The presence of at least three of these criteria had a sensitivity of 93% and a specificity of 99% (Table 2). However, some authors have published case reports describing unusual difficulty with this diagnosis. Patients with sarcoidosis may have positive rheumatoid factor, and/or positive an-tinuclear antibody assays, and/or elevated uric acid levels, and these laboratory results can lead to confusion. Most patients with sarcoidosis (> 90%) have intrathoracic lymphadenopathy. Biopsies reveal non-caseating granulomas. However, similar results occur in patients with granulomatous infection and malignancy. Most patients with Lofgren syndrome have complete resolution of their symptoms, and this syndrome represents a benign acute presentation of sarcoidosis with a good prognosis. In the series of Lofgren, 102 patients (91.9%) had complete resolution of hilar adenopathy and infiltrates, if present, by 2 years. comments
ARMI News - Part 9
Periarticular inflammation with soft tissue swelling with no direct joint involvement is considered a variant of Lofgren syndrome. Ultrasound and MRI can provide an exact anatomic location of the inflammatory process. The joint fluid, if present, is usually mildly inflammatory with a WBC in the range of 4,000 to 15,000/^L and a predominance of lymphocytes. Synovial biopsy specimens typically reveal nonspecific proliferative inflammatory changes. Noncaseating granulomas are uncommon in the acute arthritic presentation. The association between articular involvement and erythema nodosum in patients with Lofgren syndrome is not constant. comments
What is the differential diagnosis in this patient with asymmetric large-joint oligoarthritis, fever, and bilateral hilar adenopathy?
How should this patient be treated?
What clinical course should be expected?
Diagnosis: Sarcoid arthritis consistent with Lofgren syndrome
Treatment: Nonsteroidal antiinflammatory drugs or corticosteroids
Our patient presented with subacute polyarthritis, bilateral hilar adenopathy, and fever. She did not have erythema nodosum. The differential diagnosis of polyarthritis and fever is extensive. The heuristic for limiting the number of possibilities will certainly depend on the clinician’s experience. The best approach might involve consideration of the frequency of various diseases, analysis of information clusters, or assessment of a predominant symptom, sign, or laboratory test. website
A 44-Year-Old Woman With Polyarthritis, Fever, and Hilar Adenopathy: Laboratory and Radiographic Findings
Abnormal laboratory test results included glucose, 147 mg/dL; C-reactive protein, 5 mg/L (0.7 to 1.0 mg/dL); erythrocyte sedimentation rate, 44 mm/h; total complement, 88 U/mL (26 to 58 U/mL); and hepatitis B core antibody positive. CBC differential count, electrolytes, lever function, renal function, and creatine kinase were all within normal limits. Urinalysis showed hematuria and trace ketones. Rheumatoid factor, anti-streptolysin O, anti-nuclear antibody, anti-Smith antibody, anti-Smith/ribonucle-oprotein antibody, uric acid, Borrelia burgdorferi and parvovirus B19 serologies, angiotensin-converting enzyme levels, serum calcium, and purified protein derivative were all negative or within normal limits.
A 44-year-old white woman presented with an 8-week history of increasing bilateral (right more than left) ankle pain with progressive periarticular edema and inflammation. The right wrist and both elbows were additively and mildly involved. She had intermittent fever with no clear pattern, malaise, mild thoracic spine pain, and intermittent limb muscle aches. Dry cough was present 4 days before hospital admission. The patient was treated with nonsteroidal antiinflammatory drugs and morphine at another hospital for 4 days before being transferred to our institution for management of asymmetric large-joint polyarthritis of unclear etiology. She denied sputum production, dyspnea, chest pain, syncope, headaches, visual disturbances, nausea, vomiting, abdominal pain, diarrhea, history of bleeding, dysuria, night sweats, weight loss, and skin rash.
LIP is most often seen in patients with underlying immunologic abnormalities, especially Sjogren syndrome and AIDS, and is characterized histologically by diffuse hyperplasia of bronchus-associated lymphatic tissue, resulting in a diffuse, polyclonal lymphoid cell infiltrate surrounding the airways and expanding the lung intersti-tium., As reported by Johkoh et al, in a study of 22 patients with documented LIP, while subpleural nodules could be identified in 86% of cases, likely reflecting subpleural lymphatic involvement, poorly defined centrilobular nodules could be seen in 100% of cases. Additional imaging features include the presence of thickened bronchovascular bundles and, in particular, the presence of randomly distributed thin-walled cysts. Poorly defined centrilobular nodules may also be seen early in the course of LCH., However, these most often are associated with characteristic bizarrely shaped, thick walled cysts, some of which represent cavitary nodules with characteristic sparing of the lung bases. More info
Those cases in which centrilobular nodules are present in patients in the absence of tree-in-bud opacities constitute the last part of this CT scan algorithm (Table 1). Included in this category are a variety of diseases or “mixed” entities that have in common localization to the centrilobular portion of the secondary lobule. This includes diseases that primarily affect the centrilobular bronchiole, as well as those that are either primarily peribronchiolar or perivascular in origin. Most often, this group of diseases results in a pattern of diffuse, poorly defined ground-glass nodules, which are typically the result of a primarily peribronchiolar distribution. The classic example of this appearance is subacute HP (Fig 12)., This diagnosis is frequently first suggested on the basis of CT scan findings and is usually established by a combination of exposure history, clinical symptoms of a flu-like illness, the presence of specific serum antibodies when those data are available, increased numbers of lymphocytes and neutrophils in BAL fluid, and, when feasible, clinically significant improvement in symptoms when the patient is removed from the offending environmental agent.
While classically described in patients with an endobronchial spread of tuberculosis, in fact, tree-in-bud opacities can be identified in virtually any type of infectious bronchiolitis. This includes Mycobacterium tuberculosis, Mycobacterium avium-intra-cellulare, bacterial, viral, and fungal infections, and allergic bronchopulmonary mycosis. This pattern is also frequently encountered in patients with AIDS in whom recurrent episodes of bronchial infection are frequent. Differential diagnosis also includes follicular bronchiolitis, an entity that is characterized by the presence of hyperplastic lymphoid follicles and germinal centers occurring along the bronchovascu-lar bundles. Most often, infectious bronchiolitis results in clusters of tree-in-bud opacities. When they are widespread and diffuse, the differential diagnosis includes “Asiatic panbronchiolitis.”, This entity has a well-established predilection in Japanese, Chinese, and Korean populations, appears to show a genetic predisposition, and is usually seen in association with chronic sinusitis. Diffuse tree-in-bud opacities are also frequently encountered in patients with cystic fibrosis and viral bronchiolitis. read more
Group 2: In distinction with the patterns described in patients in group 1, group 2 includes those patients in whom no or very few nodules are perifis-sural or subpleural in distribution. Anatomically, these nodules are grouped together as being centri-lobular in distribution. By definition, these entities primarily involve centrilobular bronchioles and/or their accompanying pulmonary artery branches. Anatomically, these structures taper peripherally, stopping 5 to 10 mm short of the pleural or interlobular septal surfaces and consequently fail to involve pleural and fissural surfaces (Table 1). As will be discussed, these nodules typically fall into the following two broad categories: those with a “tree-in-bud” configuration; and those that appear as amorphous “ground-glass” nodules. Once nodules are characterized as being primarily centrilobular in distribution, further assessment requires determining whether or not these have a tree-in-bud configuration. Tree-in-bud opacities are characterized by the appearance of centrilobular micronodular branching structures that end several millimeters distant from nearby pleural or fissural surfaces (Fig 11). more
A basilar predominance is typically noted due to preferential blood flow to the lung bases. Individual nodules may have “feeding vessels” consistent with their hematogenous origin. On HRCT scans, a connection between nodules and the adjacent pulmonary vessels (ie, the mass-vessel sign) may be seen in approximately 75% of cases. Nodules may also be either cavitary or surrounded by a “halo” of ground-glass attenuation, which is typical of hemorrhagic metastases such as those due to choriocarcinoma. Features of lymphangitic cancer may also be present, which again is consistent with a hematogenous origin of disease.