Deciding a Treatment Plan for an Older Patient With Severe Idiopathic Pulmonary Fibrosis: A Case Report
Pulmonary Embolism Explained: Symptoms, Diagnosis, Treatment
Every year, 900,000 Americans are caught by surprise by the symptoms of a pulmonary embolism: a sudden shortness of breath, chest pain and a rapid heartbeat.
Described by Dr. Talal Dahhan, Division Chief for Pulmonary & Critical Care, Baystate Pulmonary, pulmonary embolism (PE) is a serious and potentially life-threatening condition. "But with prompt diagnosis, 92% of PE patients survive and, with proper management, go on to live healthy and full lives."
What is a Pulmonary Embolism?As Dr. Dahhan explains, "A PE is a blood clot that forms in the body—most often the legs—that breaks loose and travels to the blood vessels in the lungs where it blocks the normal flow of blood in the lungs. Many PEs—70-80%—end up getting stuck and blocking small blood vessels.
When the supply of blood that carries oxygen to other parts of the body gets restricted by the clot, it leads to a very sudden shortness of breath. In some cases, a small clot may be pushed or moved so that normal blood and oxygen flow resumes quickly, and the person will feel fine. But in the case of big clots, a large restriction of blood and oxygen can lead to cardiac arrest and loss of consciousness.
Regardless of the size of the clot, PE is very serious and can lead to lung damage and, in some cases, death."
Symptoms of Pulmonary EmbolismIt's critical to seek medical attention if you experience any symptoms of PE, including:
"Even if the shortness of breath or chest pain passes quickly, it's important to see your doctor," urges Dr. Dahhan. "While the blood clot may have moved on, it's still in your blood stream and poses a threat to your health and life."
What Causes Pulmonary Embolism?The root cause of PE is the formation of clots in the body. "Under normal circumstances," says Dr. Dahhan, "the body forms clots to stop bleeding from damaged blood vessels and allow the body to heal. Damage typically stems from an injury, trauma or even medical procedures including device implantation, invasive screenings and surgery. For most people, clots dissolve naturally and are taken up by the body." But as he notes, certain factors can increase the likelihood a clot may remain persistent and potentially break free.
"As we age, our blood naturally thickens," he says. "This increases the tendency for blood to clot and, thus, increases the chance of developing a clot that can lead to PE."
Complicating the issue for older adults is that fact that many conditions that develop as one ages often lead to people becoming less active, which can increase the risk of clotting.
Risk Factors for Pulmonary Embolism"Conditions including congestive heart failure, chronic obstructive pulmonary disease (COPD), and interstitial lung disease, can all lead to a shortness of breath," Dr. Dahhan explains. "The response of many is to become less active. But this lack of activity allows blood to pool in the lower extremities, which can increase the risk of clotting."
"Even when there aren't underlying health issues, simply being stationary—say, driving or flying for six hours or more—can increase the risk of clotting. The same is true post-surgery when the body is actively creating clots. This is why surgical patients, especially those undergoing joint replacement, are made to get up and walk even just a few hours after surgery. Movement helps stimulates circulation, which reduces clot risk."
Other factors that can increase PE risk include:
The symptoms of PE are similar to those associated with other serious conditions affecting the heart and lungs, so getting a quick diagnosis is critical.
When assessing patients experiencing symptoms of PE, the Baystate Pulmonary team relies on a clinical prediction tool called the Well's Score. Dr. Dahhan explains, "The Well's Score allows us to consider symptoms and clinical risk factors to determine the probability of PE versus another condition. Different symptoms and factors are assigned point values. The more points you have on the Score, the more likely it is that you have PE. The Score also guides what steps we'll take next to confirm a PE diagnosis if the score is leaning that way."
Common next steps for confirming a diagnosis include:
While PE can be mild or severe, the focus of treatment remains the same: keeping a clot from getting bigger, restoring blood flow, and preventing new clots from developing.
Dr. Dahhan notes that common treatment options include:
Blood thinners: Also referred to as anticoagulants, blood thinners are often the first line of treatment for patients with PE. Given intravenously or taken orally, blood thinners help to break up existing clots and keep new clots from forming.
Depending upon the patient's condition and history, they may be required to take blood thinners even after the initial clot has been resolved. In some cases, patients remain on thinners for several months while others take them for the remainder of their lives.
Systemic thrombolysis: Often called "clot busters," thrombolytic medications are used for severe cases of PE and administered by IV or by a catheter that's positioned near the site of the clot.
Surgical embolectomy: Performed only in the most severe cases or when other treatments are not resolving the clot or are not an option for a patient, an embolectomy is a minimally invasive procedure that involves surgically removing the clot.
Managing and Preventing PEOnce you've experienced PE, managing it becomes a lifetime commitment.
Dr. Dahhan says, "Most patients with PE are able to live successfully with PE provided they stay on top of their condition and takes steps to reduce risks."
In addition to consistently taking any medication prescribed for PE, patients need to make sure all their healthcare providers—including dentists or oral surgeons—are aware of their PE history and current treatment regimen.
"Here at Baystate, providers actively work across disciplines and collaborate to provide the best care based on the patient's specific needs and severity of their condition," he notes. "Together we're able to create an optimal care plan and make adjustments based on patient's response to treatment and in advance of any required procedures that may be impacted by the care plan."
Patients of Dr. Dahhan and other members of the Baystate Pulmonary team also encourage patients to:
Whipple's Disease, One Of Medicine's Great Imitators: A Case Report
Key Points1. The prevalence of Whipple's disease is likely to be vastly underestimated due to the symptoms presenting a diagnostic challenge, resulting in underdiagnosis.2. In this case report, the authors report a case of Whipple's disease where the initial presenting symptoms occurred 7 years prior to diagnosis and imitated various other multi-system diseases.3. Clinicians should consider investigating for Whipple's disease even if the 'classical' symptoms do not appear to be present, and should maintain awareness of complications such as pulmonary arterial hypertension.
INTRODUCTIONThe authors present a rare case of Whipple's disease (WD) in a patient with a background of pulmonary hypertension and on treatment that presented with chronic wasting disease and severe anaemia. The diagnosis was unexpected as there was a strong suspicion of malignancy given the nature of the presentation and investigation findings of mediastinal lymphadenopathy. WD can be seen as a 'great imitator' given its multisystem presentation and is often challenging to diagnose.1 Interestingly, this case was likely associated with pulmonary arterial hypertension, which had developed years prior to diagnosis, a rare but reported complication of WD.2
CASEA 46-year-old Caucasian woman was admitted to the authors' centre with severe symptomatic anaemia, which had required iron replacement therapy and blood transfusions over the preceding 6 months. In addition, they reported significant weight loss (20 kg), nausea, and poor appetite for 1 year prior to presentation. The patient denied any other symptoms, including diarrhoea, skin lesions, arthralgia, fevers, or neurological disturbance.
The patient had a background of learning disability and idiopathic primary pulmonary arterial hypertension (PAH). This had been diagnosed following an episode of hospitalisation with pericardial effusion and pulmonary oedema 7 years prior to presentation and was under long-term follow-up at a regional pulmonary hypertension centre after being commenced on macitentan and sildenafil. No other causes for the PAH were found at that time.
Outpatient investigations preceding admission included a CT scan that revealed mediastinal lymphadenopathy and a possible lymphoproliferative disorder. However, a bone marrow biopsy did not confirm this diagnosis, nor did endobronchial ultrasound-guided lymph needle biopsy. Their condition progressed, and they developed ascites requiring paracentesis for symptomatic relief.
Following hospital admission, a full blood count revealed haemoglobin levels of 55 g/L (reference range: 130–175 g/L). Further blood tests showed evidence of malabsorption with red cell folate 2.5 µg/L (reference range: 3.1-20 µg/L), serum iron 2.9 µmol/L (reference range 9–30.4 µmol/L), transferrin saturation 8%, serum albumin 15 g/L (reference range: 35–52 g/L), and 25-hydroxyvitamin D 22 nmol/L (reference range: <30 nmol/L; vitamin D deficiency).
Blood pressure, respiratory rate, oxygen saturation, and temperature were normal. BMI at admission was 13.2 with a weight of 37.5 kg. Further investigations were performed to identify the cause of anaemia and look for evidence of lymphoproliferative malignancy. CT imaging of the abdomen confirmed anterior peritoneal thickening, raising concerns for peritoneal malignancy. Further diagnostic procedures performed included a gastroscopy with duodenal biopsies (Figure 1), a colonoscopy, peritoneal biopsy, and ascitic drainage. Ascitic fluid cytology revealed a population of clonal B cells by immunophenotyping. Significant clinical events during hospital admission included the development of a left lower limb deep vein thrombosis and a subdural haematoma.
Figure 1: Endoscopic image of duodenum.
Histological examination of the duodenal biopsies revealed foamy macrophages consistent with a diagnosis of Whipple's disease (WD; Figure 2), and periodic acid-Schiff stain was positive (Figure 3). Tropheryma whippelei DNA was subsequently detected via PCR of the duodenal tissue, whole blood, and ascitic fluid samples. No features of malignancy were detected on histological examination of tissue biopsies and ascitic fluid.
Figure 2: Haematoxylin and eosin stain of duodenal biopsy (x100).Duodenal mucosa showing a lamina propria markedly expanded by numerous foamy macrophages.
Figure 3: Periodic acid-Schiff stain of duodenal biopsy (x10).Green arrow identifies T.Whipplei bacteria.
The patient was transferred to the care of the infectious diseases team and received four weeks of intravenous ceftriaxone 2 g once a day. Cerebrospinal fluid examination was not possible due to the potential complications of concurrent subdural haematoma and anticoagulation associated with lumbar puncture; therefore, the initial phase of treatment was selected to provide cover for probable central nervous system involvement. Echocardiogram confirmed no features of infective endocarditis were present.
Inflammatory markers and haemoglobin were normalised during the 8-week admission. The patient was discharged with a follow-on regimen of oral co-trimoxazole 960 mg twice a day for 12 months. At the time of discharge, the patient's BMI was 16.2 with a weight of 38.9 kg (with no ascites). At a 6-month follow-up appointment, the patient's weight had increased by a further 14 kg, with no side effects of the antibiotic course and a complete clinical recovery.
DISCUSSIONWD is caused by the gram-positive bacteria T.Whippelei and is characterised by progressive malabsorption, usually accompanied with gastrointestinal symptoms such as diarrhoea, abdominal pain, and weight loss, often for many years before diagnosis. It is a multisystem disorder with fever and arthralgia being cardinal symptoms in many cases.1 Anaemia and hypoalbuminaemia are often present as a manifestation of malabsorption syndrome. Lymphadenopathy can also be seen.3 Cardiac involvement is thought to be present in one-third of patients, with endocarditis an acknowledged manifestation. Pericarditis has also been reported at autopsy in up to two-thirds of cases.4
Host factors are thought to play an important role in the pathogenesis of WD. Patients with WD are thought to have impaired immune function of monocytes and macrophages, resulting in a muted inflammatory response to the organism that is carried in the gastrointestinal tract.2 Reduced expression of CD11b, the α-chain of the phagocytic receptor CR3, has been implicated in particular.2 The organism has a predilection for small-bowel macrophages; hence, duodenal biopsy with periodic acid-Schiff staining is the gold-standard method for diagnosis.1An important consideration in this case is the onset of PAH 7 years prior to the diagnosis of WD. This was treated with macitentan and sildenafil. Macitentan is an endothelin receptor antagonist approved for use for PAH. It works by occupying a pocket in the endothelin A receptor, blocking the action of endothelin-1, a potent vasoconstrictor which is found in increased levels in the plasma and pulmonary vascular endothelium of patients with PAH.5 PAH has been reported, although thought to be exceedingly rare in WD. At the completion of antibiotic treatment, clinical resolution of PAH symptoms was noted, and repeat echocardiogram revealed no evidence of PAH. It is likely that this was the initial presentation of WD given the apparent reversal of PAH with targeted antibiotic treatment. The pathophysiology of WD-PAH is poorly understood; however, evidence of bacterial invasion of the tunica media has been reported on histological examination, which may play a role in vascular resistance.6,7 Following the resolution of PAH with WD treatment, sildenafil and macitentan were gradually stopped with careful monitoring for recurrence.
The time to diagnosis from symptom onset in this case was approximately 18 months. WD is regarded as a rare diagnosis; however, the exact prevalence is unknown. One European epidemiological study estimated a prevalence of three in one million.8 The diagnosis can be challenging owing to the variety of clinical signs and symptoms that can mimic other systemic disorders, in particular malignancy, as in this case. This can result in a considerable delay between presentation and diagnosis. This case demonstrates the importance of considering investigating for WD in patients who present with evidence of severe systemic inflammation, anaemia, or malabsorption of chronic duration, even if the cardinal symptoms of arthralgia, fever, or diarrhoea are not reported.
The authors believe that this is the first reported case of WD with initial presentation of pulmonary arterial hypertension years prior to WD diagnosis.
Patient PerspectiveI have found taking antibiotics for a long time very hard. I feel very happy the treatment has worked, and I feel normal again. My family said I am much better and have put on weight. I am glad my life is back to normal again.
Evidence of Patient-Informed ConsentThe authors obtained written, informed consent from the patient to publish the case report and any accompanying images prior to publication.
Medications And Drugs For Treatment Of Pulmonary Arterial Hypertension
Pulmonary arterial hypertension can be a serious condition affecting the heart. It is defined as mean pulmonary arterial pressure >25mm Hg at rest and >30mm Hg on exertion.
EpoprostenolEpoprostenol is a prostaglandin, prescribed for pulmonary arterial hypertension.
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IloprostIloprost is a synthetic analogue of prostacyclin, prescribed for pulmonary arterial hypertension (PAH).
TreprostinilTreprostinil is a synthetic analogue of prostacyclin, prescribed for pulmonary atrial hypertension (high blood pressure in the lungs).
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