Interstitial Lung Disease Associated with Connective Tissue Diseases.

Pulmonary manifestations of connective tissue diseases (CTD) carry high morbidity and potential mortality, and the most serious pulmonary type is interstitial lung disease (ILD). Identifying and promptly intervening CTD-ILD with immune suppressor therapy will change the natural course of the disease resulting in survival improvement. Compared to idiopathic pulmonary fibrosis, the most common presentation of idiopathic interstitial pneumonia (IIP), CTD-ILD carries a better prognosis due to the response to immune suppressor therapy. Nonspecific interstitial pneumonia (NSIP) is the most common type of CTD-ILD that is different from the fibrotic classical presentation of IPF, known as usual interstitial pneumonia (UIP). An exception is rheumatoid arthritis that presents more frequently with UIP type. Occasionally, IPF may not have typical radiographic features of UIP, and a full assessment to differentiate IPF from CTD-ILD is necessary, including the intervention of a multidisciplinary team and the histopathology. Interstitial pneumonia with autoimmune features (IPAF) shows promising advantages to identify patients with ILD who have some features of a CTD without a defined autoimmune disease and who may benefit from immune suppressors. A composition of clinical, serological, and morphologic features in patients presenting with ILD will fulfill criteria for IPAF. In summary, the early recognition and treatment of CTD-ILD, differentiation from IPF-UIP, and identification of patients with IPAF fulfill the assessment by the clinician for an optimal care.

Sjogren's Syndrome and Pulmonary Disease.

Sjogren's syndrome is an autoimmune connective tissue disease targeting the exocrine glands and frequently affecting the respiratory system. The pulmonary disease is the most important extra-glandular manifestation as it carries most of the morbidity and mortality. Typically, it affects the small airways ranging from mild to severe respiratory symptoms. The upper airways are also commonly involved, predisposing sinusitis to occur more frequently than in the normal population. Lymphocytic interstitial pneumonia was initially thought to be the prevailing parenchymal disease; however, multiple cohorts report non-interstitial pneumonia to be the most frequent subtype of interstitial lung disease. In the review of high-resolution computed tomography scans, cystic lesions are commonly found and associate with both the small airways and parenchymal disease. Under their presence, amyloidosis or lymphomas should be considered in the differential. Overall, Sjogren's syndrome has a higher risk for lymphoma, and in lungs this condition should be thought of, especially when the images reveal pulmonary nodularity, lymphocytic interstitial pneumonia and lymphadenopathy. Although, pulmonary artery hypertension was traditionally and exceptionally linked with Sjogren's syndrome, together with systemic lupus erythematosus, they are now acknowledged to be the most common pulmonary vascular disease in east Asian populations, even over patients with systemic sclerosis. Although there are no controlled prospective trials to treat pulmonary disease in Sjogren's syndrome, the mainstay treatment modality still falls on glucocorticoid therapy (systemic and inhaled), combined with immune modulators or alone. Most of the evidence sustains successful outcomes based on reported cases or case series.

Prostatitis, a rare presentation of granulomatosis with polyangiitis, successfully treated with rituximab and prednisone.

A previously healthy 21-year-old man presented with an 8-month history of weight loss, lethargy and dysuria unresponsive to empiric antibiotics and paraurethral drainage of a prostatic abscess. Urinalysis showed pyuria, but cultures failed to grow any organisms. Additionally, he developed new onset sensorineural hearing loss. CT of the chest showed two right-sided cavitary lesions. CT of the abdomen and pelvis demonstrated a prostatic abscess. A prostate biopsy demonstrated necrotising granulomatous prostatitis. A lung biopsy showed necrotising granulomatous inflammation. He was diagnosed with granulomatosis with polyangiitis (GPA). He was successfully treated with rituximab and prednisone. At 6-month follow-up, he continued to be in remission with resolution of his symptoms. This case demonstrates a rare presentation of prostatitis as the presenting symptom of GPA. As far as we know, this case is the first documented report of rituximab and prednisone as successful therapy for prostatitis secondary to GPA.

Safe usage of anakinra and dexamethasone to treat refractory hemophagocytic lymphohistiocytosis secondary to acute disseminated histoplasmosis in a patient with HIV/AIDS.

Hemophagocytic lymphohistiocytosis (HLH) is a serious life-threatening disease if not recognised early. In patients with HIV/AIDS, this association has been reported following acute opportunistic infections, including histoplasmosis. However, optimal treatment is not known. We describe a male aged 46 years with AIDS who developed HLH following acute disseminated histoplasmosis. Presenting symptoms included fever, hepatosplenomegaly and pancytopenia. Bone marrow biopsy confirmed HLH. Initially, he was refractory to the treatment with amphotericin B, antiretroviral therapy and intravenous immunoglobulin (IVIG). Anakinra, an interleukin-1 receptor antagonist, and dexamethasone were initiated. He improved clinically, did not exhibit any harmful effects and ultimately was discharged from the hospital. This, we believe, is the first reported treatment of HLH with anakinra in a patient with AIDS and acute disseminated histoplasmosis.

Lymphocytic mastitis preceding Sjögren's syndrome.

Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and B cell hyperreactivity. Lacrimal and salivary glands are the most commonly involved causing keratoconjunctivitis sicca and xerostomia. A wide variety of other glandular and extraglandular manifestations can occur in SS. Lymphocytic mastitis is a rare presentation of several conditions including diabetes mellitus and autoimmune disorders. We report a case of a 43-year-old woman with a four-year history of arthralgias and positive antinuclear antibodies who developed a right painless breast mass. Biopsy revealed lymphocytic mastitis with predominant B cells. One year later she developed severe constitutional symptoms, sicca symptoms, lymphadenopathy, anemia, and interstitial lung disease. Serologies and minor salivary gland were consistent with the diagnosis of SS. This case further supports the association of lymphocytic mastitis with autoimmune diseases and demonstrates that it can even precede the clinical diagnosis of these entities.

Reactive thrombocytosis: an early manifestation of Takayasu arteritis.

A woman with Takayasu arteritis is reported who presented with constitutional symptoms and persistent thrombocytosis documented since 3 years before the diagnosis. Disease-specific symptoms such as arm claudication, transient loss of vision, and self-remitting eye ptosis present at the time were apparently missed, because she is a non-English-speaking Hispanic woman whose history was obtained through an interpreter. Extensive workup done at the time failed to reach a definite etiology. A computed tomography scan of the chest done because of midthoracic back pain and an elevated erythrocyte sedimentation rate showed circumferential wall thickening and mild surrounding edema throughout the thoracic and abdominal aorta and both carotid arteries consistent with Takayasu arteritis. Prednisone at a dosage of 1 mg/kg twice a day decreased the platelet count within 45 days of its initiation. Takayasu arteritis should be considered in the differential diagnosis of unexplained thrombocytosis, particularly in young women.

IgA deficiency: clinical correlates with IgG subclass and mannan-binding lectin deficiencies.

OBJECTIVE: To characterize an IgA deficient population in terms of the incidence of IgG subclass and mannose-binding lectin (MBL) deficiencies and the type and severity of infections and other associated disorders. BACKGROUND: Selective IgA deficiency is probably the commonest of the primary immunodeficiency disorders and although it may lead to an increased risk for respiratory and gastrointestinal infections and associated to various autoimmune diseases, it may also be asymptomatic. Several studies have suggested the need of a concomitant defect in order for manifestation of its symptoms. METHODS: A total of 27 patients fulfilling the diagnostic criteria of selective IgA deficiency were evaluated for IgG subclass and MBL deficiencies after a thorough medical history, physical examination and pertinent evaluation for concomitant medical conditions. RESULTS: The overall incidence of IgG subclass deficiency found in the IgA deficient group was 18.5%. MBL deficiency was found to be 3.7%. These frequencies may have been influenced by the age group evaluated and the size of the population studied. Severe infections were more common in patients with combined deficiencies, either IgA and any of the IgG subclasses or IgA and MBL deficiency. Atopy was widely represented in the patients studied. CONCLUSIONS: The observed relationship between combined deficiencies Ig A, IgG subclasses and MBL and the increased representation of severe infections needs to be corroborated in a larger sample of patients with an inclusion of pediatric patients.