Answer for BIR CoW 14 May 2023
Interstitial lung disease in SLE
Findings
Sub pleural reticular interlobular septal thickening in bilateral lung fields predominantly involving basal segments and anterior segments of bilateral upper lobe (seen typically in SLE ) GGO and honey combing noted no evidence of cardiomegaly /pulmonary hypertension IMPRESSION : Features suggestive of fibrotic type of NSIP
Discussion
NSIP is characterized histologically by alveolar wall thickening or fibrosis. all parts of the biopsy look the same and represent the same stage of the disease; in UIPIIPF, the biopsy appears heterogeneous. NSIP lack specific features that would allow a diagnosis of another interstitial pneumonia. Patients typically respond to treatment with steroids and the prognosis is good Fibrotic type :less GGO,traction bronchiectasis and reticular septal thickening CT chest examination reports were additionally evaluated for the presence or absence of several recently identified signs specifically associated with fibrosis in the setting of connective tissue disease, as described by Chung et al : 1.The anterior upper lobe sign was defined as a predominance of fibrosis within the anterior aspect of the upper lobes extending from the subpleural lung . 2.The exuberant honeycombing sign was defined as large, extensive, well-defined, homogeneous honeycomb cysts within the lungs constituting greater than 70% of the lobe 3.The straight-edge sign was defined as isolation of fibrosis to the lower zones with sharp demarcation between severely fibrotic and normal lung in the craniocaudal plane and without substantial extension of fibrosis along the lateral lung margins at coronal imaging . 4.The island-like fibrosis is the presence of sharply demarcated, wedge-shaped areas of peripheral fibrosis with acute margins with extension towards central lung, surrounded by normal lung
References
1. Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus: results from a single center. II. Predictor variables for mortality. J Rheumatol 1995;22
2. Keane MP, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax 2000;55(2):159–166.
3. Carmier D, Marchand-Adam S, Diot P, Diot E. Respiratory involvement in systemic lupus erythematosus. Rev Mal Respir 2010;27(8):e66–e78.
4. Dhala A. Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. Clin Dev Immunol 2012;2012:854941.
5. Hannah JR, D’Cruz DP. Pulmonary complications of systemic lupus erythematosus. Semin Re
Findings
Sub pleural reticular interlobular septal thickening in bilateral lung fields predominantly involving basal segments and anterior segments of bilateral upper lobe (seen typically in SLE ) GGO and honey combing noted no evidence of cardiomegaly /pulmonary hypertension IMPRESSION : Features suggestive of fibrotic type of NSIP
Discussion
NSIP is characterized histologically by alveolar wall thickening or fibrosis. all parts of the biopsy look the same and represent the same stage of the disease; in UIPIIPF, the biopsy appears heterogeneous. NSIP lack specific features that would allow a diagnosis of another interstitial pneumonia. Patients typically respond to treatment with steroids and the prognosis is good Fibrotic type :less GGO,traction bronchiectasis and reticular septal thickening CT chest examination reports were additionally evaluated for the presence or absence of several recently identified signs specifically associated with fibrosis in the setting of connective tissue disease, as described by Chung et al : 1.The anterior upper lobe sign was defined as a predominance of fibrosis within the anterior aspect of the upper lobes extending from the subpleural lung . 2.The exuberant honeycombing sign was defined as large, extensive, well-defined, homogeneous honeycomb cysts within the lungs constituting greater than 70% of the lobe 3.The straight-edge sign was defined as isolation of fibrosis to the lower zones with sharp demarcation between severely fibrotic and normal lung in the craniocaudal plane and without substantial extension of fibrosis along the lateral lung margins at coronal imaging . 4.The island-like fibrosis is the presence of sharply demarcated, wedge-shaped areas of peripheral fibrosis with acute margins with extension towards central lung, surrounded by normal lung
References
1. Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus: results from a single center. II. Predictor variables for mortality. J Rheumatol 1995;22
2. Keane MP, Lynch JP 3rd. Pleuropulmonary manifestations of systemic lupus erythematosus. Thorax 2000;55(2):159–166.
3. Carmier D, Marchand-Adam S, Diot P, Diot E. Respiratory involvement in systemic lupus erythematosus. Rev Mal Respir 2010;27(8):e66–e78.
4. Dhala A. Pulmonary arterial hypertension in systemic lupus erythematosus: current status and future direction. Clin Dev Immunol 2012;2012:854941.
5. Hannah JR, D’Cruz DP. Pulmonary complications of systemic lupus erythematosus. Semin Re
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!