, no history of inpatient psychiatric admissions, initially presented to inpatient psychiatry with new onset: lack of sleep, disorganization, distractibility, behavioral disinhibition, destruction of property, complaint of foot tremor and drooling. On initial inpatient psychiatric exam, the patient presented with seemingly manic symptoms and notable cranial nerve deficits including: drooling, diplopia, dysphagia, dysdiadochokinesia, dystonic posturing of bilateral lower extremities, postural and action tremor of bilateral upper extremities, gait instability. It was noted that 8 days prior to psychiatric presentation, the patient had been admitted to the intensive care unit for severe hyponatremia and prolonged QTc following elective submental liposuction. This constellation of symptoms was concerning for ODS, prompting immediate MRI and neurology consult. 
Results
MRI brain demonstrated marked symmetric T2 FLAIR hyperintense signal in the bilateral putamen, bilateral caudate heads, and the cortex of the bilateral hand knobs, without associated reduced diffusion. Within the central pons, there was focal T2 hyperintense signal. Findings were consistent with acute/subacute osmotic demyelination and extrapontine osmotic demyelination, in the setting of recently overcorrected hyponatremia. 
The patient was immediately transferred to the neurointensive care unit for stabilization with IVIG and Decadron taper. Behavioral disinhibition resolved with short-course valproic acid. Upon neuropsychiatric stabilization, the patient was discharged home with outpatient physical rehab, occupational rehab, neurology, psychiatry, plastic surgery, and primary care follow-up. 
Discussion
Medical etiologies of seemingly manic presentations must be judiciously worked-up prior to diagnosing primary psychiatric etiology. ODS can present with psychiatric symptomatology in addition to neurologic. This case demonstrates mania-like symptoms secondary to general medical condition. Interdisciplinary collaboration was critical in pursuing timely medical work-up and treatment of ODS, thus preventing serious adverse outcomes such as progression to locked-in syndrome. Inpatient psychiatry promptly recognized a neurologic emergency through its psychiatric manifestation, allowing for immediate neurointensive treatment and stabilization.

Psychiatric Symptoms in Osmotic Demyelination Syndrome: A Neuropsychiatric Treatment Effort

Background
Recent evidence suggests that the high rates of comorbidity between obsessive-compulsive symptoms (OCS) and psychosis may warrant the diagnosis of a specific condition known as schizo-obsessive disorder. Nonetheless, the lack of official diagnostic criteria in the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5) for this condition and limited research on optimal management for the overlapping symptom presentations complicate both diagnosis and treatment.

Case Presentation
CM is an 18-year-old female with a history of five past psychiatric hospitalizations resulting in various diagnoses including obsessive-compulsive disorder (OCD); anxiety; hypothyroidism, psychosis, unspecified; and schizoaffective disorder. She was brought to the hospital by ambulance after a physical dispute with her mother following months of worsening agitation. Her history is also notable for severe anxiety preventing her from attending school, compulsions causing her to command her mother to perform repetitive actions (resulting in significant interpersonal conflict), command auditory hallucinations involving angels and the devil, and persecutory delusions of being watched in public. Treatment with fluphenazine and risperidone in two prior hospitalizations resulted in the development of extra-parkinsonism symptoms (EPS). She has repeatedly displayed non-adherence to medications including sertraline and levothyroxine between hospitalizations, reporting both side effects and ineffectiveness in managing her symptoms. Throughout this active hospitalization, CM has endorsed intrusive audio-visual thoughts and isolative behavior. She also displays avolition, anhedonia, flat affect, thought blocking, and negativism. CM continuously fluctuates between acknowledging and denying her symptoms, indicating poor insight into her condition. She is currently being transitioned from oral to intramuscular paliperidone to manage her symptoms of psychosis, as well as oral clomipramine to treat her OCS. She is under routine monitoring for EPS and signs of adverse drug interactions.

Discussion
CM’s significant history of recurrent psychiatric hospitalizations, her unstable relationship with her mother, and her poor insight into her condition may be partially explained by the inconsistencies in her diagnoses over time. With schizo-obsessive disorder, difficulties in distinguishing obsessive and compulsive symptoms from potential delusions secondary to psychosis can result in unstandardized, overcomplicated treatment plans. This can lead to adverse effects such as EPS, worsening of either OCS or psychotic symptoms, medication non-adherence, or deleterious drug interactions. Further research is warranted to better understand the intersection of OCD and psychotic disorders such as schizophrenia. In addition, more research should be conducted on improving therapeutic practices and pharmacological treatment plans that target the related symptomatology while minimizing adverse effects.

Where Obsession Meets Psychosis: A Case Study on Co-Occurring OCD and Schizophrenia in Adolescents

Background
Pulmonary sarcomatoid carcinoma is a rare subtype of non-small cell lung carcinoma (NSCLC) characterized by the presence of sarcoma or sarcoma-like elements. It represents 0.2 to 1% of all lung cancers. PSC is identified by the presence of spindle and/or giant cells, which make up a minimum of 10% of the tumor or comprise the entire carcinoma.
Case Presentation 
A 49-year-old male with a medical history of cerebrovascular accident (CVA) complicated by chronic ambulatory difficulties, type 2 diabetes mellitus, chronic hydrocephalus, hypertension, hyperlipidemia, and clinically suspected chronic obstructive pulmonary disease, tobacco dependence with a history of over 50-pack years presented from a skilled nursing facility due to worsening dyspnea, acute on chronic progressive productive cough with clear to white sputum, moderate in amount. Upon presentation, the patient's vitals and initial labs were unremarkable, except for mild leukocytosis. However given the increased work of breathing on the exam, a CXR was ordered which was suggestive of consolidation in the right upper lobe. The patient was started on empiric antibiotic therapy for possible community-acquired pneumonia however, it failed to improve. This prompted a CT scan of the chest, revealing a large mass involving the right upper lobe measuring approximately 14.6 x 13.1 x 12.2 cm (anteroposterior and superior-inferior). The right upper lobe bronchus was not visible, raising suspicion of bronchogenic carcinoma. The mass caused compressive atelectasis of the right middle lobe and it was invading the upper portion of the middle mediastinum and posterior mediastinum, exerting mass effect on the esophagus. CT-guided biopsy of the mass revealed markedly atypical cells consistent with sarcomatoid non-small cell cancer, likely squamous cell lung cancer. Immunohistochemical staining was only positive for p63. Based on the labs, imaging, and biopsy results, the patient was diagnosed with Pulmonary Sarcomatoid Non-Small Cell Cancer, arising from the right upper lobe bronchus. The mass exhibited invasive properties, compressive atelectasis, and mass effect on adjacent structures in the mediastinum. Unfortunately, the patient clinically deteriorated before initiating treatment and was ultimately transitioned to inpatient hospice. 
Discussion 
With this case, we would like to reinforce the point that in patients with a history of smoking, and COPD and with initial imaging suggestive of a benign infectious process fitting with clinical exam, a low threshold must be maintained to further investigate with CT imaging, especially if patients do not improve with first-line therapy as malignant tumors may often mimic the appearance of a consolidative process which can further delay treatment and lead to a poor outcome.

Sarcomatoid NSCLC: A Rare Entity with Unpredictable Behavior

Background
Alpha-1 Antitrypsin Deficiency (AATD) is an underrecognized genetic condition affecting an estimated 1 in 2,000 to 1 in 5,000 individuals worldwide, with approximately 70,000 to 100,000 cases reported in the United States. Alpha-1 Antitrypsin (AAT) plays a crucial role in protecting the lungs by inhibiting the activity of neutrophil elastase (NE), but in cases of AAT deficiency, uncontrolled NE activity can lead to lung damage through quantum proteolysis. Recurrent spontaneous pneumothorax is a significant complication observed in patients with underlying lung diseases like AATD and chronic obstructive pulmonary disease (COPD). The association between AATD and spontaneous pneumothorax remains relatively unexplored, presenting unique challenges in managing recurrent pneumothorax in these patients. This case report aims to provide valuable insights into the clinical features, diagnostic workup, and therapeutic interventions and associated challenges in managing a patient with recurrent spontaneous
pneumothorax in the context of AATD and COPD, with the objective of offering diagnostic and therapeutic considerations for similar cases.

Case Presentation
61 yo M with A1AT deficiency with severe bullous disease, no known cirrhosis, OSA intolerant of CPAP, hx of recurrent Lt pneumothorax and associated COPD presented with dyspnea. Examination revealed absent lung sounds on the left side. Oxygen support was initiated, and a chest x-ray confirmed left-sided pneumothorax. The patient underwent a successful left-sided pigtail catheter placement procedure to relieve the pneumothorax. This was his fourth episode of Secondary Spontaneous Pneumothorax. Two episodes occurred when he was younger, and the most recent two episodes required the implantation of a left chest tube. The very last episode
appeared on 5/2021. The patient's history of heavy smoking, substance abuse, and occupational asbestos exposure contributed to the severity of respiratory issues. Although his condition improved with pulmonary rehabilitation and medical therapy, the limitations of available interventions underscore the need for individualized approaches in complex cases. Lung volume reduction surgery and endobronchial valves were not viable due to extensive lung involvement and airway hyperdynamic collapse. The recommendation for lung transplantation aimed to enhance the quality of life and life expectancy. Pleurodesis emerged as a potential option if transplantation was not feasible. Further investigations were conducted for the transplant workup, leading to approval by the Medical Review Board. Following the successful placement of the Heimlich valve, the patient was discharged with instructions for outpatient follow-up.

Discussion
This case emphasizes the association between AATD and Recurrent secondary spontaneous pneumothorax, bringing attention to the difficulties encountered in the management of this condition. Further research is needed to optimize treatment approaches, explore the efficacy of alternative interventions, and understand long-term outcomes in this complex patient population.

Alpha-1 Antitrypsin Deficiency Unleashed:
A Case Report On Tackling Recurrent Secondary Spontaneous Pneumothorax

Idiopathic Pulmonary Fibrosis (IPF) is a spontaneously occurring, progressive, fibrosing disease of the lung, most commonly identified in adults 50 years of age and older.[1] Diagnosis may be made via high-resolution CT (HRCT) when a Usual Interstitial Pneumonia (UIP) pattern is present; however, if the diagnosis remains questionable, surgical lung biopsy (SLB) is often pursued.[2]

We present the case of a 39-year-old African-American male with a medical history of bronchiectasis accompanied by chronic, productive cough and social history including ongoing tobacco use and prior shipyard employment. He presented with two weeks of progressive exertional dyspnea, increased sputum production, and fever. 

Examination revealed tachycardia, bilateral inspiratory rales and digital clubbing. Progression of baseline bilateral interstitial opacities was noted on chest x-ray, and HRCT revealed diffuse interstitial thickening and ground-glass opacities with basilar predominance, and diffuse bronchiectasis. Evaluation for alpha-1 antitrypsin deficiency, cystic fibrosis mutations, celiac disease, autoimmune serologies, and immunoglobulin deficiency were unremarkable. Bronchoalveolar lavage analysis demonstrated neutrophil predominance with negative microbiologic testing. He ultimately underwent SLB of the right upper and lower lobes demonstrating diffuse alveolar damage superimposed on patchy collagen fibrosis with associated architectural distortion in the form of honeycomb changes consistent with UIP. Per the 2019 IPF guidelines, he was diagnosed with IPF and given an “IPF Likely” grade.[2]

IPF is thought to develop from repetitive micro-injury to chronically dysregulated alveolar epithelium, though the exact etiology remains undetermined. Our patient’s tobacco history or occupational exposures may have been the nexus for his interstitial lung damage[1] 

UIP is the radiologic/histopathologic hallmark of IPF, demonstrating reticular fibrosis and subpleural honeycombing with basilar predominance. Bronchiectasis in IPF is the result of fibrosis applying traction to the airways with diffuse bronchiectasis being uncommon.[2,3] Conversely, our case demonstrates a diffuse bronchiectatic process that preceded significant fibrotic changes of IPF. Whether the development of IPF is attributable to recurrent injury from exacerbations of idiopathic bronchiectasis, or this patient’s bronchiectasis was an unappreciated indicator of an underlying, progressive ILD is difficult to ascertain.

This case raises questions about the potential benefit of periodic imaging in bronchiectasis patients. Society guidelines only recommend it at time of diagnosis and clinical deterioration.[2,4] We argue that periodic imaging should be tailored to bronchiectasis etiology, as otherwise unexplained disease may be an indicator of risk for ILD development.

We encourage physicians to be mindful that patients with bronchiectasis may be at risk for IPF development.

1. Richeldi L, Collard HR, Jones MG. Idiopathic pulmonary fibrosis. Lancet. 2017;389(10082):1941-1952. doi:10.1016/S0140-6736(17)30866-8
2. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2022;205(9):E18-E47. doi:10.1164/rccm.202202-0399ST
3. Piciucchi S, Tomassetti S, Ravaglia C, et al. From “ traction bronchiectasis ” to honeycombing in idiopathic pulmonary fibrosis : A spectrum of bronchiolar remodeling also in radiology ? BMC Pulm Med. Published online 2016:1-4. doi:10.1186/s12890-016-0245-x
4. Lynch DA, Sverzellati N, Travis WD, et al. Diagnostic criteria for idiopathic pulmonary fibrosis: a Fleischner Society White Paper. Lancet Respir Med. 2018;6(2):138-153. doi:10.1016/S2213-2600(17)30433-2

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