ABSTRACT
A twenty-two-year-old woman with a history of pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection (PANDAS) on rituximab presented with fever, abdominal pain, and worsening shortness of breath requiring supplemental oxygen via nasal cannula one month after a severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection from which she was minimally symptomatic and had recovered. Radiographic studies revealed bilateral patchy consolidations interspersed with ground-glass opacities (GGO). She was started on antibiotics for presumed community-acquired pneumonia with no improvement. Echocardiography revealed preserved biventricular function and a suspected intracardiac mass. A cardiac magnetic resonance imaging (CMRI) revealed myocarditis and no intracardiac mass. Fever persisted and oxygen requirements increased from FiO2 0.4 to 1.0. Repeat CXR showed subtotal left hemithorax opacification. Bronchoscopic samples showed a negative Gram stain and an unremarkable cell count differential. In view of this and given her lack of response to antibiotics with worsening respiratory status, high-dose steroids were started. She improved rapidly, and six days later she was off oxygen. Transbronchial biopsies showed benign parenchyma with some intra-alveolar fibrin deposition with no definitive evidence of viral cytopathic effect, vasculitis, or diffuse alveolar damage (DAD). Follow-up imaging in the pulmonary clinic revealed improvement of prior airspace disease with some new migratory opacities that completely resolved after 12 weeks. Pulmonary function tests and repeat CMRI were normal three months after discharge. Multisystem inflammatory syndrome in adults (MISA), post-covid organizing pneumonia (OP), and immune reconstitution inflammatory syndrome (IRIS) are rare and potentially steroid-responsive causes of pneumonia, which were in our differential diagnosis. It is imperative to consider the rare possibility of steroid-responsive pneumonia-like MISA, post-COVID-OP, and IRIS in patients with worsening respiratory symptoms following a recent SARS-CoV 2 infection.
ABSTRACT
A 21-year-old pregnant female with no significant past medical history presented with acute onset headache and nausea as well as tonic-clonic seizures, then rapidly decompensated into a coma with complete absence of brainstem reflexes. The patient was ultimately diagnosed with hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome) and subsequent posterior reversible encephalopathy syndrome (PRES) with brainstem involvement. Emergent delivery and blood pressure control resulted in rapid and complete neurologic recovery.
ABSTRACT
Pulmonary arterial hypertension is common in patients with SSc. Fig. 1 shows the diagnostic and therapeutic approach to PAH in SSc. Doppler echocardiography may suggest the diagnosis, but RHC is necessary to confirm PAH and to measure vasoreactivity. Therapy is directed at the underlying connective tissue disease. Vasoreactive patients often benefit from therapy with high-dose calcium-channel [figure: see text] blockers, but most patients are not vasoreactive. Intravenous epoprostenol and oral endothelin-1 receptor antagonists improve hemodynamic measurements and symptoms in SSc-associated PAH. The therapy of right ventricular failure is focused on vasodilators, inotropes, and diuretics with careful attention to avoiding systemic hypotension. The scleroderma pulmonary-renal syndrome and the scleroderma renal crisis are distinct syndromes with different clinical presentations, histopathologic manifestations, treatments, and outcomes. The scleroderma pulmonary renal syndrome is an autoimmune vasculitis of kidney and lung associated with normal blood pressure. Treatment is supportive, and prognosis is dismal. In contrast, scleroderma renal crisis is associated with systemic hypertension, onion skinning of afferent arterioles, and response to ACE inhibition and renal replacement therapy. Pericardial effusions are common but only occasionally lead to tamponade. Esophageal dysmotility is often associated with aspiration, leading to pulmonary fibrosis, pneumonia, or ARDS. Diffuse bowel involvement may result in pseudo-obstruction, bacterial overgrowth, or malabsorption. Prokinetic agents, antibiotics, and parenteral nutrition may be required.