First Report of Two Cases of Löfgren’s Syndrome after SARS-CoV-2 Vaccination-Coincidence or Causality?

Sarcoidosis can present as an acute form or take a chronic course. One of the acute presentations is Löfgren’s syndrome (LS), consisting of the symptom triad of bilateral hilar lymphadenopathy, erythema nodosum, and ankle periarthritis. In addition, there are occasional reports of sarcoid-like reactions following drug exposures. Nevertheless, reports of sarcoidosis or LS after vaccination have not been published. Here, we report two cases of de novo LS in a temporal association with different vaccines against the new coronavirus SARS-CoV-2. One patient developed the first symptoms three days after the second vaccination (first vaccination ChadOx-1, Astra Zeneca; second vaccination CX-024414, Moderna); in the second patient, symptoms started 28 days after the first vaccination (ChadOx-1, Astra Zeneca). Both patients eventually required treatment with glucocorticoids. Both patients achieved clinical improvement with treatment. In conclusion, we report the first two cases of LS shortly after SARS-CoV-2 vaccination.

An Interesting Case of Bilateral Hilar Lymphadenopathy Presented with Visual Impairment

Sarcoidosis is a systemic granulomatous disease of unknown etiology. It primarily affects the lungs and lymphatics; however, the presentation is not always pulmonary. Uveitis is the presenting symptom in 5% of patients, predominantly in females. Cystoid macular edema is the most important sight-threatening sequela of ocular sarcoidosis. Histopathological evidence of noncaseating epithelioid granulomas from a biopsy is the gold standard for the diagnosis of ocular sarcoidosis. Diagnosis of sarcoidosis is made by exclusion of other causes of granulomatous disease, mainly tuberculosis and fungal infection and documentation of involvement of at least one additional organ system. The authors present a case of a patient with sarcoidosis involving lungs and eyes, who had uveitis as an initial presentation.

A tüdő nekrotizáló sarcoid granulomatosisa

Összefoglaló. A nekrotizáló sarcoid granulomatosis a granulomatosus pulmonalis angitisek közé tartozó, ritka kórkép. Egyesek a sarcoidosis variánsának, mások primer pulmonalis vasculitisnek tartják. A kórkép klinikai és patológiai jellegzetességeit két eset bemutatásával ismertetjük. A 20 éves nőbeteg sürgősséggel került pulmonológiai osztályra száraz köhögés, jobb oldali, mély belégzéssel összefüggő mellkasi fájdalom és láz miatt, a 63 éves férfi beteget pedig pneumoniát követő kontroll-mellkasröntgenfelvételen látott elváltozás kivizsgálása során észlelték. Az autoimmun panel vizsgálata, a mikrobiológiai tesztek mindkét betegnél negatívnak bizonyultak, a légzésfunkciós vizsgálat és a bronchoszkópos vizsgálat nem talált eltérést. A mellkas-CT-felvételen lágyrész-denzitású nodulusok látszottak egyoldali dominanciával, a folyamatot nem kísérte a hilusi nyirokcsomók szimmetrikus megnagyobbodása. A nodulusok szövettani vizsgálata vált indokolttá, melyet videoasszisztált torakoszkópos tüdőreszekciós mintavétellel biztosítottak. Mikroszkóposan a tüdőparenchymában gócos nekrózisokat, a környezetükben el nem sajtosodó epitheloid sejtes granulomatosus gócokat, az átfutó artériákban pedig granulomatosus arteritist láttak; a klinikai adatok figyelembevételével a tüdő nekrotizáló sarcoid granulomatosisa diagnózisát állították fel. A tüdőbetegség mindkét betegnél egy év alatt spontán regrediált. Az irodalom adatait és az eseteket összegezve, a tüdő nekrotizáló sarcoid granulomatosisában mikrobiológiai vizsgálatokkal nem igazolható tüdőfertőzés, és az immunológiai kivizsgálás sem tár fel szisztémás autoimmun betegséget; a diagnózis a klinikai kép és a képalkotó vizsgálatok alapján indikált szövettani vizsgálattal állítható fel. A betegség szteroidkezelésre jól reagál, de előfordul spontán regresszió is, az utóbbira láttunk példát. Bár az entitás átmenetet képez a nekrotizáló vasculitisek és a sarcoidosis között, egyre több érv szól amellett, hogy a sarcoidosis spektrumába tartozik. Orv Hetil. 2021; 162(38): 1541–1547. Summary. Necrotizing sarcoid granulomatosis is a rare entity currently classified as a subtype of granulomatous pulmonary angiitis. It is considered to be either a variant of sarcoidosis or a primary pulmonary angiitis. Two cases are demonstrated to present its clinical and pathological features. A 20-year-old female patient was admitted to the department of pulmonology with dry cough, right-sided chest pain during hyperventilation and fever. A 63-year-old male patient was observed with a right-sided lesion on chest X-ray after pneumonia. In both cases, autoimmune panel examination, microbiology tests, spirometry function test and bronchoscopy were unremarkable. Chest CT scans have revealed nodules with soft-tissue density without bilateral hilar lymphadenopathy. In order to clarify the diagnosis, video-assisted thoracoscopic resection (biopsy) was performed. Microscopically, parenchymal focal necrosis with adjacent to non-caseating granulomas and granulomatous angiitis were detected. In both cases, spontaneous remission occurred within a year. Histological examination – integrated with clinical data and radiological tests’ results – is the gold standard form of evaluation to confirm necrotizing sarcoid granulomatosis; furthermore, exclusion of pneumonia and autoimmune diseases are also required. The disease responds well to corticosteroids; moreover, spontaneous remission is often reported, as it happened in both cases. Necrotizing sarcoid granulomatosis is a transition between necrotizing vasculitides and sarcoidosis; although more and more evidence appears supporting the fact that necrotizing sarcoid granulomatosis may belong to the spectrum of sarcoidosis. Orv Hetil. 2021; 162(38): 1541–1547.

Acute arthritis, skin rash and Lofgren’s syndrome

Sarcoidosis is an autoimmune multisystem granulomatous disorder of unknown aetiology, which mainly affects the adults in the age group of 20–39 years. The disease can affect any organ in the body but mainly presents as bilateral hilar lymphadenopathy, pulmonary infiltrates, cutaneous lesions, ocular manifestations and arthropathy. Lofgren’s syndrome is an uncommon initial presentation of sarcoidosis which is recognised by the classical triad of acute arthritis, erythema nodosum and bilateral hilar lymphadenopathy. We describe a newly diagnosed case of sarcoidosis who presented as Lofgren’s syndrome. Acute sarcoid arthritis should be kept as one of the differential diagnoses for patients presenting with acute arthritis and skin lesions; and chest X-ray should be considered to rule out bilateral hilar lymphadenopathy in these patients. Early suspicion and identification of classical clinical features are essential to establish early diagnosis.

Neurosarcoidosis and the Pituitary

Background: Sarcoidosis involves the nervous system in 3-10% of cases. Neurosarcoidosis (NS) can mimic other diseases, leading to delay in diagnosis and management. We present 2 cases of hypothalmo-pituitary (HP) NS with widely differing initial presentations. Case Presentations: Case 1: A 58 year old African American (AA) male presented with decreased libido and hot flashes of 8-10 months duration. He also reported headaches, pain in the face and behind the eyes and dyspnea on exertion. On evaluation, he had central hypogonadism. MRI brain showed thickening and enhancement of pituitary infundibulum (PI). CT chest showed lung lesions and bilateral hilar adenopathies. EBUS with biopsies of lung and hilar lymph node showed non caseating granulomas (NCG). He was started on Prednisone and testosterone leading to symptomatic relief. However, he stopped prednisone after 3 months due to weight gain. He was then started on azathioprine. 9 months later, he presented with recurrence of neurologic symptoms and weight loss of 30 lbs. Labs showed new findings of low prolactin, low IGF-1, central hypothyroidism and central adrenal insufficiency (AI). MRI pituitary showed diffuse thickening of 3rd and 5th cranial nerves and of PI. He was re-started on Prednisone and levothyroxine (LT4) was started with improvement of symptoms. Plan was to start him on Infliximab instead of Azathioprine. Case 2: A 26 year old AA female presented with near syncope and visual impairment in the left eye that started a few weeks prior. Testing showed superior visual field loss and evidence of optic neuropathy, disc edema with mild dyschromatopsia in the left eye. She had had polyuria and polydipsia for the preceding 2-3 months and amenorrhea for 2 years. Lab testing revealed hyperprolactinemia and central DI, hypothyroidism, AI and hypogonadism. Pituitary MRI revealed a suprasellar mass measuring 20 x 19 x 11 mm, displacing the optic chiasm. CT scan showed bilateral hilar lymphadenopathy and lesions in liver and spleen. EBUS and biopsy of mediastinal lymph nodes showed NCG. Patient was treated with IV steroids leading to improved vision. She was started on DDAVP and LT4 and initiated on methotrexate and prednisone on discharge. Discussion: Pituitary hormone abnormalities due to HP NS include anterior hypopituitarism, hyperprolactinemia and DI. The most common (MC) presentation is of DI. Among anterior pituitary hormone deficits, central hypogonadism is the MC. Systemic high dose glucocorticoids (GC) are the mainstay of initial treatment. Optic atrophy is the MC neuro-ophthalmologic manifestation of NS. Only 10 - 20 % of patients have been found to have biochemical improvement of HP axis in response to high-dose GC treatment. Since pituitary involvement commonly leads to permanent endocrine deficits, it is essential to recognize it early and to treat it aggressively to prevent further permanent hormonal and neurologic deficits.

An Orbital Mass Partially Responsive to Steroid

A 68 year old woman presented with a progressively enlarging orbital mass. MRI of her brain and orbits was consistent with an orbital pseudotumor. Although there was some improvement in the patient’s pain and the size of the mass, it did not fully resolve.The patient had a biopsy demonstrate non-caseating granulomatous inflammation. A chest X-ray and CT chest demonstrated bilateral hilar lymphadenopathy. Further examination and interviewing of the patient revealed several months of joint paints and lower extremity nodules. The patient was diagnosed with orbital sarcoidosis and was started on methotrexate by a rheumatologist

Clear Cell Carcinoma of the Thymus: An Improbable Enemy

Introduction: Thymic clear cell carcinoma is the most uncommon subtype of thymic carcinoma, with 20 cases reported worldwide. Case Description: We present the case of a 61-year-old female with dyspnoea and chest pain for 2 days. Computed tomography (CT) angiography showed pulmonary thromboembolism and the existence of mediastinal and bilateral hilar lymphadenopathy, the largest infracarinal with an inferior axis of 25 mm, and also, micronodules on the left pulmonary parenchyma. The patient was admitted for aetiological assessment and underwent anticoagulant therapy. After a month, she had an ischaemic stroke, the sequelae of which proved to be fatal. The autopsy showed a mass in the superior-anterior mediastinum, with dimensions of 11×8×6 cm, corresponding to a thymus signet ring cell primary carcinoma. The immunohistochemistry study revealed that this mass was positive for AE1/AE3, CK5/6 and CK7. Conclusion: The clinical, morphological and immunophenotypic diversity of this tumour makes its diagnosis a difficult multidisciplinary challenge, which requires a high level of clinical knowledge and accurate imaging and histological investigation.

Unsupervised Deep Anomaly Detection in Chest Radiographs

The purposes of this study are to propose an unsupervised anomaly detection method based on a deep neural network (DNN) model, which requires only normal images for training, and to evaluate its performance with a large chest radiograph dataset. We used the auto-encoding generative adversarial network (α-GAN) framework, which is a combination of a GAN and a variational autoencoder, as a DNN model. A total of 29,684 frontal chest radiographs from the Radiological Society of North America Pneumonia Detection Challenge dataset were used for this study (16,880 male and 12,804 female patients; average age, 47.0 years). All these images were labeled as “Normal,” “No Opacity/Not Normal,” or “Opacity” by board-certified radiologists. About 70% (6,853/9,790) of the Normal images were randomly sampled as the training dataset, and the rest were randomly split into the validation and test datasets in a ratio of 1:2 (7,610 and 15,221). Our anomaly detection system could correctly visualize various lesions including a lung mass, cardiomegaly, pleural effusion, bilateral hilar lymphadenopathy, and even dextrocardia. Our system detected the abnormal images with an area under the receiver operating characteristic curve (AUROC) of 0.752. The AUROCs for the abnormal labels Opacity and No Opacity/Not Normal were 0.838 and 0.704, respectively. Our DNN-based unsupervised anomaly detection method could successfully detect various diseases or anomalies in chest radiographs by training with only the normal images.

Branch Retinal Vein Occlusion in a Case of Sarcoidosis

Background: Sarcoidosis is a chronic inflammatory disorder characterized by non-caseation granuloma. It is an inflammatory disorder of unknown etiology. Sarcoidosis has variable ocular presentations from anterior uveitis to rare venous occlusions. Case: We present a rare case of sarcoidosis with bilateral hilar lymphadenopathy and a compatible uveitis. Positive findings of mutton fat keratic precipitates, Koeppes nodule, posterior synechiae, trabecular meshwork nodules, Candle wax dripping sign, Branch retinal vein occlusion and choroidal granuloma in one eye supported the diagnosis of sarcoidosis. Elevated serum acetylcholine esterase and bilateral hilar lymphadenopathy also confirmed the diagnosis of sarcoidosis. Observation: The patient responded well to oral steroids and laser photocoagulation of the vein occlusion area. The patient developed complications of steroid like Herpes Zooster of abdomen and avascular necrosis of femur. Conclusion: Branch Retinal Vein Occlusion is a rare vascular complication in ocular sarcoidosis. Systemic steroids and laser for vein occlusion is mainstay of treatment. Physician must be aware of serious complications of steroid therapy, as seen in this case. Keyword: Sarcoidosis, Branch retinal vein occlusion, Steroids.

Heerfordt’s syndrome: an uncommon manifestation of sarcoidosis

Heerfordt’s syndrome – the combination of facial palsy, parotid swelling, anterior uveitis and fever, is a rare manifestation of sarcoidosis. Here, we present case history of a patient with bilateral lower motor neuron facial nerve palsy, who was found to have bilateral parotid gland swelling and bilateral hilar lymphadenopathy. Computed tomography guided fine needle aspiration cytology from hilar lymph node findings were consistent with sarcoidosis. Subsequently, based on clinical features and cytological findings, the case was diagnosed as incomplete Heerfordt’s syndrome. Birdem Med J 2021; 11(1): 67-69