Israel Medical Association Journal

IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition. Accumulated research and data since 2003 have increasingly affirmed its classification as a systemic disease. Although IgG4-RD generally presents with involvement of one or several organ systems, it can affect almost any organ. The disease manifests gradually with the development of tumor-like lesions.

Constitutional symptoms, such as systemic fever and elevated inflammatory markers, are generally absent in association with this pathological condition. However, it is common for patients to exhibit allergy characteristics in addition to a slight increase in peripheral blood eosinophilia. The clinical manifestations of the disease exhibit variability on the specific organ system affected. Specific involvement of organs can ultimately result in organ failure. For example, retroperitoneal fibrosis commonly leads to post-renal failure [1]. The 2020 Revised Comprehensive Diagnostic (RCD) criteria for IgG4-RD include three components for diagnosis: clinical and radiological components (1), serological diagnosis (2), and unique pathological diagnosis (3). When all three are present it is a definitive diagnosis. The presence of components 1 + 3 constitutes a probable diagnosis, while the presence of components 1 + 2 indicates possible diagnosis [2].

Testicular involvement of IgG4-RD has been described in a few case reports. However, the uniqueness of our case is the manifestation of a testicular mass as a different target organ of relapse in a patient in remission from retroperitoneal fibrosis.

Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting from the accumulation of misfolded protein aggregates in the myocardial extracellular space. Cardiac amyloidosis stems primarily from the misfolding of monoclonal immunoglobulin light chains (AL) originating from abnormal clonal plasma cell proliferation or transthyretin amyloidosis (ATTR).

Diagnosis of amyloidosis demands a high index of suspicion and requires histological confirmation of pathognomonic green birefringence under polarized light when an infiltrated tissue specimen is stained with Congo-red staining [1,2].

Pleural involvement of systemic amyloidosis has rarely been reported and is considered a serious complication [3]. Pleural amyloidosis is diagnosed with the identification of amyloid deposits in pleural biopsy specimens. However, pleural biopsies are rarely performed for this indication.

We describe the case of a patient with AL cardiac amyloidosis presenting as intractable pleural effusion and diagnosed via pleural biopsy.

Reversible cerebral vasoconstriction syndrome (RCVS) comprises a group of conditions characterized by reversible vasoconstrictions of cerebral arteries. Clinical manifestations include sudden-onset severe headaches with or without additional neurologic signs and symptoms [1].

The incidence of RCVS is 2.7 cases per million adults. It predominantly affects women, and about 9% of all RCVS cases occur during the postpartum period [2,3]. Other possible precipitating factors, such as subarachnoid hemorrhage, ischemic stroke, intracranial hemorrhage, and exposure to vasoactive drugs, have also been reported in association with RCVS [2]. The exact pathophysiology of RCVS is not well understood, although hormonal influences have been suggested as possible contributing factors.

Alkalosis-induced cerebral vasoconstriction is described but not well understood. Hyperventilation is commonly used in neurologic patients to decrease intracranial pressure and cerebral blood flow. Hyperventilation causes cerebral vasoconstriction directly by hypocapnia and may indirectly affect through alkalosis.

We present a case of RCVS in a postpartum patient admitted to the intensive care unit (ICU) with severe metabolic alkalosis necessitating hemodialysis.

Higher potency bisphosphonates, typically intravenous formulations, are given at lower doses for postmenopausal women. The treatment has improved compliance compared to daily oral therapy. Since bisphosphonates are exclusively excreted via the kidneys, intravenous formulation has been associated with deterioration of renal function, specifically in the setting of preexisting renal disease or concomitant use of nephrotoxic agents [1].

In the acute settings of generalized myasthenia gravis (MG) treatment options include plasma exchange (PLEX), intravenous immunoglobulin (IVIG), and pyridostigmines. A thymoma is associated with the disease in up to 20% of cases [1,2].

In cases where a thymoma is detected, surgical treatment to remove the tumor is recommended in certain age groups. At present, there are no clear guidelines regarding the optimal time to perform thymectomy after diagnosis of acute crisis or from the last treatment to thymectomy. Treatment is at the clinician's discretion.

Adenoid surgery (adenoidectomy) is one of the most common pediatric surgical procedures. Complications of this surgery include anesthetic issues, bleeding, pain, dysphagia, and velopharyngeal insufficiency. The intraoperative complications are usually the most urgent and therefore require prompt identification and resolution. Tension pneumothorax (TPX) is a rare intraoperative. We present the first case in the English literature, to the best of our knowledge, of TPX during adenoid/tonsil surgery.

Acute or chronic aortic dissection is considered a rare emergency, with an estimated rate of 2.9 to 5 cases per 100,000 patients each year. This condition is most prevalent in males older than 65 years of age with a history of hypertension, atherosclerosis, and previous cardiac surgery [1,2]. To confirm the diagnosis, imaging is used, often by computed tomography angiography (CTA) of the chest. Although prompt treatment is required, patients often present with non-specific symptoms, such as abdominal pain or neurologic deficits, resulting in the early diagnosis of less than 20% and a high mortality rate [1].

Thyroid storm-related heart failure is a rare, life threatening complication of hyperthyroidism. In refractory cases, urgent thyroidectomy is required for definitive control of thyrotoxicosis. venoarterial extracorporeal membrane oxygenation (VA-ECMO) is a supportive measure for cardiorespiratory failure requiring continuous anticoagulation to prevent clotting. We presented two cases of thyrotoxic cardiac failure that necessitated VA-ECMO. One of the patients was successfully treated with thyroidectomy while on VA-ECMO. To the best of our knowledge, only two such cases have previously been reported.

Metastatic pulmonary calcinosis (MPC) is characterized by deposits of calcium in normal pulmonary parenchyma. Diffuse pulmonary calcinosis commonly occurs in hypercalcemia and/or hyperphosphatemia and is more commonly related to renal failure than primary hyperparathyroidism, skeletal metastases, or multiple myeloma [1]. Calcium depositions favor alkaline tissue and are thus more common in the upper lobes of the lung, which have a higher ventilation to perfusion ratio and a low capillary pCO₂, resulting in an alkaline pH [2]. Therefore, the most common radiographic manifestation consists of poorly defined nodular opacities bilaterally in the upper lung zones [3].

Romidepsin is an intravenously administered antineoplastic agent, which acts by inhibiting histone deacetylases, thus preventing removal of acetyl groups from histones. The accrual of acetyl groups on histones causes cell cycle arrest and apoptotic cell death. It was approved for use in the United States in 2009 for treatment of refractory or relapsed cutaneous and peripheral T cell lymphomas [1-3].

The most common side effects are mild to moderate in severity and include nausea, vomiting, fatigue, fever, myelosuppression (e.g., anemia, neutropenia, thrombocytopenia), elevated liver enzymes, constipation, and rash. More severe adverse events can include marked neutropenia, thrombocytopenia, serious infections such as line sepsis, acute renal failure, tumor lysis syndrome, and cardiac arrhythmias [1].

Myelodysplastic syndrome (MDS) is frequently associated with clinical manifestations of autoimmune disorders (AD) and inflammatory responses of the immune system. The biological linkage between MDS clones and the occurrence of autoimmune manifestations is mirrored by the response of the latter to MDS modifying therapeutic approaches [1]. We encountered a rare case of MDS coexisting with antiphospholipid syndrome (APS), which was effectively treated with a hypomethylating agent followed by allogenic bone marrow transplantation.